Human MitoCarta3.0

The Human MitoCarta3.0 inventory is a collection of 1136 nuclear and mtDNA genes encoding proteins with strong support of mitochondrial localization. This table provides a summary of evidence supporting mitochondrial localization, the protein distribution across 14 tissues, as well as annotation of biological pathways (based on manual literature review) and sub-compartment (based on annotator's "best guess" integrating sequence features, experimental data, literature, and pathway context; note all subunits of protein complexes are assigned the same compartment). The genes are ordered by strength of mitochondrial evidence. 

When using this data, please cite:

  • Rath, S., Sharma, R., Gupta, R., ..., Calvo, S.E., Mootha, V.K. MitoCarta3.0: An updated inventory of the mitochondrial proteome, now with sub-organelle localization and pathway annotations (2020). Nucleic Acids Research [Pubmed: 33174596] 

More complete information is available for download in Human.MitoCarta3.0.xls and fasta and bed files.

See also MitoPathways.

 

SymbolDescriptionSynonymsMaestro scoreEvidenceSub-compartmentMitoPathwaysTissues
CYC1cytochrome ­c1MC3DN6, UQCR4, P08574, gene:153743literature, APEX_IMS, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MS++MIMOXPHOS > Complex III > CIII subunits;
Metabolism > Metals and cofactors > Heme-containing proteins;
Metabolism > Electron carriers > Cytochromes;
OXPHOS > OXPHOS subunits		all 14
SDHBsuccinate ­dehydrogenase ­complex ­iron ­sulfur ­subunit ­BCWS2, IP, PGL4, SDH, SDH1, SDH2, SDHIP, P21912, gene:639043literature, APEX_matrix, targetP signal+, yeast mito homolog, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MS++MIMOXPHOS > Complex II > CII subunits;
Metabolism > Carbohydrate metabolism > TCA cycle;
Metabolism > Metals and cofactors > Fe-S-containing proteins;
OXPHOS > OXPHOS subunits		all 14
COQ7coenzyme ­Q7, ­hydroxylaseCAT5, CLK-1, CLK1, COQ10D8, Q99807, gene:1022943literature, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain+, coexpression++, MS/MS++MIMMetabolism > Metals and cofactors > Coenzyme Q metabolismall 14
SDHAsuccinate ­dehydrogenase ­complex ­flavoprotein ­subunit ­ACMD1GG, FP, PGL5, SDH1, SDH2, SDHF, P31040, gene:638942literature, APEX_matrix, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++MIMOXPHOS > Complex II > CII subunits;
Metabolism > Carbohydrate metabolism > TCA cycle;
OXPHOS > OXPHOS subunits		all 14
UQCRC1ubiquinol-cytochrome ­c ­reductase ­core ­protein ­1D3S3191, QCR1, UQCR1, P31930, gene:738442literature, APEX_matrix, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MS++MIMProtein import, sorting and homeostasis > Protein import and sorting > Preprotein cleavage;
OXPHOS > Complex III > CIII subunits;
OXPHOS > OXPHOS subunits		all 14
COQ5coenzyme ­Q5, ­methyltransferaseQ5HYK3, gene:8427442APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MS++MIMMetabolism > Metals and cofactors > Coenzyme Q metabolismall 14
PDHA1pyruvate ­dehydrogenase ­E1 ­subunit ­alpha ­1PDHA, PDHAD, PDHCE1A, PHE1A, P08559, gene:516042literature, APEX_matrix, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MS++MatrixMetabolism > Carbohydrate metabolism > Pyruvate metabolismall 14
COQ9coenzyme ­Q9C16orf49, COQ10D5, O75208, gene:5701742APEX_matrix, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain+, induction, coexpression, MS/MS++MIMMetabolism > Metals and cofactors > Coenzyme Q metabolismall 14
MRPL12mitochondrial ­ribosomal ­protein ­L125c5-2, L12mt, MRP-L31/34, MRPL7, MRPL7/L12, RPML12, P52815, gene:618242literature, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++MatrixMitochondrial central dogma > mtRNA metabolism > Transcription;
Mitochondrial central dogma > Translation > Mitochondrial ribosome		all 14
ATP5F1DATP ­synthase ­F1 ­subunit ­deltaATP5D, MC5DN5, P30049, gene:51342literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MS+MIMOXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits		all 14
COX5Acytochrome ­c ­oxidase ­subunit ­5ACOX, COX-VA, VA, P20674, gene:937742literature, APEX_IMS, APEX_matrix, targetP signal+, yeast mito homolog++, mito protein domain+, induction, coexpression++, MS/MS++MIMOXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits		all 14
ISCA2iron-sulfur ­cluster ­assembly ­2HBLD1, ISA2, MMDS4, c14_5557, Q86U28, gene:12296142APEX_matrix, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain+, induction, coexpression, MS/MS++MatrixMetabolism > Metals and cofactors > Fe-S cluster biosynthesis;
Metabolism > Metals and cofactors > Fe-S-containing proteins		all 14
PMPCBpeptidase, ­mitochondrial ­processing ­subunit ­betaBeta-MPP, MPP11, MPPB, MPPP52, P-52, O75439, gene:951241literature, APEX_matrix, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MS++MatrixProtein import, sorting and homeostasis > Protein import and sorting > Preprotein cleavage;
Protein import, sorting and homeostasis > Protein homeostasis > Proteases		all 14
UQCRFS1ubiquinol-cytochrome ­c ­reductase, ­Rieske ­iron-sulfur ­polypeptide ­1MC3DN10, RIP1, RIS1, RISP, UQCR5, P47985, gene:738640literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain+, coexpression++, MS/MS++MIMOXPHOS > Complex III > CIII subunits;
Metabolism > Metals and cofactors > Fe-S-containing proteins;
OXPHOS > OXPHOS subunits		all 14
ATP5F1AATP ­synthase ­F1 ­subunit ­alphaATP5A, ATP5A1, ATP5AL2, ATPM, COXPD22, HEL-S-123m, MC5DN4, MOM2, OMR, ORM, hATP1, P25705, gene:49840literature, APEX_matrix, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++MIMOXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits		all 14
OGDHoxoglutarate ­dehydrogenaseAKGDH, E1k, KGD1, OGDC, OGDH2, Q02218, gene:496740literature, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MS++MatrixMetabolism > Carbohydrate metabolism > TCA cycleall 14
PDHBpyruvate ­dehydrogenase ­E1 ­subunit ­betaPDHBD, PDHE1-B, PDHE1B, PHE1B, P11177, gene:516240literature, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MS++MatrixMetabolism > Carbohydrate metabolism > Pyruvate metabolismall 14
UQCRC2ubiquinol-cytochrome ­c ­reductase ­core ­protein ­2MC3DN5, QCR2, UQCR2, P22695, gene:738540literature, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MS++MIMProtein import, sorting and homeostasis > Protein import and sorting > Preprotein cleavage;
OXPHOS > Complex III > CIII subunits;
OXPHOS > OXPHOS subunits		all 14
SDHDsuccinate ­dehydrogenase ­complex ­subunit ­DCBT1, CII-4, CWS3, PGL, PGL1, QPs3, SDH4, cybS, O14521, gene:639240literature, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MS+MIMOXPHOS > Complex II > CII subunits;
Metabolism > Carbohydrate metabolism > TCA cycle;
Metabolism > Metals and cofactors > Heme-containing proteins;
OXPHOS > OXPHOS subunits		all 14
MRPS35mitochondrial ­ribosomal ­protein ­S35HDCMD11P, MDS023, MRP-S28, MRPS28, P82673, gene:6048839literature, APEX_matrix, targetP signal, yeast mito homolog++, mito protein domain+, induction, coexpression++, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
UQCRQubiquinol-cytochrome ­c ­reductase ­complex ­III ­subunit ­VIIMC3DN4, QCR8, QP-C, QPC, UQCR7, O14949, gene:2708939literature, APEX_IMS, targetP signal, yeast mito homolog++, mito protein domain+, induction, coexpression++, MS/MS++MIMOXPHOS > Complex III > CIII subunits;
OXPHOS > OXPHOS subunits		all 14
MRPL53mitochondrial ­ribosomal ­protein ­L53L53MT, Q96EL3, gene:11654039literature, APEX_matrix, targetP signal, yeast mito homolog++, mito protein domain+, induction, coexpression++, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
DBTdihydrolipoamide ­branched ­chain ­transacylase ­E2BCATE2, BCKAD-E2, BCKADE2, BCKDH-E2, BCOADC-E2, E2, E2B, P11182, gene:162939literature, APEX_matrix, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain+, induction, coexpression+, MS/MS++MatrixMetabolism > Amino acid metabolism > BCAA metabolism;
Metabolism > Amino acid metabolism > BCAA dehydrogenase complex		all 14
PDK4pyruvate ­dehydrogenase ­kinase ­4Q16654, gene:516638literature, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain+, induction, coexpression, MS/MS++MIMMetabolism > Carbohydrate metabolism > Pyruvate metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, testis
MDH2malate ­dehydrogenase ­2EIEE51, M-MDH, MDH, MGC:3559, MOR1, P40926, gene:419138literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++MatrixMetabolism > Carbohydrate metabolism > Gluconeogenesis;
Metabolism > Carbohydrate metabolism > Malate-aspartate shuttle;
Metabolism > Carbohydrate metabolism > TCA cycle		all 14
MRPS27mitochondrial ­ribosomal ­protein ­S27MRP-S27, S27mt, Q92552, gene:2310738literature, APEX_matrix, targetP signal+, yeast mito homolog++, mito protein domain+, induction, coexpression+, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
CScitrate ­synthaseO75390, gene:143137literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++MatrixMetabolism > Carbohydrate metabolism > TCA cycleall 14
GRPEL1GrpE ­like ­1, ­mitochondrialGrpE, HMGE, mt-GrpE#1, Q9HAV7, gene:8027337literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MS++MatrixProtein import, sorting and homeostasis > Protein import and sorting > Import motor;
Metabolism > Metals and cofactors > Fe-S cluster biosynthesis		all 14
DLATdihydrolipoamide ­S-acetyltransferaseDLTA, E2, PBC, PDC-E2, PDCE2, P10515, gene:173737literature, APEX_matrix, yeast mito homolog+, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MS++MatrixMetabolism > Carbohydrate metabolism > Pyruvate metabolismall 14
LRPPRCleucine ­rich ­pentatricopeptide ­repeat ­containingCLONE-23970, GP130, LRP130, LSFC, P42704, gene:1012837literature, APEX_matrix, targetP signal+, yeast mito homolog++, mito protein domain, induction, coexpression++, MS/MS++MatrixMitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay;
Mitochondrial central dogma > Translation		all 14
DLSTdihydrolipoamide ­S-succinyltransferaseDLTS, KGD2, PGL7, P36957, gene:174337literature, APEX_IMS, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MS+MatrixMetabolism > Carbohydrate metabolism > TCA cycle;
Metabolism > Amino acid metabolism > Lysine metabolism		all 14
PDHXpyruvate ­dehydrogenase ­complex ­component ­XDLDBP, E3BP, OPDX, PDHXD, PDX1, proX, O00330, gene:805037literature, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MS+MatrixMetabolism > Carbohydrate metabolism > Pyruvate metabolismall 14
GFM1G ­elongation ­factor ­mitochondrial ­1COXPD1, EFG, EFG1, EFGM, EGF1, GFM, hEFG1, mtEF-G1, Q96RP9, gene:8547637literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++MatrixMitochondrial central dogma > Translation > Translation factorsall 14
MPC2mitochondrial ­pyruvate ­carrier ­2BRP44, SLC54A2, O95563, gene:2587437literature, yeast mito homolog++, mito protein domain+, induction, coexpression++, MS/MS++MIMMetabolism > Carbohydrate metabolism > Gluconeogenesis;
Metabolism > Carbohydrate metabolism > Pyruvate metabolism;
Metabolism > Carbohydrate metabolism > TCA-associated;
Small molecule transport		all 14
NDUFS1NADH:ubiquinone ­oxidoreductase ­core ­subunit ­S1CI-75Kd, CI-75k, MC1DN5, PRO1304, P28331, gene:471936literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
Metabolism > Metals and cofactors > Fe-S-containing proteins;
OXPHOS > OXPHOS subunits		all 14
MRPL46mitochondrial ­ribosomal ­protein ­L46C15orf4, LIECG2, P2ECSL, Q9H2W6, gene:2658936literature, APEX_matrix, targetP signal+, yeast mito homolog++, mito protein domain, induction, coexpression++, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, placenta, testis
ATP5F1EATP ­synthase ­F1 ­subunit ­epsilonATP5E, ATPE, MC5DN3, P56381, gene:51436literature, yeast mito homolog++, mito protein domain+, induction, coexpression++, MS/MS++MIMOXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits		all 14
SLC25A3solute ­carrier ­family ­25 ­member ­3OK/SW-cl.48, PHC, PTP, Q00325, gene:525036literature, yeast mito homolog++, mito protein domain+, induction, coexpression++, MS/MS++MIMMetabolism > Metals and cofactors > Copper metabolism;
Signaling > Calcium homeostasis > Calcium cycle;
Small molecule transport > SLC25A family		all 14
MRPS23mitochondrial ­ribosomal ­protein ­S23CGI-138, COXPD46, HSPC329, MRP-S23, Q9Y3D9, gene:5164936literature, APEX_matrix, yeast mito homolog++, mito protein domain+, induction, coexpression++, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
FHfumarate ­hydrataseFMRD, HLRCC, HsFH, LRCC, MCL, MCUL1, P07954, gene:227135literature, APEX_matrix, targetP signal+, yeast mito homolog, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++MatrixMetabolism > Carbohydrate metabolism > TCA cycleall 14
PMPCApeptidase, ­mitochondrial ­processing ­subunit ­alphaAlpha-MPP, CLA1, CPD3, INPP5E, P-55, SCAR2, Q10713, gene:2320335literature, APEX_matrix, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain+, coexpression, MS/MS++MatrixProtein import, sorting and homeostasis > Protein import and sorting > Preprotein cleavage;
Protein import, sorting and homeostasis > Protein homeostasis > Proteases		all 14
ATP5F1BATP ­synthase ­F1 ­subunit ­betaATP5B, ATPMB, ATPSB, HEL-S-271, P06576, gene:50635literature, APEX_matrix, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression++, MS/MS++MIMOXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits		all 14
SDHAF4succinate ­dehydrogenase ­complex ­assembly ­factor ­4C6orf57, Sdh8, Q5VUM1, gene:13515435yeast mito homolog++, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MSMatrixOXPHOS > Complex II > CII assembly factors;
OXPHOS > OXPHOS assembly factors		adipose, testis
UQCR10ubiquinol-cytochrome ­c ­reductase, ­complex ­III ­subunit ­XHSPC051, HSPC119, HSPC151, QCR9, UCCR7.2, UCRC, Q9UDW1, gene:2979635literature, yeast mito homolog++, mito protein domain+, induction, coexpression++, MS/MS++MIMOXPHOS > Complex III > CIII subunits;
OXPHOS > OXPHOS subunits		all 14
ISCA1iron-sulfur ­cluster ­assembly ­1HBLD2, ISA1, MMDS5, hIscA, Q9BUE6, gene:8168935APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain+, induction, MS/MS++MatrixMetabolism > Metals and cofactors > Fe-S cluster biosynthesis;
Metabolism > Metals and cofactors > Fe-S-containing proteins		cerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
SUCLA2succinate-CoA ­ligase ­ADP-forming ­subunit ­betaA-BETA, A-SCS, LINC00444, MTDPS5, SCS-betaA, Q9P2R7, gene:880335literature, APEX_matrix, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain, induction, coexpression+, MS/MS++MatrixMetabolism > Carbohydrate metabolism > TCA cycle;
Metabolism > Carbohydrate metabolism > Itaconate metabolism;
Metabolism > Nucleotide metabolism > Nucleotide synthesis and processing		all 14
COQ3coenzyme ­Q3, ­methyltransferaseDHHBMT, DHHBMTASE, UG0215E05, bA9819.1, Q9NZJ6, gene:5180534APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain+, induction, coexpression+, MS/MS+MIMMetabolism > Metals and cofactors > Coenzyme Q metabolismcerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
IARS2isoleucyl-tRNA ­synthetase ­2, ­mitochondrialCAGSSS, ILERS, Q9NSE4, gene:5569934APEX_matrix, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression++, MS/MS++MatrixMitochondrial central dogma > Translation > mt-tRNA synthetasesall 14
MRPS15mitochondrial ­ribosomal ­protein ­S15DC37, MPR-S15, RPMS15, S15mt, P82914, gene:6496034literature, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression++, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
IDH3Aisocitrate ­dehydrogenase ­(NAD(+)) ­3 ­catalytic ­subunit ­alphaP50213, gene:341934APEX_matrix, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++MatrixMetabolism > Carbohydrate metabolism > TCA cycleall 14
COX11cytochrome ­c ­oxidase ­copper ­chaperone ­COX11COX11P, Q9Y6N1, gene:135334literature, APEX_IMS, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain+, induction, coexpression, MS/MSMIMOXPHOS > Complex IV > CIV assembly factors;
Metabolism > Metals and cofactors > Copper metabolism;
OXPHOS > OXPHOS assembly factors		cerebrum, cerebellum, spinalcord, skeletalmuscle, largeintestine, placenta
ETFDHelectron ­transfer ­flavoprotein ­dehydrogenaseETFQO, MADD, Q16134, gene:211034literature, APEX_matrix, yeast mito homolog++, mito protein domain+, induction, coexpression++, MS/MS++MIMMetabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Amino acid metabolism > BCAA metabolism;
Metabolism > Amino acid metabolism > Lysine metabolism;
Metabolism > Amino acid metabolism > Glycine metabolism;
Metabolism > Metals and cofactors > Fe-S-containing proteins;
Metabolism > Vitamin metabolism > Choline and betaine metabolism;
Metabolism > Electron carriers > Q-linked reactions, other		all 14
TIMM10translocase ­of ­inner ­mitochondrial ­membrane ­10TIM10, TIM10A, TIMM10A, P62072, gene:2651934literature, APEX_IMS, yeast mito homolog++, mito protein domain+, induction, coexpression++, MS/MS++MIMProtein import, sorting and homeostasis > Protein homeostasis > Chaperones;
Protein import, sorting and homeostasis > Protein import and sorting		cerebrum, cerebellum, brainstem, spinalcord, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
MRPL34mitochondrial ­ribosomal ­protein ­L34L34mt, Q9BQ48, gene:6498134literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
MRPL2mitochondrial ­ribosomal ­protein ­L2CGI-22, MRP-L14, RPML14, Q5T653, gene:5106934literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomespinalcord, liver, heart, adipose, smallintestine, largeintestine, placenta, testis
BCKDHAbranched ­chain ­keto ­acid ­dehydrogenase ­E1 ­subunit ­alphaBCKDE1A, MSU, MSUD1, OVD1A, P12694, gene:59334literature, APEX_matrix, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain+, coexpression+, MS/MS++MIMMetabolism > Amino acid metabolism > BCAA metabolism;
Metabolism > Amino acid metabolism > BCAA dehydrogenase complex		cerebrum, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
UQCRHubiquinol-cytochrome ­c ­reductase ­hinge ­proteinQCR6, UQCR8, P07919, gene:738834literature, APEX_IMS, yeast mito homolog++, mito protein domain+, induction, coexpression++, MS/MS++MIMOXPHOS > Complex III > CIII subunits;
OXPHOS > OXPHOS subunits		all 14
HIGD2AHIG1 ­hypoxia ­inducible ­domain ­family ­member ­2ARCF1b, Q9BW72, gene:19228634APEX_IMS, yeast mito homolog++, mito protein domain+, induction, coexpression++, MS/MS++MIMOXPHOS > OXPHOS assembly factors;
OXPHOS > Respirasome assembly		all 14
ATP5POATP ­synthase ­peripheral ­stalk ­subunit ­OSCPATP5O, ATPO, HMC08D05, OSCP, P48047, gene:53934literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain+, induction, MS/MS++MIMOXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits		all 14
ECHS1enoyl-CoA ­hydratase, ­short ­chain ­1ECHS1D, SCEH, P30084, gene:189233literature, APEX_matrix, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain, induction, coexpression+, MS/MS++MatrixMetabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Amino acid metabolism > BCAA metabolism;
Metabolism > Amino acid metabolism > Lysine metabolism		all 14
LETMD1LETM1 ­domain ­containing ­11110019O13Rik, HCCR, HCCR-1, HCCR-2, HCCR1, HCCR2, HCRR-2, SLC55A3, Q6P1Q0, gene:2587533GFP, targetP signal+, yeast mito homolog+, mito protein domain+, induction, coexpression, MS/MS++MembraneSmall molecule transportcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, placenta, testis
COX6A1cytochrome ­c ­oxidase ­subunit ­6A1CMTRID, COX6A, COX6AL, P12074, gene:133733literature, targetP signal+, yeast mito homolog+, mito protein domain+, induction, coexpression, MS/MS++MIMOXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits		all 14
COX15cytochrome ­c ­oxidase ­assembly ­homolog ­COX15CEMCOX2, Q7KZN9, gene:135533literature, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain+, coexpression++, MS/MSMIMOXPHOS > Complex IV > CIV assembly factors;
Metabolism > Metals and cofactors > Heme synthesis and processing;
OXPHOS > OXPHOS assembly factors		cerebrum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
AFG3L2AFG3 ­like ­matrix ­AAA ­peptidase ­subunit ­2OPA12, SCA28, SPAX5, Q9Y4W6, gene:1093933literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MS++MIMProtein import, sorting and homeostasis > Protein homeostasis > Proteasesall 14
HADHAhydroxyacyl-CoA ­dehydrogenase ­trifunctional ­multienzyme ­complex ­subunit ­alphaECHA, GBP, HADH, LCEH, LCHAD, MTPA, TP-ALPHA, P40939, gene:303033literature, APEX_matrix, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpression++, MS/MS++MIMMetabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Lipid metabolism > Cardiolipin synthesis;
Metabolism > Amino acid metabolism > BCAA metabolism		all 14
ETFAelectron ­transfer ­flavoprotein ­subunit ­alphaEMA, GA2, MADD, P13804, gene:210833literature, APEX_matrix, targetP signal, yeast mito homolog++, mito protein domain, induction, coexpression++, MS/MS++MatrixMetabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Amino acid metabolism > BCAA metabolism;
Metabolism > Amino acid metabolism > Lysine metabolism;
Metabolism > Amino acid metabolism > Glycine metabolism;
Metabolism > Vitamin metabolism > Choline and betaine metabolism;
Metabolism > Electron carriers > Q-linked reactions, other		all 14
NDUFS7NADH:ubiquinone ­oxidoreductase ­core ­subunit ­S7CI-20, CI-20KD, MC1DN3, MY017, PSST, O75251, gene:37429133literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
Metabolism > Metals and cofactors > Fe-S-containing proteins;
OXPHOS > OXPHOS subunits		all 14
CPT2carnitine ­palmitoyltransferase ­2CPT1, CPTASE, IIAE4, P23786, gene:137632literature, APEX_matrix, targetP signal+, yeast mito homolog++, mito protein domain, induction, coexpression+, MS/MS++MIMMetabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Metals and cofactors > Carnitine synthesis and transport;
Metabolism > Metals and cofactors > Carnitine shuttle		all 14
BCKDHBbranched ­chain ­keto ­acid ­dehydrogenase ­E1 ­subunit ­betaBCKDE1B, BCKDH E1-beta, E1B, P21953, gene:59432literature, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain+, coexpression+, MS/MS++MIMMetabolism > Amino acid metabolism > BCAA metabolism;
Metabolism > Amino acid metabolism > BCAA dehydrogenase complex		cerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
IDH3Bisocitrate ­dehydrogenase ­(NAD(+)) ­3 ­non-catalytic ­subunit ­betaRP46, O43837, gene:342032literature, APEX_matrix, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS++MatrixMetabolism > Carbohydrate metabolism > TCA cycleall 14
LARS2leucyl-tRNA ­synthetase ­2, ­mitochondrialHLASA, LEURS, PRLTS4, mtLeuRS, Q15031, gene:2339532literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression++, MS/MS++MatrixMitochondrial central dogma > Translation > mt-tRNA synthetasesall 14
ACADSacyl-CoA ­dehydrogenase ­short ­chainACAD3, SCAD, P16219, gene:3532literature, APEX_matrix, targetP signal+, mito protein domain+, induction, coexpression++, MS/MS++MatrixMetabolism > Lipid metabolism > Fatty acid oxidationall 14
LETM1leucine ­zipper ­and ­EF-hand ­containing ­transmembrane ­protein ­1SLC55A1, O95202, gene:395432literature, APEX_IMS, APEX_matrix, targetP signal, yeast mito homolog+, mito protein domain+, coexpression++, MS/MS++MIMSignaling > Calcium homeostasis > Calcium cycleall 14
ATP5MEATP ­synthase ­membrane ­subunit ­eATP5I, ATP5K, P56385, gene:52132literature, APEX_IMS, yeast mito homolog++, mito protein domain+, coexpression++, MS/MS++MIMOXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits;
Mitochondrial dynamics and surveillance > Cristae formation		all 14
OPA1OPA1 ­mitochondrial ­dynamin ­like ­GTPaseBERHS, MGM1, MTDPS14, NPG, NTG, largeG, O60313, gene:497632literature, APEX_IMS, targetP signal, yeast mito homolog++, mito protein domain, induction, coexpression++, MS/MS++MIMMitochondrial dynamics and surveillance > Fusion;
Mitochondrial dynamics and surveillance > Cristae formation		all 14
AUHAU ­RNA ­binding ­methylglutaconyl-CoA ­hydrataseQ13825, gene:54932literature, APEX_matrix, yeast mito homolog+, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++MatrixMetabolism > Carbohydrate metabolism > Itaconate metabolism;
Metabolism > Amino acid metabolism > BCAA metabolism		all 14
SUCLG1succinate-CoA ­ligase ­GDP/ADP-forming ­subunit ­alphaGALPHA, MTDPS9, SUCLA1, P53597, gene:880232yeast mito homolog+, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++MatrixMetabolism > Carbohydrate metabolism > TCA cycle;
Metabolism > Carbohydrate metabolism > Itaconate metabolism;
Metabolism > Nucleotide metabolism > Nucleotide synthesis and processing		all 14
NDUFV2NADH:ubiquinone ­oxidoreductase ­core ­subunit ­V2CI-24k, MC1DN7, P19404, gene:472932literature, APEX_matrix, targetP signal+, Rickettsial homolog, induction, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
Metabolism > Metals and cofactors > Fe-S-containing proteins;
OXPHOS > OXPHOS subunits		all 14
COQ6coenzyme ­Q6, ­monooxygenaseCGI-10, CGI10, COQ10D6, Q9Y2Z9, gene:5100432literature, APEX_matrix, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression+, MS/MS+MIMMetabolism > Metals and cofactors > Coenzyme Q metabolismcerebrum, cerebellum, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
MRPL43mitochondrial ­ribosomal ­protein ­L43L43mt, MRP-L43, bMRP36a, Q8N983, gene:8454531literature, targetP signal+, yeast mito homolog++, mito protein domain, induction, coexpression, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
ABHD11abhydrolase ­domain ­containing ­11PP1226, WBSCR21, Q8NFV4, gene:8345131literature, APEX_matrix, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression+, MS/MS++MatrixMetabolism > Carbohydrate metabolism > TCA-associatedcerebrum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
ATP5PFATP ­synthase ­peripheral ­stalk ­subunit ­F6ATP5, ATP5A, ATP5J, ATPM, CF6, F6, P18859, gene:52231literature, APEX_IMS, targetP signal+, mito protein domain+, induction, coexpression++, MS/MS++MIMOXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits		all 14
NDUFB8NADH:ubiquinone ­oxidoreductase ­subunit ­B8ASHI, CI-ASHI, MC1DN32, O95169, gene:471431literature, APEX_IMS, APEX_matrix, targetP signal+, mito protein domain+, induction, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		all 14
LONP1lon ­peptidase ­1, ­mitochondrialCODASS, LON, LONP, LonHS, PIM1, PRSS15, hLON, P36776, gene:936131literature, APEX_matrix, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS++MatrixProtein import, sorting and homeostasis > Protein homeostasis > Proteasesall 14
DLDdihydrolipoamide ­dehydrogenaseDLDD, DLDH, E3, GCSL, LAD, OGDC-E3, PHE3, P09622, gene:173831literature, APEX_matrix, targetP signal, yeast mito homolog, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++MatrixMetabolism > Carbohydrate metabolism > Pyruvate metabolism;
Metabolism > Carbohydrate metabolism > TCA cycle;
Metabolism > Amino acid metabolism > BCAA metabolism;
Metabolism > Amino acid metabolism > BCAA dehydrogenase complex;
Metabolism > Amino acid metabolism > Lysine metabolism;
Metabolism > Amino acid metabolism > Glycine metabolism;
Metabolism > Amino acid metabolism > Glycine cleavage system		all 14
AIFM1apoptosis ­inducing ­factor ­mitochondria ­associated ­1AIF, AUNX1, CMT2D, CMTX4, COWCK, COXPD6, DFNX5, NADMR, NAMSD, PDCD8, SEMDHL, O95831, gene:913131literature, APEX_IMS, targetP signal, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++MIMProtein import, sorting and homeostasis > Protein import and sorting > MIA40;
OXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors;
Mitochondrial dynamics and surveillance > Apoptosis		all 14
ECHDC3enoyl-CoA ­hydratase ­domain ­containing ­3Q96DC8, gene:7974631targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS++Matrixnonespinalcord, kidney, liver, heart, adipose, largeintestine, stomach, placenta, testis
APOOLapolipoprotein ­O ­likeCXorf33, FAM121A, MICOS27, Mic27, UNQ8193, Q6UXV4, gene:13932231literature, yeast mito homolog, mito protein domain+, induction, coexpression++, MS/MS++MIMMitochondrial dynamics and surveillance > Cristae formation > MICOS complexall 14
MRPL10mitochondrial ­ribosomal ­protein ­L10L10MT, MRP-L10, MRP-L8, MRPL8, RPML8, Q7Z7H8, gene:12499531literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression+, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
MRRFmitochondrial ­ribosome ­recycling ­factorMRFF, MTRRF, RRF, Q96E11, gene:9239930literature, APEX_matrix, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMitochondrial central dogma > Translation > Translation factorsall 14
NDUFS8NADH:ubiquinone ­oxidoreductase ­core ­subunit ­S8CI-23k, CI23KD, MC1DN2, TYKY, O00217, gene:472830literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
Metabolism > Metals and cofactors > Fe-S-containing proteins;
OXPHOS > OXPHOS subunits		all 14
ACADMacyl-CoA ­dehydrogenase ­medium ­chainACAD1, MCAD, MCADH, P11310, gene:3430literature, APEX_matrix, targetP signal, mito protein domain+, induction, coexpression++, MS/MS++MatrixMetabolism > Lipid metabolism > Fatty acid oxidationall 14
IMMTinner ­membrane ­mitochondrial ­proteinHMP, MICOS60, MINOS2, Mic60, P87, P87/89, P89, PIG4, PIG52, Q16891, gene:1098930literature, targetP signal, yeast mito homolog, mito protein domain+, coexpression++, MS/MS++MIMMitochondrial dynamics and surveillance > Cristae formation > MICOS complexall 14
TIMM9translocase ­of ­inner ­mitochondrial ­membrane ­9TIM9, TIM9A, Q9Y5J7, gene:2652030literature, APEX_IMS, yeast mito homolog++, mito protein domain+, induction, coexpression+, MS/MS++MIMProtein import, sorting and homeostasis > Protein homeostasis > Chaperones;
Protein import, sorting and homeostasis > Protein import and sorting		all 14
SLC25A4solute ­carrier ­family ­25 ­member ­4AAC1, ANT, ANT 1, ANT1, MTDPS12, MTDPS12A, PEO2, PEO3, PEOA2, T1, P12235, gene:29130literature, yeast mito homolog++, mito protein domain+, induction, coexpression, MS/MS++MIMMetabolism > Nucleotide metabolism > Nucleotide import;
Signaling > Calcium homeostasis > Mitochondrial permeability transition pore;
Small molecule transport > SLC25A family		all 14
SAMM50SAMM50 ­sorting ­and ­assembly ­machinery ­componentCGI-51, OMP85, SAM50, TOB55, TRG-3, YNL026W, Q9Y512, gene:2581330literature, APEX_IMS, APEX_matrix, yeast mito homolog++, Rickettsial homolog, induction, coexpression++, MS/MS++MOMProtein import, sorting and homeostasis > Protein import and sorting > SAM;
Mitochondrial dynamics and surveillance > Intramitochondrial membrane interactions		all 14
NDUFS2NADH:ubiquinone ­oxidoreductase ­core ­subunit ­S2CI-49, MC1DN6, O75306, gene:472030literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
Metabolism > Metals and cofactors > Fe-S-containing proteins;
OXPHOS > OXPHOS subunits		all 14
NDUFV1NADH:ubiquinone ­oxidoreductase ­core ­subunit ­V1CI-51K, CI51KD, MC1DN4, UQOR1, P49821, gene:472330literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
Metabolism > Metals and cofactors > Fe-S-containing proteins;
OXPHOS > OXPHOS subunits		all 14
ACO2aconitase ­2ACONM, HEL-S-284, ICRD, OCA8, OPA9, Q99798, gene:5030literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++MatrixMetabolism > Carbohydrate metabolism > TCA cycle;
Metabolism > Metals and cofactors > Fe-S-containing proteins		all 14
SUPV3L1Suv3 ­like ­RNA ­helicaseSUV3, Q8IYB8, gene:683230literature, APEX_matrix, targetP signal+, yeast mito homolog++, mito protein domain, induction, coexpression+, MS/MS+MatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
FECHferrochelataseEPP, EPP1, FCE, P22830, gene:223530literature, APEX_matrix, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MIMMetabolism > Metals and cofactors > Heme synthesis and processing;
Metabolism > Metals and cofactors > Fe-S-containing proteins		all 14
MTIF2mitochondrial ­translational ­initiation ­factor ­2P46199, gene:452830literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MSMatrixMitochondrial central dogma > Translation > Translation factorsall 14
PHBprohibitinHEL-215, HEL-S-54e, PHB1, P35232, gene:524529literature, yeast mito homolog+, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++MIMProtein import, sorting and homeostasis > Protein homeostasis > Chaperonesall 14
HIBCH3-hydroxyisobutyryl-CoA ­hydrolaseHIBYLCOAH, Q6NVY1, gene:2627529literature, APEX_matrix, targetP signal+, yeast mito homolog+, mito protein domain, induction, coexpression, MS/MS++MatrixMetabolism > Amino acid metabolism > BCAA metabolismall 14
MRPS2mitochondrial ­ribosomal ­protein ­S2CGI-91, COXPD36, MRP-S2, S2mt, Q9Y399, gene:5111629literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression+, MS/MS+MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, brainstem, spinalcord, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
HSPA9heat ­shock ­protein ­family ­A ­(Hsp70) ­member ­9CRP40, CSA, EVPLS, GRP-75, GRP75, HEL-S-124m, HSPA9B, MOT, MOT2, MTHSP75, PBP74, SAAN, SIDBA4, P38646, gene:331329APEX_matrix, targetP signal+, yeast mito homolog, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS++MatrixMetabolism > Metals and cofactors > Fe-S cluster biosynthesisall 14
SURF1SURF1 ­cytochrome ­c ­oxidase ­assembly ­factorCMT4K, Q15526, gene:683429literature, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain+, coexpression, MS/MSMIMOXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
PRDX3peroxiredoxin ­3AOP-1, AOP1, HBC189, MER5, PRO1748, SP-22, prx-III, P30048, gene:1093529literature, APEX_IMS, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++MatrixMetabolism > Detoxification > ROS and glutathione metabolismall 14
GHITMgrowth ­hormone ­inducible ­transmembrane ­proteinDERP2, HSPC282, MICS1, My021, PTD010, TMBIM5, Q9H3K2, gene:2706929literature, APEX_IMS, targetP signal+, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MSMIMMitochondrial dynamics and surveillance > Apoptosisall 14
GUF1GUF1 ­homolog, ­GTPaseEF-4, EF4, EIEE40, Q8N442, gene:6055829APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain+, coexpression, MS/MS+MatrixMitochondrial central dogma > Translationcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
TIMM13translocase ­of ­inner ­mitochondrial ­membrane ­13TIM13, TIM13B, TIMM13A, TIMM13B, ppv1, Q9Y5L4, gene:2651729literature, APEX_IMS, yeast mito homolog++, mito protein domain+, induction, coexpression, MS/MS++MIMProtein import, sorting and homeostasis > Protein homeostasis > Chaperones;
Protein import, sorting and homeostasis > Protein import and sorting		all 14
LYRM4LYR ­motif ­containing ­4C6orf149, CGI-203, COXPD19, ISD11, Q9HD34, gene:5712829literature, APEX_matrix, targetP signal+, yeast mito homolog, mito protein domain+, induction, coexpression, MS/MS++MatrixMetabolism > Metals and cofactors > Fe-S cluster biosynthesisall 14
MRPL16mitochondrial ­ribosomal ­protein ­L16L16mt, MRP-L16, PNAS-111, Q9NX20, gene:5494828literature, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression++, MS/MS+MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, placenta, testis
MRPL40mitochondrial ­ribosomal ­protein ­L40L40mt, MRP-L22, MRP-L40, MRPL22, NLVCF, URIM, Q9NQ50, gene:6497628literature, APEX_matrix, targetP signal+, yeast mito homolog++, mito protein domain+, induction, coexpression, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
IDH3Gisocitrate ­dehydrogenase ­(NAD(+)) ­3 ­non-catalytic ­subunit ­gammaH-IDHG, P51553, gene:342128literature, APEX_matrix, yeast mito homolog+, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++MatrixMetabolism > Carbohydrate metabolism > TCA cycleall 14
SDHCsuccinate ­dehydrogenase ­complex ­subunit ­CCYB560, CYBL, PGL3, QPS1, SDH3, Q99643, gene:639128literature, targetP signal+, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS+MIMOXPHOS > Complex II > CII subunits;
Metabolism > Carbohydrate metabolism > TCA cycle;
Metabolism > Metals and cofactors > Heme-containing proteins;
OXPHOS > OXPHOS subunits		all 14
NDUFB5NADH:ubiquinone ­oxidoreductase ­subunit ­B5CISGDH, SGDH, O43674, gene:471128literature, APEX_IMS, APEX_matrix, targetP signal+, mito protein domain+, induction, coexpression++, MS/MS+MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		all 14
SDHAF2succinate ­dehydrogenase ­complex ­assembly ­factor ­2C11orf79, PGL2, SDH5, Q9NX18, gene:5494928GFP, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain+, induction, coexpression, MS/MSMatrixOXPHOS > Complex II > CII assembly factors;
OXPHOS > OXPHOS assembly factors		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
COQ10Acoenzyme ­Q10AQ96MF6, gene:9305828targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain+, induction, MS/MS++MIMMetabolism > Metals and cofactors > Coenzyme Q metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, largeintestine, stomach
GATD3Aglutamine ­amidotransferase ­like ­class ­1 ­domain ­containing ­3AC21orf33, ES1, GATD3, GT335, HES1, KNPH, KNPI, P30042, gene:820928APEX_matrix, targetP signal+, mito protein domain, induction, coexpression++, MS/MS++Matrixnoneall 14
TXN2thioredoxin ­2COXPD29, MT-TRX, MTRX, TRX2, TXN, Q99757, gene:2582828literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS+MatrixMetabolism > Vitamin metabolism > Vitamin C metabolism;
Metabolism > Detoxification > ROS and glutathione metabolism		all 14
MRPS18Amitochondrial ­ribosomal ­protein ­S18AHumanS18b, MRP-S18-3, MRP-S18-a, MRPS18-3, S18bmt, S18mt-a, Q9NVS2, gene:5516828literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, brainstem, spinalcord, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
COX6Ccytochrome ­c ­oxidase ­subunit ­6CP09669, gene:134528literature, APEX_IMS, APEX_matrix, targetP signal, mito protein domain+, induction, coexpression++, MS/MS++MIMOXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits		all 14
NDUFB9NADH:ubiquinone ­oxidoreductase ­subunit ­B9B22, CI-B22, LYRM3, MC1DN24, UQOR22, Q9Y6M9, gene:471528literature, APEX_IMS, APEX_matrix, targetP signal, mito protein domain+, induction, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		all 14
MTCH2mitochondrial ­carrier ­2HSPC032, MIMP, SLC25A50, Q9Y6C9, gene:2378828literature, APEX_IMS, yeast mito homolog+, mito protein domain+, induction, coexpression+, MS/MS++MOMSmall molecule transport > SLC25A family;
Mitochondrial dynamics and surveillance > Fusion		all 14
NDUFA6NADH:ubiquinone ­oxidoreductase ­subunit ­A6B14, CI-B14, LYRM6, MC1DN33, NADHB14, P56556, gene:470028literature, APEX_matrix, mito protein domain+, induction, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		all 14
SLC25A20solute ­carrier ­family ­25 ­member ­20CAC, CACT, O43772, gene:78828literature, yeast mito homolog+, mito protein domain+, induction, coexpression, MS/MS++MIMMetabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Metals and cofactors > Carnitine synthesis and transport;
Metabolism > Metals and cofactors > Carnitine shuttle;
Small molecule transport > SLC25A family		all 14
MRPL1mitochondrial ­ribosomal ­protein ­L1BM022, L1MT, MRP-L1, Q9BYD6, gene:6500828literature, APEX_matrix, targetP signal, yeast mito homolog++, mito protein domain, induction, coexpression, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, spinalcord, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
TIMM44translocase ­of ­inner ­mitochondrial ­membrane ­44TIM44, O43615, gene:1046928literature, APEX_matrix, targetP signal, yeast mito homolog, mito protein domain, induction, coexpression++, MS/MS++MIMProtein import, sorting and homeostasis > Protein import and sorting > Import motorall 14
COX17cytochrome ­c ­oxidase ­copper ­chaperone ­COX17Q14061, gene:1006328literature, APEX_IMS, yeast mito homolog, mito protein domain+, induction, coexpression++, MS/MS++IMSOXPHOS > Complex IV > CIV assembly factors;
Metabolism > Metals and cofactors > Copper metabolism;
OXPHOS > OXPHOS assembly factors		heart, adipose, smallintestine, placenta, testis
MICOS10mitochondrial ­contact ­site ­and ­cristae ­organizing ­system ­subunit ­10C1orf151, MINOS1, MIO10, Mic10, Q5TGZ0, gene:44057428literature, APEX_IMS, yeast mito homolog, mito protein domain+, induction, coexpression++, MS/MS++MIMMitochondrial dynamics and surveillance > Cristae formation > MICOS complexall 14
SOD2superoxide ­dismutase ­2GClnc1, IPO-B, IPOB, MNSOD, MVCD6, Mn-SOD, P04179, gene:664828literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, MS/MS++MatrixMetabolism > Detoxification > ROS and glutathione metabolismall 14
COX6B1cytochrome ­c ­oxidase ­subunit ­6B1COX6B, COXG, COXVIb1, P14854, gene:134028literature, APEX_IMS, yeast mito homolog+, mito protein domain+, coexpression++, MS/MS++MIMOXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits		all 14
VDAC1voltage ­dependent ­anion ­channel ­1PORIN, VDAC-1, P21796, gene:741628literature, APEX_IMS, yeast mito homolog+, mito protein domain+, coexpression++, MS/MS++MOMSignaling > Calcium homeostasis > Mitochondrial permeability transition pore;
Mitochondrial dynamics and surveillance > Organelle contact sites;
Small molecule transport		all 14
CLPPcaseinolytic ­mitochondrial ­matrix ­peptidase ­proteolytic ­subunitDFNB81, PRLTS3, Q16740, gene:819228literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, induction, coexpression+, MS/MS++MatrixProtein import, sorting and homeostasis > Protein homeostasis > Proteasesall 14
HADHhydroxyacyl-CoA ­dehydrogenaseHAD, HADH1, HADHSC, HCDH, HHF4, MSCHAD, SCHAD, Q16836, gene:303328literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++MatrixMetabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Amino acid metabolism > Lysine metabolism		all 14
ACADLacyl-CoA ­dehydrogenase ­long ­chainACAD4, LCAD, P28330, gene:3328literature, targetP signal+, mito protein domain+, induction, coexpression+, MS/MS++MIMMetabolism > Lipid metabolism > Fatty acid oxidationall 14
ACAT1acetyl-CoA ­acetyltransferase ­1ACAT, MAT, T2, THIL, P24752, gene:3828literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, induction, coexpression+, MS/MS++MatrixMetabolism > Carbohydrate metabolism > Ketone metabolism;
Metabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Amino acid metabolism > BCAA metabolism;
Metabolism > Amino acid metabolism > Lysine metabolism		all 14
MALSU1mitochondrial ­assembly ­of ­ribosomal ­large ­subunit ­1C7orf30, mtRsfA, Q96EH3, gene:11541628literature, APEX_matrix, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression+, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosome assemblycerebrum, cerebellum, liver, smallintestine, largeintestine, placenta
CLPXcaseinolytic ­mitochondrial ­matrix ­peptidase ­chaperone ­subunit ­XEPP2, O76031, gene:1084527literature, APEX_matrix, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS++MatrixProtein import, sorting and homeostasis > Protein homeostasis > Proteasesall 14
NDUFS4NADH:ubiquinone ­oxidoreductase ­subunit ­S4AQDQ, CI-18, CI-18 kDa, CI-AQDQ, MC1DN1, O43181, gene:472427literature, APEX_matrix, targetP signal+, mito protein domain+, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		all 14
MRPL4mitochondrial ­ribosomal ­protein ­L4CGI-28, L4mt, Q9BYD3, gene:5107327literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, placenta, testis
C1QBPcomplement ­C1q ­binding ­proteinCOXPD33, GC1QBP, HABP1, SF2AP32, SF2p32, gC1Q-R, gC1qR, p32, Q07021, gene:70827literature, APEX_matrix, targetP signal+, yeast mito homolog++, mito protein domain, induction, coexpression, MS/MS++MatrixSignaling > Immune responseall 14
PITRM1pitrilysin ­metallopeptidase ­1MP1, PreP, Q5JRX3, gene:1053127literature, APEX_matrix, targetP signal, yeast mito homolog++, mito protein domain+, coexpression, MS/MS++MatrixProtein import, sorting and homeostasis > Protein homeostasis > Proteasesall 14
UQCC1ubiquinol-cytochrome ­c ­reductase ­complex ­assembly ­factor ­1BFZB, C20orf44, CBP3, UQCC, Q9NVA1, gene:5524527literature, APEX_matrix, targetP signal, yeast mito homolog, mito protein domain+, coexpression++, MS/MS++MIMOXPHOS > Complex III > CIII assembly factors;
OXPHOS > OXPHOS assembly factors		cerebrum, cerebellum, brainstem, spinalcord, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
MRPL55mitochondrial ­ribosomal ­protein ­L55AAVG5835, L55nt, MRP-L55, PRO19675, Q7Z7F7, gene:12830827literature, APEX_matrix, targetP signal, mito protein domain+, induction, coexpression++, MS/MS+MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
MRM1mitochondrial ­rRNA ­methyltransferase ­1Q6IN84, gene:7992227APEX_matrix, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain+, induction, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications		spinalcord, placenta, testis
MECRmitochondrial ­trans-2-enoyl-CoA ­reductaseCGI-63, DYTOABG, ETR1, FASN2B, NRBF1, Q9BV79, gene:5110227literature, APEX_matrix, targetP signal+, yeast mito homolog++, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Lipid metabolism > Type II fatty acid synthesisall 14
MRPL44mitochondrial ­ribosomal ­protein ­L44COXPD16, L44MT, MRP-L44, Q9H9J2, gene:6508027literature, APEX_matrix, targetP signal, yeast mito homolog++, induction, coexpression+, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, spinalcord, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
HSDL2hydroxysteroid ­dehydrogenase ­like ­2C9orf99, SDR13C1, Q6YN16, gene:8426327APEX_matrix, GFP, Rickettsial homolog, mito protein domain+, induction, coexpression+, MS/MS++Matrixnoneall 14
MRPS14mitochondrial ­ribosomal ­protein ­S14COXPD38, DJ262D12.2, HSMRPS14, MRP-S14, S14mt, O60783, gene:6393127literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
ABCB8ATP ­binding ­cassette ­subfamily ­B ­member ­8EST328128, M-ABC1, MABC1, MITOSUR, Q9NUT2, gene:1119427literature, targetP signal+, Rickettsial homolog, mito protein domain, coexpression++, MS/MS++MIMSmall molecule transport > ABC transportersall 14
ATPAF2ATP ­synthase ­mitochondrial ­F1 ­complex ­assembly ­factor ­2ATP12, ATP12p, LP3663, MC5DN1, Q8N5M1, gene:9164727literature, targetP signal, yeast mito homolog++, mito protein domain+, induction, coexpression, MS/MSMatrixOXPHOS > Complex V > CV assembly factors;
OXPHOS > OXPHOS assembly factors		cerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
NDUFA9NADH:ubiquinone ­oxidoreductase ­subunit ­A9CC6, CI-39k, CI39k, COQ11, MC1DN26, NDUFS2L, SDR22E1, Q16795, gene:470427literature, APEX_matrix, targetP signal+, mito protein domain, induction, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		all 14
COA6cytochrome ­c ­oxidase ­assembly ­factor ­6C1orf31, CEMCOX4, Q5JTJ3, gene:38875327APEX_IMS, yeast mito homolog++, mito protein domain+, induction, coexpression+, MS/MSIMSOXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors		cerebrum, cerebellum, spinalcord, liver, adipose, smallintestine, largeintestine, placenta, testis
POLRMTRNA ­polymerase ­mitochondrialAPOLMT, MTRNAP, MTRPOL, h-mtRPOL, O00411, gene:544227literature, APEX_matrix, targetP signal, yeast mito homolog++, mito protein domain+, coexpression++, MS/MSMatrixMitochondrial central dogma > mtDNA maintenance > mtDNA replication;
Mitochondrial central dogma > mtDNA maintenance > mtDNA nucleoid;
Mitochondrial central dogma > mtRNA metabolism > Transcription		cerebrum, cerebellum, brainstem, spinalcord, kidney, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
ISCUiron-sulfur ­cluster ­assembly ­enzyme2310020H20Rik, HML, ISU2, NIFU, NIFUN, hnifU, Q9H1K1, gene:2347927literature, APEX_matrix, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain, MS/MS++MatrixMetabolism > Metals and cofactors > Fe-S cluster biosynthesis;
Metabolism > Metals and cofactors > Fe-S-containing proteins		all 14
RTN4IP1reticulon ­4 ­interacting ­protein ­1NIMP, OPA10, Q8WWV3, gene:8481627literature, APEX_matrix, targetP signal, yeast mito homolog, mito protein domain, induction, coexpression++, MS/MS++Matrixnoneall 14
OGDHLoxoglutarate ­dehydrogenase ­LQ9ULD0, gene:5575327APEX_matrix, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain+, MS/MS++MatrixMetabolism > Carbohydrate metabolism > TCA cycleall 14
ATP5F1CATP ­synthase ­F1 ­subunit ­gammaATP5C, ATP5C1, ATP5CL1, P36542, gene:50927literature, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, MS/MS++MIMOXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits		all 14
BCKDKbranched ­chain ­keto ­acid ­dehydrogenase ­kinaseBCKDKD, BDK, O14874, gene:1029527literature, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain+, MS/MS++MatrixMetabolism > Amino acid metabolism > BCAA metabolism;
Metabolism > Amino acid metabolism > BCAA dehydrogenase complex		cerebrum, cerebellum, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
GFM2GTP ­dependent ­ribosome ­recycling ­factor ­mitochondrial ­2EF-G2mt, EFG2, MRRF2, MST027, MSTP027, RRF, RRF2, RRF2mt, hEFG2, mEF-G 2, Q969S9, gene:8434027literature, APEX_matrix, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression++, MS/MS++MatrixMitochondrial central dogma > Translation > Translation factorsall 14
NDUFAF4NADH:ubiquinone ­oxidoreductase ­complex ­assembly ­factor ­4C6orf66, HRPAP20, HSPC125, MC1DN15, My013, bA22L21.1, Q9P032, gene:2907827APEX_matrix, GFP, mito protein domain+, induction, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors		all 14
SLC25A35solute ­carrier ­family ­25 ­member ­35Q3KQZ1, gene:39951227APEX_matrix, yeast mito homolog+, mito protein domain+, induction, coexpression, MS/MS++MIMSmall molecule transport > SLC25A familycerebrum, cerebellum, kidney, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
SLC25A11solute ­carrier ­family ­25 ­member ­11OGC, PGL6, SLC20A4, Q02978, gene:840227literature, yeast mito homolog+, mito protein domain+, induction, coexpression, MS/MS++MIMMetabolism > Carbohydrate metabolism > Gluconeogenesis;
Metabolism > Carbohydrate metabolism > Malate-aspartate shuttle;
Metabolism > Carbohydrate metabolism > TCA-associated;
Small molecule transport > SLC25A family		all 14
TIMM8Btranslocase ­of ­inner ­mitochondrial ­membrane ­8 ­homolog ­BDDP2, TIM8B, Q9Y5J9, gene:2652127literature, yeast mito homolog+, mito protein domain+, induction, coexpression, MS/MS++MIMProtein import, sorting and homeostasis > Protein homeostasis > Chaperones;
Protein import, sorting and homeostasis > Protein import and sorting		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
MCATmalonyl-CoA-acyl ­carrier ­protein ­transacylaseFASN2C, MCT, MCT1, MT, NET62, fabD, Q8IVS2, gene:2734926literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Lipid metabolism > Type II fatty acid synthesisspinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
IBA57iron-sulfur ­cluster ­assembly ­factor ­IBA57C1orf69, MMDS3, SPG74, Q5T440, gene:20020526APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Metals and cofactors > Fe-S cluster biosynthesiscerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
PHB2prohibitin ­2BAP, BCAP37, Bap37, PNAS-141, REA, hBAP, p22, Q99623, gene:1133126literature, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS++MIMProtein import, sorting and homeostasis > Protein homeostasis > Chaperonesall 14
DAP3death ­associated ­protein ­3DAP-3, MRP-S29, MRPS29, S29mt, bMRP-10, P51398, gene:781826literature, APEX_matrix, targetP signal, yeast mito homolog++, mito protein domain+, coexpression, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
CMC2C-X9-C ­motif ­containing ­22310061C15Rik, C16orf61, DC13, Q9NRP2, gene:5694226APEX_IMS, yeast mito homolog++, mito protein domain+, induction, coexpression, MS/MS+IMSOXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors		cerebrum, cerebellum, spinalcord, liver, heart, adipose, largeintestine, stomach, placenta, testis
SDHAF1succinate ­dehydrogenase ­complex ­assembly ­factor ­1LYRM8, A6NFY7, gene:64409626literature, targetP signal+, yeast mito homolog, mito protein domain+, induction, coexpression+, MS/MSMatrixOXPHOS > Complex II > CII assembly factors;
OXPHOS > OXPHOS assembly factors		cerebrum, cerebellum, brainstem, spinalcord, kidney, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
MRPS17mitochondrial ­ribosomal ­protein ­S17HSPC011, MRP-S17, RPMS17, S17mt, Q9Y2R5, gene:5137326literature, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS+MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
ADHFE1alcohol ­dehydrogenase ­iron ­containing ­1ADH8, HMFT2263, HOT, Q8IWW8, gene:13787226targetP signal+, yeast mito homolog++, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Amino acid metabolism > GABA metabolismkidney, liver, heart, adipose, stomach, placenta
NFS1NFS1 ­cysteine ­desulfuraseHUSSY-08, IscS, NIFS, Q9Y697, gene:905426literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain+, coexpression+, MS/MS++MatrixMetabolism > Metals and cofactors > Fe-S cluster biosynthesis;
Metabolism > Metals and cofactors > Fe-S-containing proteins		all 14
NDUFAF5NADH:ubiquinone ­oxidoreductase ­complex ­assembly ­factor ­5C20orf7, MC1DN16, bA526K24.2, dJ842G6.1, Q5TEU4, gene:7913326literature, APEX_matrix, GFP, targetP signal+, Rickettsial homolog, mito protein domain+, induction, coexpression, MS/MSMIMOXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors		cerebellum, brainstem, skeletalmuscle, largeintestine, stomach, placenta
MRPL17mitochondrial ­ribosomal ­protein ­L17L17mt, LIP2, MRP-L17, MRP-L26, RPL17L, RPML26, Q9NRX2, gene:6387526literature, APEX_matrix, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
TACO1translational ­activator ­of ­cytochrome ­c ­oxidase ­ICCDC44, Q9BSH4, gene:5120426literature, APEX_matrix, targetP signal+, yeast mito homolog, Rickettsial homolog, mito protein domain+, MS/MS++MatrixMitochondrial central dogma > Translation > Translation factors;
OXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors		all 14
MFN1mitofusin ­1hfzo1, hfzo2, Q8IWA4, gene:5566926literature, yeast mito homolog++, mito protein domain+, induction, coexpression, MS/MS+MOMMitochondrial dynamics and surveillance > Fusion;
Mitochondrial dynamics and surveillance > Organelle contact sites		cerebellum, brainstem, spinalcord, kidney, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
PPTC7protein ­phosphatase ­targeting ­COQ7TA-PP2C, TAPP2C, Q8NI37, gene:16076026APEX_matrix, yeast mito homolog, mito protein domain+, induction, coexpression, MS/MS++MatrixSignalingcerebrum, cerebellum, brainstem, spinalcord, heart, skeletalmuscle, largeintestine, placenta, testis
MRPL11mitochondrial ­ribosomal ­protein ­L11CGI-113, L11MT, MRP-L11, Q9Y3B7, gene:6500326literature, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression+, MS/MS+MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
COX4I1cytochrome ­c ­oxidase ­subunit ­4I1COX IV-1, COX4, COX4-1, COXIV, COXIV-1, P13073, gene:132726literature, APEX_IMS, APEX_matrix, targetP signal, mito protein domain+, coexpression++, MS/MS++MIMOXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits		all 14
NDUFS6NADH:ubiquinone ­oxidoreductase ­subunit ­S6CI-13kA, CI-13kD-A, CI13KDA, MC1DN9, O75380, gene:472626literature, APEX_matrix, targetP signal, mito protein domain+, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		all 14
MRPL13mitochondrial ­ribosomal ­protein ­L13L13, L13A, L13mt, RPL13, RPML13, Q9BYD1, gene:2899826literature, APEX_matrix, targetP signal, yeast mito homolog, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
OXCT13-oxoacid ­CoA-transferase ­1OXCT, SCOT, P55809, gene:501926literature, APEX_matrix, targetP signal, mito protein domain, induction, coexpression++, MS/MS++MatrixMetabolism > Carbohydrate metabolism > Ketone metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
COX5Bcytochrome ­c ­oxidase ­subunit ­5BCOXVB, P10606, gene:132926literature, APEX_IMS, APEX_matrix, targetP signal+, yeast mito homolog++, mito protein domain+, MS/MS++MIMOXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits		all 14
PDK2pyruvate ­dehydrogenase ­kinase ­2PDHK2, PDKII, Q15119, gene:516426literature, APEX_matrix, yeast mito homolog+, mito protein domain+, induction, coexpression, MS/MS++MatrixMetabolism > Carbohydrate metabolism > Pyruvate metabolismall 14
ALAS15'-aminolevulinate ­synthase ­1ALAS, ALAS-H, ALAS3, ALASH, MIG4, P13196, gene:21126literature, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain+, induction, coexpression, MS/MSMatrixMetabolism > Metals and cofactors > Heme synthesis and processingliver, heart, adipose, largeintestine, placenta
MRPL33mitochondrial ­ribosomal ­protein ­L33C2orf1, L33mt, MRP-L33, RPL33L, O75394, gene:955326literature, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, stomach, placenta, testis
MTO1mitochondrial ­tRNA ­translation ­optimization ­1CGI-02, COXPD10, Q9Y2Z2, gene:2582126literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression++MatrixMitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications;
Metabolism > Vitamin metabolism > Folate and 1-C metabolism		cerebellum, spinalcord, stomach, placenta
LIASlipoic ­acid ­synthetaseHGCLAS, HUSSY-01, LAS, LIP1, LS, PDHLD, O43766, gene:1101926literature, APEX_matrix, targetP signal, yeast mito homolog, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MSMatrixMetabolism > Lipid metabolism > Lipoate insertion;
Metabolism > Metals and cofactors > Fe-S-containing proteins		brainstem, skeletalmuscle, adipose, largeintestine, placenta
NDUFA5NADH:ubiquinone ­oxidoreductase ­subunit ­A5B13, CI-13KD-B, CI-13kB, NUFM, UQOR13, Q16718, gene:469826literature, APEX_matrix, mito protein domain+, induction, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		all 14
NDUFB6NADH:ubiquinone ­oxidoreductase ­subunit ­B6B17, CI, O95139, gene:471226literature, APEX_IMS, APEX_matrix, mito protein domain+, induction, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		all 14
MTX2metaxin ­2metaxin-2, O75431, gene:1065126literature, mito protein domain+, induction, coexpression++, MS/MS++MOMProtein import, sorting and homeostasis > Protein import and sorting > SAM;
Mitochondrial dynamics and surveillance > Intramitochondrial membrane interactions		all 14
SUCLG2succinate-CoA ­ligase ­GDP-forming ­subunit ­betaG-SCS, GBETA, GTPSCS, Q96I99, gene:880126literature, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Carbohydrate metabolism > TCA cycle;
Metabolism > Carbohydrate metabolism > Itaconate metabolism;
Metabolism > Nucleotide metabolism > Nucleotide synthesis and processing		all 14
FDX1ferredoxin ­1ADX, FDX, LOH11CR1D, P10109, gene:223026literature, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS+MatrixMetabolism > Lipid metabolism > Cholesterol, bile acid, steroid synthesis;
Metabolism > Metals and cofactors > Heme synthesis and processing;
Metabolism > Metals and cofactors > Fe-S cluster biosynthesis;
Metabolism > Metals and cofactors > Fe-S-containing proteins;
Metabolism > Vitamin metabolism > Vitamin D metabolism		all 14
SLC25A1solute ­carrier ­family ­25 ­member ­1CMS23, CTP, D2L2AD, SEA, SLC20A3, P53007, gene:657626literature, yeast mito homolog++, mito protein domain+, coexpression, MS/MS++MIMMetabolism > Carbohydrate metabolism > Gluconeogenesis;
Metabolism > Carbohydrate metabolism > TCA-associated;
Metabolism > Lipid metabolism;
Small molecule transport > SLC25A family		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
MRPS28mitochondrial ­ribosomal ­protein ­S28COXPD47, HSPC007, MRP-S28, MRP-S35, MRPS35, Q9Y2Q9, gene:2895726literature, targetP signal, mito protein domain+, coexpression++, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
TIMM50translocase ­of ­inner ­mitochondrial ­membrane ­50MGCA9, TIM50, TIM50L, Q3ZCQ8, gene:9260926literature, APEX_IMS, yeast mito homolog++, mito protein domain, coexpression++, MS/MS++MIMProtein import, sorting and homeostasis > Protein import and sorting > TIM23 presequence pathwayall 14
VDAC2voltage ­dependent ­anion ­channel ­2POR, P45880, gene:741726literature, APEX_IMS, yeast mito homolog+, mito protein domain+, induction, coexpression, MS/MS++MOMSmall molecule transportall 14
SLC25A42solute ­carrier ­family ­25 ­member ­42MECREN, Q86VD7, gene:28443926literature, yeast mito homolog+, mito protein domain+, induction, coexpression, MS/MS++MIMMetabolism > Metals and cofactors > Coenzyme A metabolism;
Small molecule transport > SLC25A family		all 14
TOMM7translocase ­of ­outer ­mitochondrial ­membrane ­7TOM7, Q9P0U1, gene:5454326literature, yeast mito homolog++, mito protein domain+, coexpression, MS/MS++MOMProtein import, sorting and homeostasis > Protein import and sorting > TOM;
Mitochondrial dynamics and surveillance > Mitophagy;
Mitochondrial dynamics and surveillance > Autophagy		cerebrum, cerebellum, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, stomach, placenta, testis
ECH1enoyl-CoA ­hydratase ­1HPXEL, Q13011, gene:189126APEX_matrix, targetP signal+, yeast mito homolog+, mito protein domain, coexpression+, MS/MS++MatrixMetabolism > Lipid metabolismall 14
GATBglutamyl-tRNA ­amidotransferase ­subunit ­BCOXPD41, HSPC199, PET112, PET112L, O75879, gene:518825literature, APEX_matrix, targetP signal, yeast mito homolog, Rickettsial homolog, mito protein domain+, coexpression+, MS/MSMatrixMitochondrial central dogma > Translation > mt-tRNA synthetasescerebrum, skeletalmuscle, adipose, stomach, testis
BCS1LBCS1 ­homolog, ­ubiquinol-cytochrome ­c ­reductase ­complex ­chaperoneBCS, BCS1, BJS, FLNMS, GRACILE, Hs.6719, MC3DN1, PTD, h-BCS, h-BCS1, Q9Y276, gene:61725literature, APEX_matrix, yeast mito homolog++, Rickettsial homolog, mito protein domain+, coexpression+, MS/MS+MIMOXPHOS > Complex III > CIII assembly factors;
OXPHOS > OXPHOS assembly factors		cerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
ERAL1Era ­like ­12S ­mitochondrial ­rRNA ­chaperone ­1CEGA, ERA, ERA-W, ERAL1A, ERAL1B, H-ERA, HERA-A, HERA-B, PRLTS6, O75616, gene:2628425APEX_matrix, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain, induction, coexpression++MatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly		none
CMC1C-X9-C ­motif ­containing ­1C3orf68, Q7Z7K0, gene:15210025APEX_IMS, GFP, yeast mito homolog++, mito protein domain+, induction, coexpression, MS/MS+IMSOXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
MRPL28mitochondrial ­ribosomal ­protein ­L28MAAT1, p15, Q13084, gene:1057325literature, APEX_matrix, yeast mito homolog++, Rickettsial homolog, coexpression, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, brainstem, spinalcord, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
TSFMTs ­translation ­elongation ­factor, ­mitochondrialEFTS, EFTSMT, P43897, gene:1010225literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS++MatrixMitochondrial central dogma > Translation > Translation factorscerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
FXNfrataxinCyaY, FA, FARR, FRDA, X25, Q16595, gene:239525literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MS+MatrixMetabolism > Metals and cofactors > Fe-S cluster biosynthesisall 14
NFU1NFU1 ­iron-sulfur ­cluster ­scaffoldCGI-33, HIRIP, HIRIP5, MMDS1, NIFUC, Nfu, NifU, Q9UMS0, gene:2724725APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MS+MatrixMetabolism > Metals and cofactors > Fe-S cluster biosynthesis;
Metabolism > Metals and cofactors > Fe-S-containing proteins		all 14
YARS2tyrosyl-tRNA ­synthetase ­2CGI-04, MLASA2, MT-TYRRS, TYRRS, Q9Y2Z4, gene:5106725literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MS+MatrixMitochondrial central dogma > Translation > mt-tRNA synthetasesbrainstem, spinalcord, kidney, adipose, smallintestine, largeintestine, stomach, placenta, testis
MRPL21mitochondrial ­ribosomal ­protein ­L21L21mt, MRP-L21, Q7Z2W9, gene:21992725literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MS+MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
ALDH4A1aldehyde ­dehydrogenase ­4 ­family ­member ­A1ALDH4, P5CD, P5CDh, P30038, gene:865925literature, APEX_matrix, targetP signal+, yeast mito homolog++, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Amino acid metabolism > Proline metabolism;
Metabolism > Amino acid metabolism > Glyoxylate metabolism		all 14
TOMM40Ltranslocase ­of ­outer ­mitochondrial ­membrane ­40 ­likeTOMM40B, Q969M1, gene:8413425targetP signal, yeast mito homolog+, mito protein domain+, induction, coexpression+, MS/MSMOMProtein import, sorting and homeostasis > Protein import and sorting > TOMcerebrum, cerebellum, brainstem, spinalcord, adipose, largeintestine, stomach, testis
NDUFAF1NADH:ubiquinone ­oxidoreductase ­complex ­assembly ­factor ­1CGI-65, CGI65, CIA30, MC1DN11, Q9Y375, gene:5110325literature, APEX_matrix, targetP signal, mito protein domain, induction, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors		cerebellum, brainstem, spinalcord, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
NDUFB10NADH:ubiquinone ­oxidoreductase ­subunit ­B10PDSW, O96000, gene:471625literature, APEX_matrix, mito protein domain+, induction, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		all 14
ACADSBacyl-CoA ­dehydrogenase ­short/branched ­chain2-MEBCAD, ACAD7, SBCAD, P45954, gene:3625literature, APEX_matrix, targetP signal, mito protein domain+, induction, coexpression, MS/MS++MatrixMetabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Amino acid metabolism > BCAA metabolism		all 14
MRPS9mitochondrial ­ribosomal ­protein ­S9MRP-S9, RPMS9, S9mt, P82933, gene:6496525literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MSMatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > Translation > Mitochondrial ribosome		all 14
COQ8Acoenzyme ­Q8AADCK3, ARCA2, CABC1, COQ10D4, COQ8, SCAR9, Q8NI60, gene:5699725literature, APEX_matrix, yeast mito homolog+, Rickettsial homolog, mito protein domain, induction, coexpression+, MS/MS++MIMMetabolism > Metals and cofactors > Coenzyme Q metabolismall 14
ACADVLacyl-CoA ­dehydrogenase ­very ­long ­chainACAD6, LCACD, VLCAD, P49748, gene:3725literature, APEX_matrix, targetP signal+, mito protein domain+, coexpression+, MS/MS++MIMMetabolism > Lipid metabolism > Fatty acid oxidationall 14
COQ4coenzyme ­Q4CGI-92, COQ10D7, Q9Y3A0, gene:5111725literature, targetP signal+, yeast mito homolog++, mito protein domain+, coexpression, MS/MSMIMMetabolism > Metals and cofactors > Coenzyme Q metabolismcerebrum, cerebellum, brainstem, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta
MRPL15mitochondrial ­ribosomal ­protein ­L15HSPC145, L15mt, MRP-L15, MRP-L7, RPML7, Q9P015, gene:2908825literature, APEX_matrix, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
NDUFA2NADH:ubiquinone ­oxidoreductase ­subunit ­A2B8, CD14, CIB8, MC1DN13, O43678, gene:469525literature, APEX_matrix, targetP signal, mito protein domain, induction, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		all 14
MRPL24mitochondrial ­ribosomal ­protein ­L24L24mt, MRP-L18, MRP-L24, Q96A35, gene:7959025literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
HSPD1heat ­shock ­protein ­family ­D ­(Hsp60) ­member ­1CPN60, GROEL, HLD4, HSP-60, HSP60, HSP65, HuCHA60, SPG13, P10809, gene:332925literature, APEX_matrix, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain, MS/MS++MatrixProtein import, sorting and homeostasis > Protein homeostasis > Chaperonesall 14
NDUFS5NADH:ubiquinone ­oxidoreductase ­subunit ­S5CI-15k, CI15K, O43920, gene:472525literature, APEX_IMS, mito protein domain+, induction, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		all 14
AK3adenylate ­kinase ­3AK3L1, AK6, AKL3L, AKL3L1, FIX, Q9UIJ7, gene:5080825literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS++MatrixMetabolism > Nucleotide metabolism > Nucleotide synthesis and processingall 14
CYCScytochrome ­c, ­somaticCYC, HCS, THC4, P99999, gene:5420524literature, APEX_IMS, yeast mito homolog++, Rickettsial homolog, mito protein domain+, MS/MS++IMSMetabolism > Metals and cofactors > Heme-containing proteins;
Metabolism > Electron carriers > Cytochromes;
OXPHOS > OXPHOS subunits;
OXPHOS > Cytochrome C;
Mitochondrial dynamics and surveillance > Apoptosis		all 14
MIPEPmitochondrial ­intermediate ­peptidaseCOXPD31, HMIP, MIP, Q99797, gene:428524literature, APEX_matrix, targetP signal+, yeast mito homolog++, mito protein domain, induction, MS/MS++MatrixProtein import, sorting and homeostasis > Protein import and sorting > Preprotein cleavage;
Protein import, sorting and homeostasis > Protein homeostasis > Proteases		cerebrum, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
LYRM7LYR ­motif ­containing ­7C5orf31, MC3DN8, MZM1L, Q5U5X0, gene:9062424literature, APEX_matrix, targetP signal, yeast mito homolog++, mito protein domain+, MS/MS++MatrixOXPHOS > Complex III > CIII assembly factors;
OXPHOS > OXPHOS assembly factors		cerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
CRATcarnitine ­O-acetyltransferaseCAT, CAT1, NBIA8, P43155, gene:138424literature, APEX_matrix, targetP signal, yeast mito homolog+, mito protein domain, induction, coexpression, MS/MS++MIMMetabolism > Lipid metabolism > Fatty acid oxidationall 14
PCCBpropionyl-CoA ­carboxylase ­subunit ­betaP05166, gene:509624literature, targetP signal+, Rickettsial homolog, mito protein domain, coexpression+, MS/MS++MatrixMetabolism > Carbohydrate metabolism > Propanoate metabolism;
Metabolism > Lipid metabolism > Fatty acid oxidation		all 14
MRPS7mitochondrial ­ribosomal ­protein ­S7COXPD34, MRP-S, MRP-S7, RP-S7, RPMS7, S7mt, bMRP27a, Q9Y2R9, gene:5108124literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression+, MS/MSMatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > Translation > Mitochondrial ribosome		all 14
MRPL3mitochondrial ­ribosomal ­protein ­L3COXPD9, MRL3, RPML3, P09001, gene:1122224literature, APEX_matrix, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpressionMatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, placenta, testis
PRODHproline ­dehydrogenase ­1HSPOX2, PIG6, POX, PRODH1, PRODH2, TP53I6, O43272, gene:562524literature, targetP signal, yeast mito homolog+, mito protein domain+, coexpression, MS/MS++MIMMetabolism > Amino acid metabolism > Proline metabolism;
Metabolism > Electron carriers > Q-linked reactions, other		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
PCCApropionyl-CoA ­carboxylase ­subunit ­alphaP05165, gene:509524literature, targetP signal, Rickettsial homolog, mito protein domain, induction, coexpression+, MS/MS++MatrixMetabolism > Carbohydrate metabolism > Propanoate metabolism;
Metabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Vitamin metabolism > Biotin utilizing proteins		all 14
MCCC1methylcrotonoyl-CoA ­carboxylase ­1MCC-B, MCCA, Q96RQ3, gene:5692224literature, targetP signal, Rickettsial homolog, mito protein domain, induction, coexpression+, MS/MS++MatrixMetabolism > Amino acid metabolism > BCAA metabolism;
Metabolism > Vitamin metabolism > Biotin utilizing proteins		all 14
MRPS12mitochondrial ­ribosomal ­protein ­S12MPR-S12, MT-RPS12, RPMS12, RPS12, RPSM12, O15235, gene:618324literature, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression+, MS/MS+MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, brainstem, spinalcord, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
CLPBcaseinolytic ­mitochondrial ­matrix ­peptidase ­chaperone ­subunit ­BANKCLB, HSP78, MEGCANN, MGCA7, SKD3, Q9H078, gene:8157024APEX_IMS, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain, MS/MS++IMSProtein import, sorting and homeostasis > Protein homeostasis > Proteasescerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
PDK1pyruvate ­dehydrogenase ­kinase ­1Q15118, gene:516324literature, APEX_matrix, targetP signal+, yeast mito homolog+, mito protein domain+, MS/MS++MatrixMetabolism > Carbohydrate metabolism > Pyruvate metabolismall 14
MRPL49mitochondrial ­ribosomal ­protein ­L49C11orf4, L49mt, MRP-L49, NOF, NOF1, Q13405, gene:74024literature, APEX_matrix, targetP signal, yeast mito homolog++, mito protein domain, coexpression, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
COX7A2cytochrome ­c ­oxidase ­subunit ­7A2COX7AL, COX7AL1, COXVIIAL, COXVIIa-L, VIIAL, P14406, gene:134724literature, mito protein domain+, induction, coexpression++, MS/MS++MIMOXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits		all 14
TMEM126Atransmembrane ­protein ­126AOPA7, Q9H061, gene:8423324literature, mito protein domain+, induction, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors		all 14
ECHDC2enoyl-CoA ­hydratase ­domain ­containing ­2Q86YB7, gene:5526824targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpression, MS/MS++Matrixnoneall 14
HCCSholocytochrome ­c ­synthaseCCHL, LSDMCA1, MCOPS7, MLS, P53701, gene:305224literature, APEX_IMS, yeast mito homolog++, mito protein domain+, induction, coexpression, MS/MSIMSMetabolism > Metals and cofactors > Heme synthesis and processing;
OXPHOS > OXPHOS subunits;
OXPHOS > Cytochrome C		cerebrum, spinalcord, heart, adipose, smallintestine, largeintestine, placenta, testis
HIBADH3-hydroxyisobutyrate ­dehydrogenaseNS5ATP1, P31937, gene:1111224literature, APEX_matrix, targetP signal+, mito protein domain, induction, coexpression+, MS/MS++MatrixMetabolism > Amino acid metabolism > BCAA metabolismall 14
MRPL19mitochondrial ­ribosomal ­protein ­L19L19mt, MRP-L15, MRP-L19, MRPL15, RLX1, RPML15, P49406, gene:980124literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MS+MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, brainstem, spinalcord, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
MRPL36mitochondrial ­ribosomal ­protein ­L36BRIP1, L36mt, MRP-L36, PRPL36, RPMJ, Q9P0J6, gene:6497924literature, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression+MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomenone
SLC25A30solute ­carrier ­family ­25 ­member ­30KMCP1, Q5SVS4, gene:25351224literature, yeast mito homolog+, mito protein domain+, coexpression, MS/MS++MIMMetabolism > Sulfur metabolism;
Small molecule transport > SLC25A family		cerebrum, cerebellum, liver, placenta, testis
BDH13-hydroxybutyrate ­dehydrogenase ­1BDH, SDR9C1, Q02338, gene:62224literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS++MIMMetabolism > Carbohydrate metabolism > Ketone metabolismall 14
FARS2phenylalanyl-tRNA ­synthetase ­2, ­mitochondrialCOXPD14, FARS1, HSPC320, PheRS, SPG77, mtPheRS, O95363, gene:1066723literature, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MSMatrixMitochondrial central dogma > Translation > mt-tRNA synthetasesadipose, largeintestine, stomach, placenta, testis
ABCB7ATP ­binding ­cassette ­subfamily ­B ­member ­7ABC7, ASAT, Atm1p, EST140535, O75027, gene:2223literature, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpression+, MS/MS+MIMMetabolism > Metals and cofactors > Heme synthesis and processing;
Metabolism > Metals and cofactors > Fe-S cluster biosynthesis;
Metabolism > Metals and cofactors > Molybdenum cofactor synthesis and proteins;
Small molecule transport > ABC transporters		all 14
MTX1metaxin ­1MTX, MTXN, Q13505, gene:458023literature, yeast mito homolog++, mito protein domain+, coexpression, MS/MS++MOMProtein import, sorting and homeostasis > Protein import and sorting > SAM;
Mitochondrial dynamics and surveillance > Intramitochondrial membrane interactions		all 14
NDUFA7NADH:ubiquinone ­oxidoreductase ­subunit ­A7B14.5a, CI-B14.5a, O95182, gene:470123literature, APEX_matrix, mito protein domain+, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		all 14
TIMM17Atranslocase ­of ­inner ­mitochondrial ­membrane ­17ATIM17, TIM17A, Q99595, gene:1044023literature, APEX_IMS, yeast mito homolog+, mito protein domain+, induction, coexpression, MS/MS+MIMProtein import, sorting and homeostasis > Protein import and sorting > TIM23 presequence pathwayall 14
ALDH9A1aldehyde ­dehydrogenase ­9 ­family ­member ­A1ALDH4, ALDH7, ALDH9, E3, TMABA-DH, TMABADH, TMABALDH, P49189, gene:22323APEX_matrix, targetP signal+, yeast mito homolog+, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Amino acid metabolism > GABA metabolism;
Metabolism > Detoxification > ROS and glutathione metabolism		brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
MRPS18Cmitochondrial ­ribosomal ­protein ­S18CCGI-134, MRP-S18-1, MRP-S18-c, MRPS18-1, S18mt-c, mrps18-c, Q9Y3D5, gene:5102323literature, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
MARS2methionyl-tRNA ­synthetase ­2, ­mitochondrialCOXPD25, MetRS, mtMetRS, Q96GW9, gene:9293523literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MS+MatrixMitochondrial central dogma > Translation > mt-tRNA synthetasescerebrum, cerebellum, brainstem, spinalcord, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
ALDH6A1aldehyde ­dehydrogenase ­6 ­family ­member ­A1MMSADHA, MMSDH, Q02252, gene:432923literature, APEX_matrix, targetP signal, yeast mito homolog+, mito protein domain, coexpression, MS/MS++MIMMetabolism > Amino acid metabolism > BCAA metabolismall 14
FDXRferredoxin ­reductaseADR, ADXR, ANOA, P22570, gene:223223literature, APEX_matrix, targetP signal+, yeast mito homolog++, coexpression, MS/MS++MatrixMetabolism > Lipid metabolism > Cholesterol, bile acid, steroid synthesis;
Metabolism > Metals and cofactors > Heme synthesis and processing;
Metabolism > Metals and cofactors > Fe-S cluster biosynthesis;
Metabolism > Vitamin metabolism > Vitamin D metabolism		spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
GATCglutamyl-tRNA ­amidotransferase ­subunit ­C15E1.2, COXPD42, O43716, gene:28345923literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain+, induction, coexpression, MS/MSMatrixMitochondrial central dogma > Translation > mt-tRNA synthetasescerebrum, cerebellum, spinalcord, kidney, largeintestine, testis
TRAP1TNF ­receptor ­associated ­protein ­1HSP 75, HSP75, HSP90L, TRAP-1, Q12931, gene:1013123APEX_matrix, GFP, targetP signal+, Rickettsial homolog, mito protein domain, coexpression++, MS/MS+MatrixProtein import, sorting and homeostasis > Protein homeostasis > Chaperonesall 14
ACAD8acyl-CoA ­dehydrogenase ­family ­member ­8ACAD-8, ARC42, IBDH, Q9UKU7, gene:2703422literature, APEX_matrix, targetP signal+, mito protein domain+, coexpression, MS/MS++MatrixMetabolism > Amino acid metabolism > BCAA metabolismall 14
ALDH2aldehyde ­dehydrogenase ­2 ­family ­memberALDH-E2, ALDHI, ALDM, P05091, gene:21722literature, APEX_matrix, targetP signal+, yeast mito homolog+, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Detoxification > Xenobiotic metabolismall 14
PPIFpeptidylprolyl ­isomerase ­FCYP3, CyP-M, Cyp-D, CypD, P30405, gene:1010522literature, APEX_matrix, targetP signal+, mito protein domain, induction, coexpression, MS/MS++MatrixSignaling > Calcium homeostasis > Mitochondrial permeability transition poreall 14
TIMM22translocase ­of ­inner ­mitochondrial ­membrane ­22COXPD43, TEX4, TIM22, Q9Y584, gene:2992822literature, yeast mito homolog++, mito protein domain+, induction, coexpression, MS/MSMIMProtein import, sorting and homeostasis > Protein import and sorting > TIM22 carrier pathwayall 14
IVDisovaleryl-CoA ­dehydrogenaseACAD2, P26440, gene:371222literature, APEX_matrix, targetP signal, mito protein domain+, coexpression+, MS/MS++MatrixMetabolism > Amino acid metabolism > BCAA metabolismall 14
L2HGDHL-2-hydroxyglutarate ­dehydrogenaseC14orf160, L2HGA, Q9H9P8, gene:7994422literature, APEX_matrix, targetP signal+, mito protein domain, induction, coexpression, MS/MS++MIMMetabolism > Carbohydrate metabolism > TCA-associatedcerebrum, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, testis
ETHE1ETHE1 ­persulfide ­dioxygenaseHSCO, YF13H12, O95571, gene:2347422literature, APEX_matrix, targetP signal, yeast mito homolog+, mito protein domain, induction, MS/MS++MatrixMetabolism > Sulfur metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta
MRPL20mitochondrial ­ribosomal ­protein ­L20L20mt, MRP-L20, Q9BYC9, gene:5505222literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
SLC25A5solute ­carrier ­family ­25 ­member ­52F1, AAC2, ANT2, T2, T3, P05141, gene:29222literature, yeast mito homolog+, mito protein domain+, induction, MS/MS++MIMMetabolism > Nucleotide metabolism > Nucleotide import;
Signaling > Calcium homeostasis > Mitochondrial permeability transition pore;
Small molecule transport > SLC25A family		all 14
SLC25A12solute ­carrier ­family ­25 ­member ­12AGC1, ARALAR, EIEE39, O75746, gene:860422literature, yeast mito homolog+, mito protein domain+, induction, MS/MS++MIMMetabolism > Carbohydrate metabolism > Malate-aspartate shuttle;
Metabolism > Amino acid metabolism > Glutamate metabolism;
Signaling > Calcium homeostasis > EF hand proteins;
Small molecule transport > SLC25A family		all 14
MRPS21mitochondrial ­ribosomal ­protein ­S21MDS016, MRP-S21, RPMS21, P82921, gene:5446022literature, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
TOMM22translocase ­of ­outer ­mitochondrial ­membrane ­221C9-2, MST065, MSTP065, TOM22, Q9NS69, gene:5699322literature, yeast mito homolog++, mito protein domain+, coexpression, MS/MS++MOMProtein import, sorting and homeostasis > Protein import and sorting > TOMall 14
ACAA2acetyl-CoA ­acyltransferase ­2DSAEC, P42765, gene:1044922literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, induction, coexpression+, MS/MS++MatrixMetabolism > Carbohydrate metabolism > Ketone metabolism;
Metabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Amino acid metabolism > Lysine metabolism		all 14
MRPL30mitochondrial ­ribosomal ­protein ­L30L28MT, L30MT, MRP-L28, MRP-L30, MRPL28, MRPL28M, RPML28, Q8TCC3, gene:5126322literature, APEX_matrix, yeast mito homolog++, mito protein domain, coexpression++, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
DNAJA3DnaJ ­heat ­shock ­protein ­family ­(Hsp40) ­member ­A3HCA57, TID1, hTID-1, Q96EY1, gene:909322literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS+MatrixProtein import, sorting and homeostasis > Protein homeostasis > Chaperonesall 14
NDUFB2NADH:ubiquinone ­oxidoreductase ­subunit ­B2AGGG, CI-AGGG, O95178, gene:470822literature, targetP signal, induction, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		all 14
MRPS34mitochondrial ­ribosomal ­protein ­S34COXPD32, MRP-S12, MRP-S34, MRPS12, P82930, gene:6599322literature, APEX_matrix, targetP signal, induction, coexpression++, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
ETFBelectron ­transfer ­flavoprotein ­subunit ­betaFP585, MADD, P38117, gene:210922literature, APEX_matrix, yeast mito homolog, mito protein domain, induction, coexpression+, MS/MS++MatrixMetabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Amino acid metabolism > BCAA metabolism;
Metabolism > Amino acid metabolism > Lysine metabolism;
Metabolism > Amino acid metabolism > Glycine metabolism;
Metabolism > Vitamin metabolism > Choline and betaine metabolism;
Metabolism > Electron carriers > Q-linked reactions, other		all 14
AFG1LAFG1 ­like ­ATPaseAFG1, LACE1, c222389, Q8WV93, gene:24626922APEX_matrix, yeast mito homolog++, Rickettsial homolog, coexpression, MS/MS+MIMProtein import, sorting and homeostasis > Protein homeostasis > Proteasescerebrum, cerebellum, spinalcord, heart, adipose, smallintestine, largeintestine, stomach, placenta
ATP5IF1ATP ­synthase ­inhibitory ­factor ­subunit ­1ATPI, ATPIF1, ATPIP, IP, Q9UII2, gene:9397422literature, APEX_matrix, targetP signal, yeast mito homolog++, mito protein domain+, MS/MS++MatrixOXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits		all 14
ATP5PBATP ­synthase ­peripheral ­stalk-membrane ­subunit ­bATP5F1, PIG47, P24539, gene:51522literature, APEX_matrix, yeast mito homolog++, mito protein domain+, MS/MS++MIMOXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits		all 14
COX7Ccytochrome ­c ­oxidase ­subunit ­7CP15954, gene:135022literature, APEX_IMS, yeast mito homolog++, mito protein domain+, MS/MS++MIMOXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits		all 14
CHCHD3coiled-coil-helix-coiled-coil-helix ­domain ­containing ­3MICOS19, MINOS3, Mic19, PPP1R22, Q9NX63, gene:5492722literature, APEX_IMS, APEX_matrix, GFP, yeast mito homolog, induction, coexpression++, MS/MS++MIMMitochondrial dynamics and surveillance > Cristae formation > MICOS complex;
Mitochondrial dynamics and surveillance > Intramitochondrial membrane interactions		all 14
COQ10Bcoenzyme ­Q10BQ9H8M1, gene:8021922GFP, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain+, MS/MS+MIMMetabolism > Metals and cofactors > Coenzyme Q metabolismcerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta
ACSF3acyl-CoA ­synthetase ­family ­member ­3Q4G176, gene:19732222APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, coexpression+, MS/MS++MatrixMetabolism > Lipid metabolism > Type II fatty acid synthesisall 14
POLDIP2DNA ­polymerase ­delta ­interacting ­protein ­2PDIP38, POLD4, p38, Q9Y2S7, gene:2607322literature, APEX_matrix, targetP signal+, induction, coexpression++, MS/MS++MatrixMitochondrial central dogma > mtDNA maintenance > mtDNA nucleoidall 14
SLC25A10solute ­carrier ­family ­25 ­member ­10DIC, MTDPS19, Q9UBX3, gene:146822literature, yeast mito homolog+, mito protein domain+, coexpression, MS/MS++MIMMetabolism > Carbohydrate metabolism > Gluconeogenesis;
Metabolism > Carbohydrate metabolism > Malate-aspartate shuttle;
Small molecule transport > SLC25A family		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
SLC25A13solute ­carrier ­family ­25 ­member ­13ARALAR2, CITRIN, CTLN2, NICCD, Q9UJS0, gene:1016522literature, yeast mito homolog+, mito protein domain+, coexpression, MS/MS++MIMMetabolism > Carbohydrate metabolism > Malate-aspartate shuttle;
Metabolism > Amino acid metabolism > Urea cycle;
Signaling > Calcium homeostasis > EF hand proteins;
Small molecule transport > SLC25A family		all 14
PDK3pyruvate ­dehydrogenase ­kinase ­3CMTX6, GS1-358P8.4, Q15120, gene:516521literature, APEX_matrix, targetP signal, yeast mito homolog+, mito protein domain+, MS/MS++MatrixMetabolism > Carbohydrate metabolism > Pyruvate metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
ME3malic ­enzyme ­3NADP-ME, Q16798, gene:1087321literature, yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Carbohydrate metabolism > TCA-associatedcerebrum, cerebellum, brainstem, spinalcord, kidney, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
MRPL22mitochondrial ­ribosomal ­protein ­L22HSPC158, L22mt, MRP-L22, MRP-L25, RPML25, Q9NWU5, gene:2909321literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
IDH2isocitrate ­dehydrogenase ­(NADP(+)) ­2D2HGA2, ICD-M, IDH, IDHM, IDP, IDPM, mNADP-IDH, P48735, gene:341821literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS++MatrixMetabolism > Carbohydrate metabolism > TCA cycleall 14
GCDHglutaryl-CoA ­dehydrogenaseACAD5, GCD, Q92947, gene:263921literature, APEX_matrix, targetP signal+, mito protein domain+, coexpression, MS/MS++MatrixMetabolism > Amino acid metabolism > Lysine metabolismall 14
MRPL47mitochondrial ­ribosomal ­protein ­L47CGI-204, L47mt, MRP-L47, NCM1, Q9HD33, gene:5712921literature, APEX_matrix, targetP signal+, yeast mito homolog++, mito protein domain, induction, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > Translation > Mitochondrial ribosome		all 14
PPA2inorganic ­pyrophosphatase ­2HSPC124, SCFAI, SCFI, SID6-306, Q9H2U2, gene:2706821literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMitochondrial central dogma > mtDNA maintenance;
Mitochondrial central dogma > mtRNA metabolism;
Mitochondrial central dogma > Translation;
Metabolism > Nucleotide metabolism		all 14
MRPL9mitochondrial ­ribosomal ­protein ­L9L9mt, Q9BYD2, gene:6500521literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, induction, coexpression+, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
CHCHD10coiled-coil-helix-coiled-coil-helix ­domain ­containing ­10C22orf16, FTDALS2, IMMD, MIX17A, N27C7-4, SMAJ, Q8WYQ3, gene:40091621literature, targetP signal, yeast mito homolog+, mito protein domain, induction, coexpression++IMSnonenone
WARS2tryptophanyl ­tRNA ­synthetase ­2, ­mitochondrialNEMMLAS, TrpRS, mtTrpRS, Q9UGM6, gene:1035221literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MSMatrixMitochondrial central dogma > Translation > mt-tRNA synthetasescerebrum, cerebellum, brainstem, kidney, skeletalmuscle, adipose, largeintestine, placenta, testis
SLC25A19solute ­carrier ­family ­25 ­member ­19DNC, MCPHA, MUP1, THMD3, THMD4, TPC, Q9HC21, gene:6038621literature, yeast mito homolog++, mito protein domain+, induction, coexpression, MS/MSMIMMetabolism > Vitamin metabolism > Vitamin B1 metabolism;
Small molecule transport > SLC25A family		cerebellum, spinalcord, adipose, testis
CBR4carbonyl ­reductase ­4SDR45C1, Q8N4T8, gene:8486921literature, APEX_matrix, Rickettsial homolog, mito protein domain, induction, coexpression+, MS/MS++MatrixMetabolism > Lipid metabolism > Type II fatty acid synthesisall 14
SMDT1single-pass ­membrane ­protein ­with ­aspartate ­rich ­tail ­1C22orf32, DDDD, EMRE, Q9H4I9, gene:9168921literature, targetP signal, mito protein domain+, induction, coexpression+, MS/MSMIMSignaling > Calcium homeostasis > Calcium cycle;
Small molecule transport > Calcium uniporter		cerebrum, cerebellum, brainstem, spinalcord, kidney, adipose, smallintestine, largeintestine, stomach, placenta, testis
HAGHhydroxyacylglutathione ­hydrolaseGLO2, GLX2, GLXII, HAGH1, Q16775, gene:302921literature, APEX_matrix, targetP signal+, mito protein domain, induction, coexpression, MS/MS++MatrixMetabolism > Detoxification > ROS and glutathione metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
COX7A1cytochrome ­c ­oxidase ­subunit ­7A1COX7A, COX7AH, COX7AM, P24310, gene:134621literature, targetP signal, mito protein domain+, induction, coexpression, MS/MS+MIMOXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits		cerebrum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
MTG1mitochondrial ­ribosome ­associated ­GTPase ­1GTP, GTPBP7, Q9BT17, gene:9217021literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression+, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosome assemblylargeintestine, testis
COX14cytochrome ­c ­oxidase ­assembly ­factor ­COX14C12orf62, PCAG1, Q96I36, gene:8498721literature, GFP, mito protein domain+, induction, coexpression++, MS/MS+MIMMitochondrial central dogma > Translation > Translation factors;
OXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors		cerebrum, cerebellum, brainstem, spinalcord, kidney, heart, smallintestine, testis
NDUFA12NADH:ubiquinone ­oxidoreductase ­subunit ­A12B17.2, DAP13, MC1DN23, Q9UI09, gene:5596721literature, APEX_matrix, Rickettsial homolog, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		all 14
MRPS16mitochondrial ­ribosomal ­protein ­S16CGI-132, COXPD2, MRP-S16, RPMS16, Q9Y3D3, gene:5102121literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, MS/MS+MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
MTERF2mitochondrial ­transcription ­termination ­factor ­2MTERFD3, mTERFL, Q49AM1, gene:8029821literature, APEX_matrix, targetP signal+, mito protein domain+, induction, coexpression, MS/MSMatrixMitochondrial central dogma > mtDNA maintenance > mtDNA nucleoidcerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, largeintestine, testis
ETFRF1electron ­transfer ­flavoprotein ­regulatory ­factor ­1LYRM5, Q6IPR1, gene:14436321mito protein domain+, induction, coexpression, MS/MS++MatrixMetabolism > Electron carriers > Q-linked reactions, othercerebrum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta
GPD2glycerol-3-phosphate ­dehydrogenase ­2GDH2, GPDM, mGDH, mGPDH, P43304, gene:282021literature, APEX_IMS, yeast mito homolog++, mito protein domain, coexpression, MS/MS++MIMMetabolism > Carbohydrate metabolism > Glycerol phosphate shuttle;
Metabolism > Electron carriers > Q-linked reactions, other		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
NDUFA10NADH:ubiquinone ­oxidoreductase ­subunit ­A10CI-42KD, CI-42k, MC1DN22, O95299, gene:470521literature, targetP signal, mito protein domain, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		all 14
NDUFC2NADH:ubiquinone ­oxidoreductase ­subunit ­C2B14.5b, CI-B14.5b, HLC-1, NADHDH2, O95298, gene:471821literature, APEX_IMS, mito protein domain+, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		all 14
SLIRPSRA ­stem-loop ­interacting ­RNA ­binding ­proteinC14orf156, DC50, PD04872, Q9GZT3, gene:8189221literature, APEX_matrix, GFP, targetP signal+, mito protein domain, induction, coexpression, MS/MS++MatrixMitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay;
Mitochondrial central dogma > Translation		all 14
MT-ATP6ATP ­synthase ­F0 ­subunit ­6ATPase6, MTATP6, P00846, gene:450821literature, yeast mito homolog++, Rickettsial homolog, mito protein domain+, MS/MS+MIMOXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits		all 14
MT-CO2cytochrome ­c ­oxidase ­subunit ­IICOII, MTCO2, P00403, gene:451321literature, APEX_IMS, APEX_matrix, yeast mito homolog++, Rickettsial homolog, mito protein domain+, MS/MS+MIMOXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits		all 14
TIMM21translocase ­of ­inner ­mitochondrial ­membrane ­21C18orf55, HSPC154, TIM21, Q9BVV7, gene:2909021APEX_IMS, GFP, yeast mito homolog++, mito protein domain+, coexpression, MS/MSMIMMitochondrial central dogma > Translation > Translation factors;
Protein import, sorting and homeostasis > Protein import and sorting > TIM23 presequence pathway;
OXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors		cerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
NDUFA8NADH:ubiquinone ­oxidoreductase ­subunit ­A8CI-19KD, CI-PGIV, PGIV, P51970, gene:470221literature, APEX_IMS, mito protein domain, induction, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		all 14
GLSglutaminaseAAD20, CASGID, EIEE71, GAC, GAM, GDPAG, GLS1, KGA, O94925, gene:274421literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain+, MS/MS++MIMMetabolism > Amino acid metabolism > Glutamate metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
NDUFB11NADH:ubiquinone ­oxidoreductase ­subunit ­B11CI-ESSS, ESSS, MC1DN30, NP17.3, Np15, P17.3, Q9NX14, gene:5453920literature, APEX_IMS, APEX_matrix, targetP signal+, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		all 14
DHTKD1dehydrogenase ­E1 ­and ­transketolase ­domain ­containing ­1AMOXAD, CMT2Q, Q96HY7, gene:5552620APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain+, MS/MSMatrixMetabolism > Amino acid metabolism > Lysine metabolismcerebrum, kidney, liver, adipose, placenta, testis
FAHD2Afumarylacetoacetate ­hydrolase ­domain ­containing ­2ACGI-105, Q96GK7, gene:5101120APEX_matrix, targetP signal, mito protein domain, induction, coexpression, MS/MS++Matrixnoneall 14
HSD17B8hydroxysteroid ­17-beta ­dehydrogenase ­8D6S2245E, FABG, FABGL, H2-KE6, HKE6, KE6, RING2, SDR30C1, dJ1033B10.9, Q92506, gene:792320APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Lipid metabolism > Type II fatty acid synthesis;
Metabolism > Lipid metabolism > Cholesterol, bile acid, steroid synthesis		all 14
HINT2histidine ­triad ­nucleotide ­binding ­protein ­2HIT-17, Q9BX68, gene:8468120APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Lipid metabolism > Cholesterol, bile acid, steroid synthesisall 14
MRPS5mitochondrial ­ribosomal ­protein ­S5MRP-S5, S5mt, P82675, gene:6496920literature, APEX_matrix, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
C6orf136chromosome ­6 ­open ­reading ­frame ­136Q5SQH8, gene:22154520targetP signal, induction, coexpression++, MS/MS+Matrixnoneall 14
SPRYD4SPRY ­domain ­containing ­4Q8WW59, gene:28337720APEX_matrix, GFP, targetP signal+, mito protein domain, coexpression+, MS/MS++Matrixnoneall 14
LIPT2lipoyl(octanoyl) ­transferase ­2A6NK58, gene:38778720targetP signal, yeast mito homolog, Rickettsial homolog, mito protein domain, coexpression, MS/MSMatrixMetabolism > Lipid metabolism > Lipoate insertioncerebrum, cerebellum, brainstem, spinalcord, skeletalmuscle, adipose, largeintestine, placenta, testis
DECR12,4-dienoyl-CoA ­reductase ­1DECR, NADPH, SDR18C1, Q16698, gene:166620literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, coexpression+, MS/MS++MatrixMetabolism > Lipid metabolism > Fatty acid oxidationall 14
MRPS26mitochondrial ­ribosomal ­protein ­S26C20orf193, GI008, MRP-S13, MRP-S26, MRPS13, NY-BR-87, RPMS13, dJ534B8.3, Q9BYN8, gene:6494920literature, APEX_matrix, targetP signal, mito protein domain+, induction, coexpression, MS/MS+MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
SLC25A15solute ­carrier ­family ­25 ­member ­15D13S327, HHH, LNC-HC, ORC1, ORNT1, Q9Y619, gene:1016620literature, yeast mito homolog+, mito protein domain+, coexpression, MS/MS++MIMMetabolism > Amino acid metabolism > Urea cycle;
Small molecule transport > SLC25A family		spinalcord, kidney, liver, adipose, smallintestine, largeintestine, placenta
NDUFV3NADH:ubiquinone ­oxidoreductase ­subunit ­V3CI-10k, CI-9KD, P56181, gene:473120literature, APEX_matrix, targetP signal, induction, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		all 14
BPHLbiphenyl ­hydrolase ­likeBPH-RP, MCNAA, VACVASE, Q86WA6, gene:67020targetP signal+, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Detoxification > Xenobiotic metabolismall 14
STOML2stomatin ­like ­2HSPC108, SLP-2, Q9UJZ1, gene:3096820literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MIMProtein import, sorting and homeostasis > Protein homeostasisall 14
MSRB2methionine ­sulfoxide ­reductase ­B2CBS-1, CBS1, CGI-131, MSRB, PILB, Q9Y3D2, gene:2292120APEX_matrix, targetP signal, yeast mito homolog+, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Detoxification > ROS and glutathione metabolism;
Metabolism > Sulfur metabolism		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
LACTBlactamase ­betaG24, MRPL56, P83111, gene:11429420literature, APEX_IMS, targetP signal+, Rickettsial homolog, mito protein domain, coexpression, MS/MS++IMSProtein import, sorting and homeostasis > Protein homeostasis > Proteases;
Metabolism > Lipid metabolism > Phospholipid metabolism		all 14
TOMM40translocase ­of ­outer ­mitochondrial ­membrane ­40C19orf1, D19S1177E, PER-EC1, PEREC1, TOM40, O96008, gene:1045220literature, yeast mito homolog+, mito protein domain+, coexpression, MS/MS++MOMProtein import, sorting and homeostasis > Protein import and sorting > TOMcerebrum, spinalcord, kidney, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
SLC25A25solute ­carrier ­family ­25 ­member ­25MCSC, PCSCL, SCAMC-2, Q6KCM7, gene:11478920literature, yeast mito homolog+, mito protein domain+, coexpression, MS/MS++MIMMetabolism > Nucleotide metabolism > Nucleotide import;
Signaling > Calcium homeostasis > EF hand proteins;
Small molecule transport > SLC25A family		cerebrum, brainstem, spinalcord, liver, adipose, smallintestine, largeintestine, placenta
ADCK5aarF ­domain ­containing ­kinase ­5Q3MIX3, gene:20305420targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MSunknownnonecerebrum, largeintestine, testis
ACAD10acyl-CoA ­dehydrogenase ­family ­member ­10Q6JQN1, gene:8072420APEX_matrix, targetP signal+, mito protein domain+, coexpression, MS/MS++MatrixMetabolism > Lipid metabolism > Fatty acid oxidationcerebrum, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
VWA8von ­Willebrand ­factor ­A ­domain ­containing ­8KIAA0564, P7BP2, A3KMH1, gene:2307820APEX_matrix, targetP signal, mito protein domain, coexpression+, MS/MS++Matrixnoneall 14
CCDC90Bcoiled-coil ­domain ­containing ­90BMDS011, MDS025, Q9GZT6, gene:6049220literature, APEX_matrix, targetP signal+, yeast mito homolog+, mito protein domain+, MS/MSMIMnoneall 14
DNAJC11DnaJ ­heat ­shock ­protein ­family ­(Hsp40) ­member ­C11dJ126A5.1, Q9NVH1, gene:5573520GFP, yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MOMProtein import, sorting and homeostasis > Protein import and sorting;
Mitochondrial dynamics and surveillance > Intramitochondrial membrane interactions		all 14
ALKBH7alkB ­homolog ­7ABH7, SPATA11, UNQ6002, Q9BT30, gene:8426620APEX_matrix, targetP signal, mito protein domain, induction, coexpression++, MS/MSMatrixnonecerebellum, brainstem, largeintestine, testis
GLDCglycine ­decarboxylaseGCE, GCSP, HYGN1, P23378, gene:273120literature, APEX_matrix, targetP signal, yeast mito homolog, mito protein domain+, coexpression, MS/MS++MatrixMetabolism > Amino acid metabolism > Glycine metabolism;
Metabolism > Amino acid metabolism > Glycine cleavage system		cerebrum, cerebellum, kidney, liver, adipose, placenta
MPC1Lmitochondrial ­pyruvate ­carrier ­1 ­likeSLC54A3, P0DKB6, gene:34741120targetP signal, yeast mito homolog+, mito protein domain+, MS/MS++MIMMetabolism > Carbohydrate metabolism > Gluconeogenesis;
Metabolism > Carbohydrate metabolism > Pyruvate metabolism;
Metabolism > Carbohydrate metabolism > TCA-associated;
Small molecule transport		testis
THEM4thioesterase ­superfamily ­member ­4CTMP, Q5T1C6, gene:11714520literature, APEX_matrix, targetP signal+, yeast mito homolog, mito protein domain, induction, MS/MS++MIMMetabolism > Lipid metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, placenta, testis
SSBP1single ­stranded ­DNA ­binding ­protein ­1Mt-SSB, SOSS-B1, SSBP, mtSSB, Q04837, gene:674219literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, MS/MSMatrixMitochondrial central dogma > mtDNA maintenance > mtDNA replication;
Mitochondrial central dogma > mtDNA maintenance > mtDNA nucleoid		all 14
MRPL27mitochondrial ­ribosomal ­protein ­L27L27mt, Q9P0M9, gene:5126419literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
HSCBHscB ­mitochondrial ­iron-sulfur ­cluster ­cochaperoneDNAJC20, HSC20, JAC1, Q8IWL3, gene:15027419APEX_matrix, GFP, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, MS/MSMatrixMetabolism > Metals and cofactors > Fe-S cluster biosynthesiscerebrum, cerebellum, brainstem, spinalcord, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
MRPS10mitochondrial ­ribosomal ­protein ­S10MRP-S10, PNAS-122, P82664, gene:5517319literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
AK4adenylate ­kinase ­4AK 4, AK3, AK3L1, AK3L2, P27144, gene:20519APEX_matrix, yeast mito homolog++, Rickettsial homolog, mito protein domain, MS/MS++MatrixMetabolism > Nucleotide metabolism > Nucleotide synthesis and processingall 14
ATAD3AATPase ­family ­AAA ­domain ­containing ­3AHAYOS, PHRINL, Q9NVI7, gene:5521019APEX_IMS, APEX_matrix, GFP, Rickettsial homolog, mito protein domain+, coexpression, MS/MS++MIMMitochondrial central dogma > mtDNA maintenance > mtDNA nucleoid;
Mitochondrial dynamics and surveillance > Organelle contact sites;
Mitochondrial dynamics and surveillance > Intramitochondrial membrane interactions		all 14
MRPL37mitochondrial ­ribosomal ­protein ­L37L2mt, L37mt, MRP-L2, MRP-L37, MRPL2, RPML2, Q9BZE1, gene:5125319literature, APEX_matrix, targetP signal, mito protein domain, induction, coexpression+, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomekidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
PTGES2prostaglandin ­E ­synthase ­2C9orf15, GBF-1, GBF1, PGES2, mPGES-2, Q9H7Z7, gene:8014219literature, APEX_IMS, APEX_matrix, targetP signal, mito protein domain, induction, coexpression+, MS/MS++MIMMetabolism > Lipid metabolism > Eicosanoid metabolismall 14
TXNRD2thioredoxin ­reductase ­2GCCD5, SELZ, TR, TR-BETA, TR3, TRXR2, Q9NNW7, gene:1058719literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Detoxification > ROS and glutathione metabolism;
Metabolism > Detoxification > Selenoproteins		cerebrum, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
ACSM5acyl-CoA ­synthetase ­medium ­chain ­family ­member ­5Q6NUN0, gene:5498819literature, targetP signal+, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Detoxification > Xenobiotic metabolism		kidney, liver, adipose, smallintestine, largeintestine
CISD1CDGSH ­iron ­sulfur ­domain ­1C10orf70, MDS029, ZCD1, mitoNEET, Q9NZ45, gene:5584719literature, mito protein domain, induction, coexpression++, MS/MS++MOMMetabolism > Metals and cofactors > Fe-S cluster biosynthesis;
Metabolism > Metals and cofactors > Fe-S-containing proteins		all 14
MRPL41mitochondrial ­ribosomal ­protein ­L41BMRP, MRP-L27, MRPL27, PIG3, RPML27, Q8IXM3, gene:6497519literature, APEX_matrix, yeast mito homolog++, mito protein domain, induction, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
MTARC2mitochondrial ­amidoxime ­reducing ­component ­2MARC2, MOSC2, Q969Z3, gene:5499619literature, targetP signal, mito protein domain+, induction, coexpression, MS/MS++MOMMetabolism > Metals and cofactors > Molybdenum cofactor synthesis and proteins;
Metabolism > Detoxification > Xenobiotic metabolism;
Metabolism > Detoxification > Amidoxime reducing complex		all 14
MRPS6mitochondrial ­ribosomal ­protein ­S6C21orf101, MRP-S6, RPMS6, S6mt, P82932, gene:6496819literature, APEX_matrix, yeast mito homolog++, Rickettsial homolog, mito protein domain, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
MRPL23mitochondrial ­ribosomal ­protein ­L23L23MRP, RPL23, RPL23L, Q16540, gene:615019literature, APEX_matrix, targetP signal, yeast mito homolog++, mito protein domain, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
SQORsulfide ­quinone ­oxidoreductaseCGI-44, PRO1975, SQR, SQRDL, Q9Y6N5, gene:5847219literature, targetP signal, yeast mito homolog++, mito protein domain, MS/MS++MIMMetabolism > Electron carriers > Q-linked reactions, other;
Metabolism > Sulfur metabolism		spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
SCO1synthesis ­of ­cytochrome ­C ­oxidase ­1SCOD1, O75880, gene:634119literature, APEX_IMS, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, MS/MS++MIMOXPHOS > Complex IV > CIV assembly factors;
Metabolism > Metals and cofactors > Copper metabolism;
OXPHOS > OXPHOS assembly factors		cerebrum, cerebellum, brainstem, spinalcord, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
GCATglycine ­C-acetyltransferaseKBL, O75600, gene:2346419literature, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, MS/MS++unknownMetabolism > Amino acid metabolism > Glycine metabolismbrainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
MTHFD1Lmethylenetetrahydrofolate ­dehydrogenase ­(NADP+ ­dependent) ­1 ­likeFTHFSDC1, MTC1THFS, dJ292B18.2, Q6UB35, gene:2590219literature, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, MS/MS++MatrixMetabolism > Vitamin metabolism > Folate and 1-C metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
ECI2enoyl-CoA ­delta ­isomerase ­2ACBD2, DRS-1, DRS1, HCA88, PECI, dJ1013A10.3, O75521, gene:1045519literature, APEX_matrix, targetP signal+, mito protein domain, coexpression+, MS/MS++MatrixMetabolism > Lipid metabolism > Fatty acid oxidationall 14
UQCRBubiquinol-cytochrome ­c ­reductase ­binding ­proteinMC3DN3, QCR7, QP-C, QPC, UQBC, UQBP, UQCR6, UQPC, P14927, gene:738119literature, APEX_matrix, yeast mito homolog++, mito protein domain+, MS/MS++MIMOXPHOS > Complex III > CIII subunits;
OXPHOS > OXPHOS subunits		all 14
ATP5PDATP ­synthase ­peripheral ­stalk ­subunit ­dAPT5H, ATP5H, ATPQ, O75947, gene:1047619literature, APEX_matrix, yeast mito homolog++, mito protein domain+, MS/MS++MIMOXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits		all 14
ATP5MGATP ­synthase ­membrane ­subunit ­gATP5JG, ATP5L, O75964, gene:1063219literature, APEX_matrix, yeast mito homolog++, mito protein domain+, MS/MS++MIMOXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits;
Mitochondrial dynamics and surveillance > Cristae formation		all 14
CCDC58coiled-coil ­domain ­containing ­58Q4VC31, gene:13107619APEX_IMS, yeast mito homolog++, mito protein domain+, MS/MS++IMSnoneall 14
MCCC2methylcrotonoyl-CoA ­carboxylase ­2MCCB, Q9HCC0, gene:6408719literature, targetP signal, Rickettsial homolog, mito protein domain, coexpression+, MS/MS++MatrixMetabolism > Amino acid metabolism > BCAA metabolismall 14
MCEEmethylmalonyl-CoA ­epimeraseGLOD2, MCE, Q96PE7, gene:8469319targetP signal, induction, coexpression++, MS/MS++MatrixMetabolism > Carbohydrate metabolism > Propanoate metabolism;
Metabolism > Lipid metabolism > Fatty acid oxidation		all 14
MOCS1molybdenum ­cofactor ­synthesis ­1MIG11, MOCOD, MOCS1A, MOCS1B, Q9NZB8, gene:433719APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain+, MS/MS+MatrixMetabolism > Metals and cofactors > Molybdenum cofactor synthesis and proteinscerebrum, liver, adipose, smallintestine, largeintestine, stomach, placenta
MMUTmethylmalonyl-CoA ­mutaseMCM, MUT, P22033, gene:459419literature, APEX_matrix, targetP signal, mito protein domain+, coexpression, MS/MS++MatrixMetabolism > Carbohydrate metabolism > Propanoate metabolism;
Metabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Vitamin metabolism > Vitamin B12 metabolism		all 14
SLC25A51solute ­carrier ­family ­25 ­member ­51CG7943, MCART1, Q9H1U9, gene:9201419GFP, yeast mito homolog+, mito protein domain+, coexpression, MS/MS++MIMMetabolism > Metals and cofactors > NAD biosynthesis and metabolism;
Small molecule transport > SLC25A family		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
SPG7SPG7 ­matrix ­AAA ­peptidase ­subunit, ­parapleginCAR, CMAR, PGN, SPG5C, Q9UQ90, gene:668719literature, targetP signal, Rickettsial homolog, mito protein domain+, coexpression+, MS/MSMIMProtein import, sorting and homeostasis > Protein homeostasis > Proteasescerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
ATP5MC3ATP ­synthase ­membrane ­subunit ­c ­locus ­3ATP5G3, P3, P48201, gene:51819literature, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpression++MIMOXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits		none
ATAD1ATPase ­family ­AAA ­domain ­containing ­1AFDC1, FNP001, HKPX4, Msp1, THORASE, Q8NBU5, gene:8489619yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MOMProtein import, sorting and homeostasis > Protein homeostasis > Proteasescerebrum, cerebellum, brainstem, spinalcord, kidney, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
OXNAD1oxidoreductase ­NAD ­binding ­domain ­containing ­1Q96HP4, gene:9210619APEX_matrix, GFP, mito protein domain, coexpression++, MS/MS++Matrixnonecerebrum, spinalcord, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
MTG2mitochondrial ­ribosome ­associated ­GTPase ­2GTPBP5, ObgH1, dJ1005F21.2, Q9H4K7, gene:2616419literature, APEX_matrix, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpressionMatrixMitochondrial central dogma > Translation > Mitochondrial ribosome assemblylargeintestine, placenta
DARS2aspartyl-tRNA ­synthetase ­2, ­mitochondrialASPRS, LBSL, MT-ASPRS, mtAspRS, Q6PI48, gene:5515719APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MSMatrixMitochondrial central dogma > Translation > mt-tRNA synthetasescerebrum, cerebellum, brainstem, spinalcord, kidney, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
MRPL51mitochondrial ­ribosomal ­protein ­L51CDA09, HSPC241, MRP64, bMRP64, Q4U2R6, gene:5125819literature, APEX_matrix, targetP signal, mito protein domain+, coexpression, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
PCpyruvate ­carboxylasePCB, P11498, gene:509119literature, targetP signal, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Carbohydrate metabolism > Gluconeogenesis;
Metabolism > Carbohydrate metabolism > TCA-associated;
Metabolism > Vitamin metabolism > Biotin utilizing proteins		all 14
CLYBLcitramalyl-CoA ­lyaseCLB, Q8N0X4, gene:17142519APEX_matrix, targetP signal+, induction, coexpression+, MS/MS++MatrixMetabolism > Carbohydrate metabolism > Itaconate metabolismall 14
PNPLA8patatin ­like ­phospholipase ­domain ­containing ­8IPLA2-2, IPLA2G, MMLA, PNPLA-gamma, iPLA2gamma, Q9NP80, gene:5064019APEX_matrix, Rickettsial homolog, induction, coexpression++, MS/MS+MIMMetabolism > Lipid metabolism > Cardiolipin synthesis;
Metabolism > Lipid metabolism > Phospholipid metabolism		cerebrum, cerebellum, brainstem, spinalcord, liver, adipose, largeintestine, testis
MLYCDmalonyl-CoA ­decarboxylaseMCD, O95822, gene:2341719literature, APEX_matrix, targetP signal+, mito protein domain, coexpression++, MS/MSMatrixMetabolism > Lipid metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta
PYURFPIGY ­upstream ­reading ­framePREY, Q96I23, gene:10099693919targetP signal+, induction, coexpression++, MS/MSunknownnonecerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta
TIMM17Btranslocase ­of ­inner ­mitochondrial ­membrane ­17BDXS9822, JM3, TIM17B, O60830, gene:1024519literature, APEX_IMS, yeast mito homolog+, mito protein domain+, coexpression, MS/MS+MIMProtein import, sorting and homeostasis > Protein import and sorting > TIM23 presequence pathwayall 14
MRPL32mitochondrial ­ribosomal ­protein ­L32HSPC283, L32mt, MRP-L32, bMRP-59b, Q9BYC8, gene:6498319literature, APEX_matrix, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
SLC25A26solute ­carrier ­family ­25 ­member ­26COXPD28, SAMC, Q70HW3, gene:11528619literature, yeast mito homolog++, mito protein domain+, coexpression, MS/MSMIMSmall molecule transport > SLC25A familycerebrum, cerebellum, adipose, largeintestine, placenta, testis
PRODH2proline ­dehydrogenase ­2HSPOX1, HYPDH, Q9UF12, gene:5851019targetP signal, yeast mito homolog+, mito protein domain+, MS/MS+MIMMetabolism > Amino acid metabolism > Glyoxylate metabolism;
Metabolism > Electron carriers > Q-linked reactions, other		kidney, liver
NIT2nitrilase ­family ­member ­2HEL-S-8a, Q9NQR4, gene:5695419Rickettsial homolog, mito protein domain, coexpression, MS/MS++unknownMetabolism > Detoxificationcerebellum, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
FDX2ferredoxin ­2FDX1L, MEOAL, Q6P4F2, gene:11281218APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpression, MS/MSMatrixMetabolism > Metals and cofactors > Heme synthesis and processing;
Metabolism > Metals and cofactors > Fe-S cluster biosynthesis;
Metabolism > Metals and cofactors > Fe-S-containing proteins		cerebrum, cerebellum, brainstem, spinalcord, liver, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
ME2malic ­enzyme ­2ODS1, P23368, gene:420018literature, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, MS/MS++MatrixMetabolism > Carbohydrate metabolism > TCA-associatedcerebrum, cerebellum, brainstem, spinalcord, kidney, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
TUFMTu ­translation ­elongation ­factor, ­mitochondrialCOXPD4, EF-TuMT, EFTU, P43, P49411, gene:728418literature, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, MS/MS++MatrixMitochondrial central dogma > Translation > Translation factorsall 14
COX6A2cytochrome ­c ­oxidase ­subunit ­6A2COX6AH, COXVIAH, Q02221, gene:133918literature, targetP signal, yeast mito homolog+, mito protein domain+, induction, coexpressionMIMOXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits		heart, skeletalmuscle
SLC30A9solute ­carrier ­family ­30 ­member ­9BILAPES, C4orf1, GAC63, HUEL, ZNT9, Q6PML9, gene:1046318APEX_matrix, targetP signal+, yeast mito homolog++, mito protein domain, induction, MS/MSMIMSmall molecule transportcerebrum, cerebellum, brainstem, spinalcord, adipose, largeintestine, stomach, placenta, testis
TAMM41TAM41 ­mitochondrial ­translocator ­assembly ­and ­maintenance ­homologC3orf31, RAM41, TAM41, Q96BW9, gene:13200118GFP, yeast mito homolog++, mito protein domain+, MS/MS++MIMMetabolism > Lipid metabolism > Cardiolipin synthesis;
Metabolism > Lipid metabolism > Phospholipid metabolism		cerebrum, cerebellum, brainstem, spinalcord, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
TIMM23translocase ­of ­inner ­mitochondrial ­membrane ­23TIM23, O14925, gene:10028793218literature, APEX_IMS, yeast mito homolog++, mito protein domain+, MS/MS++MIMProtein import, sorting and homeostasis > Protein import and sorting > TIM23 presequence pathwayall 14
RMND1required ­for ­meiotic ­nuclear ­division ­1 ­homologC6orf96, COXPD11, RMD1, bA351K16, bA351K16.3, Q9NWS8, gene:5500518literature, GFP, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, MS/MSMIMMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > Translation		cerebrum, cerebellum, brainstem, spinalcord, adipose, smallintestine, largeintestine, stomach, placenta, testis
MFN2mitofusin ­2CMT2A, CMT2A2, CMT2A2A, CMT2A2B, CPRP1, HMSN6A, HSG, MARF, O95140, gene:992718literature, mito protein domain+, induction, coexpression, MS/MS+MOMMitochondrial dynamics and surveillance > Fusion;
Mitochondrial dynamics and surveillance > Organelle contact sites		cerebrum, cerebellum, brainstem, spinalcord, liver, adipose, largeintestine, stomach, placenta, testis
DNLZDNL-type ­zinc ­fingerC9orf151, HEP, HEP1, TIMM15, ZIM17, bA413M3.2, Q5SXM8, gene:72848918APEX_matrix, targetP signal, yeast mito homolog++, mito protein domain+, MS/MSMatrixProtein import, sorting and homeostasis > Protein homeostasis > Chaperonescerebrum, cerebellum, brainstem, heart, adipose, smallintestine, largeintestine, stomach, placenta
CHCHD4coiled-coil-helix-coiled-coil-helix ­domain ­containing ­4MIA40, TIMM40, Q8N4Q1, gene:13147418literature, APEX_IMS, yeast mito homolog++, mito protein domain, induction, coexpression, MS/MS+IMSProtein import, sorting and homeostasis > Protein import and sorting > MIA40cerebrum, cerebellum, brainstem, spinalcord, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
NDUFAB1NADH:ubiquinone ­oxidoreductase ­subunit ­AB1ACP, ACP1, FASN2A, SDAP, O14561, gene:470618literature, APEX_matrix, targetP signal+, yeast mito homolog, Rickettsial homolog, mito protein domain, MS/MS+MIMOXPHOS > Complex I > CI subunits;
Metabolism > Lipid metabolism > Type II fatty acid synthesis;
Metabolism > Metals and cofactors > Fe-S cluster biosynthesis;
OXPHOS > OXPHOS subunits		all 14
ACSM1acyl-CoA ­synthetase ­medium ­chain ­family ­member ­1BUCS1, MACS1, Q08AH1, gene:11628518literature, targetP signal, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Lipid metabolism > Fatty acid oxidationspinalcord, kidney, liver, smallintestine
COA5cytochrome ­c ­oxidase ­assembly ­factor ­56330578E17Rik, C2orf64, CEMCOX3, Pet191, Q86WW8, gene:49375318literature, APEX_IMS, yeast mito homolog++, mito protein domain+, induction, MS/MSIMSOXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors		adipose, placenta, testis
COQ2coenzyme ­Q2, ­polyprenyltransferaseCL640, COQ10D1, MSA1, PHB:PPT, Q96H96, gene:2723518literature, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression+MIMMetabolism > Metals and cofactors > Coenzyme Q metabolismnone
PDSS2decaprenyl ­diphosphate ­synthase ­subunit ­2C6orf210, COQ10D3, COQ1B, DLP1, bA59I9.3, hDLP1, Q86YH6, gene:5710718literature, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpression+MIMMetabolism > Metals and cofactors > Coenzyme Q metabolismsmallintestine, largeintestine
EHHADHenoyl-CoA ­hydratase ­and ­3-hydroxyacyl ­CoA ­dehydrogenaseECHD, FRTS3, L-PBE, LBFP, LBP, MFE1, PBFE, Q08426, gene:196218yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpression, MS/MS+unknownMetabolism > Lipid metabolismspinalcord, kidney, liver, adipose, smallintestine, largeintestine, placenta
NDUFS3NADH:ubiquinone ­oxidoreductase ­core ­subunit ­S3CI-30, MC1DN8, O75489, gene:472218literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		all 14
ABCB10ATP ­binding ­cassette ­subfamily ­B ­member ­10EST20237, M-ABC2, MTABC2, Q9NRK6, gene:2345618literature, APEX_matrix, yeast mito homolog+, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS+MIMMetabolism > Metals and cofactors > Heme synthesis and processing;
Small molecule transport > ABC transporters		cerebellum, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
CKMT2creatine ­kinase, ­mitochondrial ­2SMTCK, P17540, gene:116018literature, targetP signal, mito protein domain, induction, coexpression, MS/MS++MIMMetabolism > Nucleotide metabolism > Creatine metabolismcerebrum, cerebellum, brainstem, spinalcord, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta
MRPS25mitochondrial ­ribosomal ­protein ­S25MRP-S25, RPMS25, P82663, gene:6443218literature, APEX_matrix, targetP signal, mito protein domain, induction, coexpression, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
ACAD9acyl-CoA ­dehydrogenase ­family ­member ­9MC1DN20, NPD002, Q9H845, gene:2897618literature, APEX_matrix, targetP signal+, mito protein domain+, MS/MS++MIMOXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors		all 14
COX16cytochrome ­c ­oxidase ­assembly ­factor ­COX16C14orf112, HSPC203, hCOX16, Q9P0S2, gene:5124118APEX_IMS, GFP, yeast mito homolog++, mito protein domain+, MS/MS+MIMOXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors		kidney, liver, adipose, largeintestine, placenta, testis
FAM210Afamily ­with ­sequence ­similarity ­210 ­member ­AC18orf19, HsT2329, Q96ND0, gene:12522818GFP, targetP signal, induction, coexpression++, MS/MSMIMnoneall 14
ACOT13acyl-CoA ­thioesterase ­13HT012, PNAS-27, THEM2, Q9NPJ3, gene:5585618mito protein domain, induction, coexpression, MS/MS++MatrixMetabolism > Lipid metabolismall 14
CPT1Acarnitine ­palmitoyltransferase ­1ACPT1, CPT1-L, L-CPT1, P50416, gene:137418literature, yeast mito homolog+, mito protein domain, induction, coexpression, MS/MS++MOMMetabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Metals and cofactors > Carnitine synthesis and transport;
Metabolism > Metals and cofactors > Carnitine shuttle		all 14
DHRS4dehydrogenase/reductase ­4CR, NRDR, PHCR, PSCD, SCAD-SRL, SDR-SRL, SDR25C1, SDR25C2, Q9BTZ2, gene:1090118targetP signal, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Vitamin metabolism > Vitamin A metabolismall 14
PRELID2PRELI ­domain ­containing ­2Q8N945, gene:15376818yeast mito homolog+, mito protein domain+, induction, coexpression, MS/MSIMSnonelargeintestine, stomach
CARS2cysteinyl-tRNA ­synthetase ­2, ­mitochondrialCOXPD27, cysRS, Q9HA77, gene:7958718APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, coexpression++, MS/MSMatrixMitochondrial central dogma > Translation > mt-tRNA synthetasescerebrum, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
NIPSNAP2nipsnap ­homolog ­2GBAS, O75323, gene:263118literature, APEX_matrix, targetP signal+, induction, coexpression, MS/MS++MatrixMitochondrial dynamics and surveillance > Mitophagy;
Mitochondrial dynamics and surveillance > Autophagy		all 14
NDUFB7NADH:ubiquinone ­oxidoreductase ­subunit ­B7B18, CI-B18, P17568, gene:471318literature, APEX_IMS, APEX_matrix, induction, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		all 14
MICOS13mitochondrial ­contact ­site ­and ­cristae ­organizing ­system ­subunit ­13C19orf70, MIC13, P117, QIL1, Q5XKP0, gene:12598818induction, coexpression++, MS/MS++MIMMitochondrial dynamics and surveillance > Cristae formation > MICOS complexall 14
GLRX2glutaredoxin ­2CGI-133, GRX2, Q9NS18, gene:5102217literature, targetP signal+, yeast mito homolog, Rickettsial homolog, mito protein domain, coexpression, MS/MSMatrixMetabolism > Metals and cofactors > Fe-S-containing proteins;
Metabolism > Vitamin metabolism > Vitamin C metabolism;
Metabolism > Detoxification > ROS and glutathione metabolism		cerebrum, cerebellum, brainstem, spinalcord, skeletalmuscle, largeintestine, placenta, testis
MRPS30mitochondrial ­ribosomal ­protein ­S30MRP-S30, PAP, PDCD9, S30mt, Q9NP92, gene:1088417literature, APEX_matrix, targetP signal, mito protein domain, induction, coexpression, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, spinalcord, kidney, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
SCO2synthesis ­of ­cytochrome ­C ­oxidase ­2CEMCOX1, ECGF1, Gliostatin, MYP6, PD-ECGF, SCO1L, TP, TYMP, TdRPase, O43819, gene:999717literature, APEX_IMS, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain, MS/MSMIMOXPHOS > Complex IV > CIV assembly factors;
Metabolism > Metals and cofactors > Copper metabolism;
OXPHOS > OXPHOS assembly factors		all 14
HSD17B10hydroxysteroid ­17-beta ­dehydrogenase ­1017b-HSD10, ABAD, CAMR, DUPXp11.22, ERAB, HADH2, HCD2, HSD10MD, MHBD, MRPP2, MRX17, MRX31, MRXS10, SCHAD, SDR5C1, Q99714, gene:302817literature, APEX_matrix, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS++MatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > Polycistronic mtRNA processing;
Mitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications;
Metabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Lipid metabolism > Cholesterol, bile acid, steroid synthesis;
Metabolism > Amino acid metabolism > BCAA metabolism		all 14
NARS2asparaginyl-tRNA ­synthetase ­2, ­mitochondrialDFNB94, SLM5, asnRS, Q96I59, gene:7973117GFP, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpressionMatrixMitochondrial central dogma > Translation > mt-tRNA synthetasesspinalcord, adipose, largeintestine, stomach, placenta
RPUSD3RNA ­pseudouridine ­synthase ­D3Q6P087, gene:28536717APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications		largeintestine
SUOXsulfite ­oxidaseP51687, gene:682117APEX_IMS, targetP signal, mito protein domain+, coexpression, MS/MS++IMSMetabolism > Metals and cofactors > Molybdenum cofactor synthesis and proteins;
Metabolism > Sulfur metabolism		cerebrum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
SARDHsarcosine ­dehydrogenaseBPR-2, DMGDHL1, SAR, SARD, SDH, Q9UL12, gene:175717literature, targetP signal+, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Amino acid metabolism > Glycine metabolism;
Metabolism > Vitamin metabolism > Choline and betaine metabolism;
Metabolism > Vitamin metabolism > Folate and 1-C metabolism		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
RIDAreactive ­intermediate ­imine ­deaminase ­A ­homologHRSP12, P14.5, PSP, UK114, hp14.5, P52758, gene:1024717APEX_matrix, yeast mito homolog++, mito protein domain, coexpression, MS/MS+Matrixnonecerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
COX8Acytochrome ­c ­oxidase ­subunit ­8ACOX, COX8, COX8-2, COX8L, VIII, VIII-L, P10176, gene:135117literature, targetP signal, mito protein domain+, coexpression+, MS/MSMIMOXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits		stomach, placenta, testis
SPRsepiapterin ­reductaseSDR38C1, P35270, gene:669717Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS++unknownMetabolism > Metals and cofactors > Tetrahydrobiopterin synthesiscerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, largeintestine, stomach
MPV17mitochondrial ­inner ­membrane ­protein ­MPV17CMT2EE, MTDPS6, SYM1, P39210, gene:435817literature, yeast mito homolog+, mito protein domain, coexpression, MS/MS++MIMSmall molecule transportall 14
ZADH2zinc ­binding ­alcohol ­dehydrogenase ­domain ­containing ­2PRG-3, Q8N4Q0, gene:28427317mito protein domain, induction, coexpression+, MS/MS++unknownMetabolism > Lipid metabolism > Eicosanoid metabolismall 14
MRPL18mitochondrial ­ribosomal ­protein ­L18HSPC071, L18mt, MRP-L18, Q9H0U6, gene:2907417literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, coexpression, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
ALDH1B1aldehyde ­dehydrogenase ­1 ­family ­member ­B1ALDH5, ALDHX, P30837, gene:21917literature, APEX_matrix, targetP signal, yeast mito homolog+, mito protein domain, MS/MS++MatrixMetabolism > Detoxification > Xenobiotic metabolismall 14
CHCHD1coiled-coil-helix-coiled-coil-helix ­domain ­containing ­1C10orf34, C2360, MRP-S37, Q96BP2, gene:11848717GFP, targetP signal, yeast mito homolog+, mito protein domain, coexpression+, MS/MSMIMMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
TIMM8Atranslocase ­of ­inner ­mitochondrial ­membrane ­8ADDP, DDP1, DFN1, MTS, TIM8, O60220, gene:167817literature, yeast mito homolog+, mito protein domain+, MS/MS++MIMProtein import, sorting and homeostasis > Protein homeostasis > Chaperones;
Protein import, sorting and homeostasis > Protein import and sorting		all 14
VDAC3voltage ­dependent ­anion ­channel ­3HD-VDAC3, VDAC-3, Q9Y277, gene:741917literature, yeast mito homolog+, mito protein domain+, MS/MS++MOMSignaling > Calcium homeostasis > Mitochondrial permeability transition pore;
Small molecule transport		all 14
SLC25A24solute ­carrier ­family ­25 ­member ­24APC1, SCAMC-1, SCAMC1, Q6NUK1, gene:2995717literature, APEX_IMS, yeast mito homolog+, mito protein domain+, MS/MS++MIMMetabolism > Nucleotide metabolism > Nucleotide import;
Signaling > Calcium homeostasis > EF hand proteins;
Small molecule transport > SLC25A family		adipose, smallintestine, largeintestine, stomach, placenta, testis
MPC1mitochondrial ­pyruvate ­carrier ­1BRP44L, CGI-129, MPYCD, SLC54A1, Q9Y5U8, gene:5166017GFP, yeast mito homolog+, mito protein domain+, MS/MS++MIMMetabolism > Carbohydrate metabolism > Gluconeogenesis;
Metabolism > Carbohydrate metabolism > Pyruvate metabolism;
Metabolism > Carbohydrate metabolism > TCA-associated;
Small molecule transport		all 14
SLC25A22solute ­carrier ­family ­25 ­member ­22EIEE3, GC-1, GC1, NET44, Q9H936, gene:7975117literature, yeast mito homolog+, mito protein domain+, MS/MS++MIMSmall molecule transport > SLC25A familycerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
SLC25A31solute ­carrier ­family ­25 ­member ­31AAC4, ANT 4, ANT4, SFEC35kDa, Q9H0C2, gene:8344717literature, yeast mito homolog+, mito protein domain+, MS/MS++MIMMetabolism > Nucleotide metabolism > Nucleotide import;
Signaling > Calcium homeostasis > Mitochondrial permeability transition pore;
Small molecule transport > SLC25A family		all 14
SLC25A18solute ­carrier ­family ­25 ­member ­18GC2, Q9H1K4, gene:8373317literature, yeast mito homolog+, mito protein domain+, MS/MS++MIMSmall molecule transport > SLC25A familycerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
TRNT1tRNA ­nucleotidyl ­transferase ­1CCA1, CGI-47, MtCCA, RPEM, SIFD, Q96Q11, gene:5109517literature, targetP signal, yeast mito homolog, Rickettsial homolog, mito protein domain, induction, MS/MSMatrixMitochondrial central dogma > mtRNA metabolism > mt-tRNA modificationsspinalcord, adipose, placenta, testis
IDEinsulin ­degrading ­enzymeINSULYSIN, P14735, gene:341617literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain+, coexpression, MS/MS+MatrixProtein import, sorting and homeostasis > Protein homeostasis > Proteasescerebrum, cerebellum, brainstem, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
GSTK1glutathione ­S-transferase ­kappa ­1GST, GST 13-13, GST13, GST13-13, GSTK1-1, hGSTK1, Q9Y2Q3, gene:37315617literature, mito protein domain+, coexpression+, MS/MS++MatrixMetabolism > Detoxification > Xenobiotic metabolismall 14
VARS2valyl-tRNA ­synthetase ­2, ­mitochondrialCOXPD20, VALRS, VARS2L, VARSL, Q5ST30, gene:5717617APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMitochondrial central dogma > Translation > mt-tRNA synthetasescerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
HSPE1heat ­shock ­protein ­family ­E ­(Hsp10) ­member ­1CPN10, EPF, GROES, HSP10, P61604, gene:333617literature, APEX_IMS, APEX_matrix, yeast mito homolog++, Rickettsial homolog, mito protein domain, MS/MS++MatrixProtein import, sorting and homeostasis > Protein homeostasis > Chaperonesall 14
MRPL54mitochondrial ­ribosomal ­protein ­L54L54mt, MRP-L54, Q6P161, gene:11654117literature, APEX_matrix, yeast mito homolog++, mito protein domain, coexpression, MS/MS+MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
MRPL38mitochondrial ­ribosomal ­protein ­L38HSPC262, L38MT, MRP-L3, MRP-L38, RPML3, Q96DV4, gene:6497817APEX_matrix, targetP signal, mito protein domain, coexpression++, MS/MS+MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, spinalcord, heart, smallintestine, largeintestine, stomach, placenta, testis
MRPL58mitochondrial ­ribosomal ­protein ­L58DS-1, DS1, ICT1, MRP-L58, Q14197, gene:339617APEX_matrix, GFP, targetP signal+, mito protein domain, induction, coexpression, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosome;
Mitochondrial central dogma > Translation > Translation factors		all 14
MTCH1mitochondrial ­carrier ­1CGI-64, PIG60, PSAP, SLC25A49, Q9NZJ7, gene:2378717literature, yeast mito homolog+, mito protein domain+, MS/MS++MOMSmall molecule transport > SLC25A familycerebrum, cerebellum, brainstem, spinalcord, kidney, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
TRIAP1TP53 ­regulated ­inhibitor ­of ­apoptosis ­1HSPC132, MDM35, P53CSV, WF-1, O43715, gene:5149917literature, yeast mito homolog++, mito protein domain, induction, MS/MS++IMSMetabolism > Lipid metabolism > Cardiolipin synthesis;
Metabolism > Lipid metabolism > Phospholipid metabolism		adipose, stomach, placenta
PTCD3pentatricopeptide ­repeat ­domain ­3MRP-S39, Q96EY7, gene:5503717literature, APEX_matrix, targetP signal+, mito protein domain+, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosome;
Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly		all 14
COX10cytochrome ­c ­oxidase ­assembly ­factor ­heme ­A:farnesyltransferase ­COX10Q12887, gene:135217literature, yeast mito homolog, Rickettsial homolog, mito protein domain, induction, coexpression++MIMOXPHOS > Complex IV > CIV assembly factors;
Metabolism > Metals and cofactors > Heme synthesis and processing;
OXPHOS > OXPHOS assembly factors		none
AKAP1A-kinase ­anchoring ­protein ­1AKAP, AKAP121, AKAP149, AKAP84, D-AKAP1, PPP1R43, PRKA1, SAKAP84, TDRD17, Q92667, gene:816517literature, mito protein domain, induction, coexpression++, MS/MS+MOMSignaling > cAMP-PKA signalingcerebellum, skeletalmuscle, testis
MRM3mitochondrial ­rRNA ­methyltransferase ­3RMTL1, RNMTL1, Q9HC36, gene:5517817APEX_matrix, GFP, targetP signal+, mito protein domain+, induction, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications;
Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly		none
AK2adenylate ­kinase ­2ADK2, P54819, gene:20417literature, APEX_IMS, Rickettsial homolog, mito protein domain, coexpression+, MS/MS++IMSMetabolism > Nucleotide metabolism > Nucleotide synthesis and processingall 14
TMEM70transmembrane ­protein ­70MC5DN2, Q9BUB7, gene:5496817literature, APEX_matrix, mito protein domain+, coexpression++, MS/MSMIMOXPHOS > Complex I > CI assembly factors;
OXPHOS > Complex V > CV assembly factors;
OXPHOS > OXPHOS assembly factors		cerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
NOA1nitric ­oxide ­associated ­1C4orf14, MTG3, hAtNOS1, hNOA1, mAtNOS1, Q8NC60, gene:8427317literature, APEX_matrix, targetP signal+, yeast mito homolog++, mito protein domain, coexpressionMIMMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly		adipose, largeintestine
MCUR1mitochondrial ­calcium ­uniporter ­regulator ­1C6orf79, CCDC90A, FMP32, Q96AQ8, gene:6393317APEX_matrix, yeast mito homolog+, mito protein domain+, induction, MS/MSMIMnonecerebrum, cerebellum, brainstem, spinalcord, kidney, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta
TMEM14Ctransmembrane ­protein ­14CC6orf53, HSPC194, MSTP073, NET26, bA421M1.6, Q9P0S9, gene:5152217literature, mito protein domain+, induction, coexpression, MS/MS+MIMnoneall 14
NDUFC1NADH:ubiquinone ­oxidoreductase ­subunit ­C1KFYI, O43677, gene:471717literature, targetP signal+, mito protein domain+, coexpression++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		none
MRPL35mitochondrial ­ribosomal ­protein ­L35L35mt, MRP-L35, Q9NZE8, gene:5131817literature, APEX_matrix, targetP signal, mito protein domain, induction, coexpression++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomelargeintestine, testis
NDUFA3NADH:ubiquinone ­oxidoreductase ­subunit ­A3B9, CI-B9, O95167, gene:469617literature, induction, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		all 14
PDSS1decaprenyl ­diphosphate ­synthase ­subunit ­1COQ1, COQ10D2, COQ1A, DPS, SPS, TPRT, TPT, TPT 1, hDPS1, Q5T2R2, gene:2359017APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpressionMIMMetabolism > Metals and cofactors > Coenzyme Q metabolismskeletalmuscle, largeintestine, placenta, testis
SLC25A16solute ­carrier ­family ­25 ­member ­16D10S105E, GDA, GDC, HGT.1, ML7, hML7, P16260, gene:803417literature, yeast mito homolog+, mito protein domain+, coexpression, MS/MSMIMSmall molecule transport > SLC25A familycerebrum, brainstem, spinalcord, liver, adipose, largeintestine, stomach, placenta, testis
FMC1formation ­of ­mitochondrial ­complex ­V ­assembly ­factor ­1 ­homologC7orf55, HSPC268, 0, gene:15479117literature, APEX_matrix, targetP signal, induction, coexpression++, MS/MSMatrixOXPHOS > Complex V > CV assembly factors;
OXPHOS > OXPHOS assembly factors		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
ACSM3acyl-CoA ­synthetase ­medium ­chain ­family ­member ­3SA, SAH, Q53FZ2, gene:629617literature, targetP signal+, Rickettsial homolog, mito protein domain, MS/MS++MatrixMetabolism > Lipid metabolism > Fatty acid oxidationkidney, liver, adipose, smallintestine, largeintestine, stomach
NDUFAF6NADH:ubiquinone ­oxidoreductase ­complex ­assembly ­factor ­6C8orf38, FRTS5, MC1DN17, Q330K2, gene:13768216literature, targetP signal, mito protein domain, induction, coexpression++, MS/MSMIMOXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors		adipose, smallintestine, largeintestine, placenta, testis
UCP1uncoupling ­protein ­1SLC25A7, UCP, P25874, gene:735016literature, yeast mito homolog+, mito protein domain+, coexpression, MS/MSMIMSmall molecule transport > SLC25A familyadipose
SFXN1sideroflexin ­1SLC56A1, TCC, Q9H9B4, gene:9408116literature, mito protein domain+, coexpression, MS/MS++MIMMetabolism > Amino acid metabolism > Serine metabolism;
Metabolism > Vitamin metabolism > Folate and 1-C metabolism;
Small molecule transport > Sideroflexins		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
GLRX5glutaredoxin ­5C14orf87, FLB4739, GRX5, PR01238, PRO1238, PRSA, SIDBA3, SPAHGC, Q86SX6, gene:5121816literature, APEX_matrix, yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpression, MS/MS+MatrixMetabolism > Metals and cofactors > Fe-S cluster biosynthesis;
Metabolism > Metals and cofactors > Fe-S-containing proteins		all 14
PTRH1peptidyl-tRNA ­hydrolase ­1 ­homologC9orf115, PTH1, Q86Y79, gene:13842816APEX_matrix, GFP, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, MS/MSMatrixnonecerebrum, cerebellum, brainstem, largeintestine, placenta
MRPS11mitochondrial ­ribosomal ­protein ­S11HCC-2, MRP-S11, S11mt, P82912, gene:6496316literature, targetP signal, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
XPNPEP3X-prolyl ­aminopeptidase ­3APP3, ICP55, NPHPL1, Q9NQH7, gene:6392916literature, APEX_matrix, yeast mito homolog, Rickettsial homolog, mito protein domain, coexpression, MS/MS+MatrixProtein import, sorting and homeostasis > Protein import and sorting > Preprotein cleavage;
Protein import, sorting and homeostasis > Protein homeostasis > Proteases		cerebrum, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
PDP1pyruvate ­dehyrogenase ­phosphatase ­catalytic ­subunit ­1PDH, PDP, PDPC, PPM2A, PPM2C, Q9P0J1, gene:5470416literature, APEX_matrix, targetP signal, yeast mito homolog+, mito protein domain, MS/MS++MIMMetabolism > Carbohydrate metabolism > Pyruvate metabolismcerebrum, cerebellum, spinalcord, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
ECI1enoyl-CoA ­delta ­isomerase ­1DCI, P42126, gene:163216literature, APEX_matrix, targetP signal, mito protein domain, coexpression+, MS/MS++MatrixMetabolism > Lipid metabolism > Fatty acid oxidationall 14
SLC25A21solute ­carrier ­family ­25 ­member ­21MTDPS18, ODC, ODC1, Q9BQT8, gene:8987416literature, yeast mito homolog+, mito protein domain+, MS/MS++MIMMetabolism > Amino acid metabolism;
Small molecule transport > SLC25A family		spinalcord, kidney, liver, testis
SLC25A29solute ­carrier ­family ­25 ­member ­29C14orf69, CACL, ORNT3, Q8N8R3, gene:12309616literature, yeast mito homolog+, mito protein domain+, MS/MS++MIMMetabolism > Amino acid metabolism > Lysine metabolism;
Small molecule transport > SLC25A family		placenta, testis
SLC25A45solute ­carrier ­family ­25 ­member ­45Q8N413, gene:28313016yeast mito homolog+, mito protein domain+, MS/MS++MIMSmall molecule transport > SLC25A familyliver, adipose, smallintestine, stomach, placenta, testis
SDHAF3succinate ­dehydrogenase ­complex ­assembly ­factor ­3ACN9, DC11, LYRM10, Sdh7, Q9NRP4, gene:5700116targetP signal, yeast mito homolog++, coexpression+, MS/MSMatrixOXPHOS > Complex II > CII assembly factors;
OXPHOS > OXPHOS assembly factors		adipose, testis
PRELID3BPRELI ­domain ­containing ­3BC20orf45, SLMO2, dJ543J19.5, Q9Y3B1, gene:5101216GFP, yeast mito homolog+, mito protein domain+, induction, coexpressionIMSMetabolism > Lipid metabolism > Phospholipid metabolismnone
SLC25A39solute ­carrier ­family ­25 ­member ­39CGI-69, CGI69, Q9BZJ4, gene:5162916literature, yeast mito homolog+, mito protein domain+, induction, coexpressionMIMSmall molecule transport > SLC25A familynone
NDUFB3NADH:ubiquinone ­oxidoreductase ­subunit ­B3B12, CI-B12, MC1DN25, O43676, gene:470916literature, APEX_IMS, APEX_matrix, mito protein domain+, coexpression+, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		all 14
REXO2RNA ­exonuclease ­2CGI-114, REX2, RFN, SFN, Q9Y3B8, gene:2599616APEX_matrix, targetP signal, yeast mito homolog++, mito protein domain, MS/MS+MatrixMitochondrial central dogma > mtRNA metabolism > mtRNA stability and decayall 14
GATMglycine ­amidinotransferaseAGAT, AT, CCDS3, FRTS1, P50440, gene:262816literature, targetP signal, mito protein domain+, MS/MS++MatrixMetabolism > Nucleotide metabolism > Creatine metabolismspinalcord, kidney, liver, stomach, placenta, testis
TIMM10Btranslocase ­of ­inner ­mitochondrial ­membrane ­10BFXC1, TIM10B, Tim9b, Q9Y5J6, gene:2651516literature, APEX_IMS, yeast mito homolog+, mito protein domain+, MS/MS+MIMProtein import, sorting and homeostasis > Protein homeostasis > Chaperones;
Protein import, sorting and homeostasis > Protein import and sorting		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, largeintestine, stomach, placenta, testis
SARS2seryl-tRNA ­synthetase ­2, ­mitochondrialSARS, SARSM, SERS, SYS, SerRS, SerRSmt, mtSerRS, Q9NP81, gene:5493816literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, MS/MSMatrixMitochondrial central dogma > Translation > mt-tRNA synthetasesskeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
CHDHcholine ­dehydrogenaseQ8NE62, gene:5534916APEX_matrix, targetP signal+, mito protein domain, coexpression, MS/MS++MIMMetabolism > Amino acid metabolism > Glycine metabolism;
Metabolism > Vitamin metabolism > Choline and betaine metabolism;
Metabolism > Vitamin metabolism > Folate and 1-C metabolism;
Metabolism > Electron carriers > Q-linked reactions, other		spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
MRPL57mitochondrial ­ribosomal ­protein ­L57MRP63, bMRP63, Q9BQC6, gene:7898816literature, APEX_matrix, targetP signal, mito protein domain+, coexpression, MS/MS+MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, placenta, testis
C8orf82chromosome ­8 ­open ­reading ­frame ­82Q6P1X6, gene:41491916APEX_matrix, targetP signal, coexpression+, MS/MS++Matrixnoneall 14
EARS2glutamyl-tRNA ­synthetase ­2, ­mitochondrialCOXPD12, MSE1, gluRS, mtGlnRS, Q5JPH6, gene:12445416literature, APEX_matrix, GFP, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpressionMatrixMitochondrial central dogma > Translation > mt-tRNA synthetasesadipose, largeintestine, placenta
GRHPRglyoxylate ­and ­hydroxypyruvate ­reductaseGLXR, GLYD, PH2, Q9UBQ7, gene:938016APEX_matrix, targetP signal, yeast mito homolog, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Amino acid metabolism > Glyoxylate metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
MTFMTmitochondrial ­methionyl-tRNA ­formyltransferaseCOXPD15, FMT1, MC1DN27, Q96DP5, gene:12326316literature, APEX_matrix, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpressionMatrixMitochondrial central dogma > Translation > fMet processing;
Metabolism > Vitamin metabolism > Folate and 1-C metabolism		none
PTCD2pentatricopeptide ­repeat ­domain ­2Q8WV60, gene:7981016literature, mito protein domain+, induction, coexpression, MS/MSMatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > Polycistronic mtRNA processing		smallintestine, largeintestine, placenta
ECHDC1ethylmalonyl-CoA ­decarboxylase ­1HEL-S-76, MMCD, dJ351K20.2, Q9NTX5, gene:5586216yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpression, MS/MS+unknownMetabolism > Lipid metabolismbrainstem, spinalcord, liver, adipose, largeintestine, placenta, testis
HARS2histidyl-tRNA ­synthetase ­2, ­mitochondrialHARSL, HARSR, HO3, HisRS, PRLTS2, P49590, gene:2343816literature, APEX_matrix, GFP, targetP signal, Rickettsial homolog, mito protein domain, coexpression, MS/MS+MatrixMitochondrial central dogma > Translation > mt-tRNA synthetasescerebellum, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
NDUFA4NDUFA4 ­mitochondrial ­complex ­associatedCI-9k, CI-MLRQ, COXFA4, MLRQ, O00483, gene:469716literature, mito protein domain, coexpression++, MS/MS++MIMOXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits		all 14
NDUFA13NADH:ubiquinone ­oxidoreductase ­subunit ­A13B16.6, CDA016, CGI-39, GRIM-19, GRIM19, MC1DN28, Q9P0J0, gene:5107916literature, APEX_IMS, mito protein domain, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		all 14
NAXENAD(P)HX ­epimeraseAIBP, APOA1BP, PEBEL, YJEFN1, Q8NCW5, gene:12824016APEX_matrix, targetP signal, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Metals and cofactors > NAD biosynthesis and metabolismall 14
TMEM11transmembrane ­protein ­11C17orf35, PM1, PMI, P17152, gene:883416GFP, targetP signal, mito protein domain+, induction, coexpression, MS/MSMIMMitochondrial dynamics and surveillanceall 14
SELENOOselenoprotein ­OSELO, Q9BVL4, gene:8364215targetP signal+, yeast mito homolog, mito protein domain+, coexpressionMatrixMetabolism > Detoxification > Selenoproteinsspinalcord, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
NAGSN-acetylglutamate ­synthaseAGAS, ARGA, Q8N159, gene:16241715literature, targetP signal, yeast mito homolog++, mito protein domain+, MS/MSMatrixMetabolism > Amino acid metabolism > Urea cycleliver
HSD17B4hydroxysteroid ­17-beta ­dehydrogenase ­4DBP, MFE-2, MFP-2, MPF-2, PRLTS1, SDR8C1, P51659, gene:329515Rickettsial homolog, mito protein domain+, induction, coexpressionunknownMetabolism > Lipid metabolismkidney, liver, adipose, largeintestine, stomach, testis
NDUFAF7NADH:ubiquinone ­oxidoreductase ­complex ­assembly ­factor ­7C2orf56, MidA, PRO1853, Q7L592, gene:5547115APEX_matrix, GFP, targetP signal, Rickettsial homolog, mito protein domain+, coexpression, MS/MSMIMOXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors		cerebrum, brainstem, spinalcord, kidney, adipose, largeintestine, stomach, placenta, testis
OATornithine ­aminotransferaseGACR, HOGA, OATASE, OKT, P04181, gene:494215literature, APEX_matrix, targetP signal+, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Amino acid metabolism > Proline metabolismall 14
MRPL50mitochondrial ­ribosomal ­protein ­L50MRP-L50, Q8N5N7, gene:5453415literature, APEX_matrix, targetP signal, mito protein domain+, coexpression+, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
COX7Bcytochrome ­c ­oxidase ­subunit ­7BAPLCC, LSDMCA2, P24311, gene:134915literature, targetP signal, mito protein domain+, induction, MS/MS+MIMOXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta
RARS2arginyl-tRNA ­synthetase ­2, ­mitochondrialArgRS, DALRD2, PCH6, PRO1992, RARSL, Q5T160, gene:5703815literature, APEX_matrix, GFP, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMitochondrial central dogma > Translation > mt-tRNA synthetasesall 14
OXLD1oxidoreductase ­like ­domain ­containing ­1C17orf90, Q5BKU9, gene:33922915targetP signal, yeast mito homolog, mito protein domain+, coexpression+unknownnoneadipose, largeintestine, stomach
LACTB2lactamase ­beta ­2CGI-83, Q53H82, gene:5111015targetP signal, yeast mito homolog+, mito protein domain, induction, MS/MS+MatrixMitochondrial central dogma > mtRNA metabolism > mtRNA stability and decaykidney, liver, largeintestine, stomach
NDUFA11NADH:ubiquinone ­oxidoreductase ­subunit ­A11B14.7, CI-B14.7, MC1DN14, Q86Y39, gene:12632815literature, APEX_IMS, mito protein domain+, induction, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		all 14
AURKAIP1aurora ­kinase ­A ­interacting ­protein ­1AIP, AKIP, MRP-S38, Q9NWT8, gene:5499815GFP, targetP signal, yeast mito homolog++, mito protein domain, coexpression+MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomenone
CHCHD7coiled-coil-helix-coiled-coil-helix ­domain ­containing ­7COX23, Q9BUK0, gene:7914515yeast mito homolog, induction, coexpression, MS/MS+IMSMetabolism > Metals and cofactors > Copper metabolismcerebrum, smallintestine, placenta, testis
SHMT2serine ­hydroxymethyltransferase ­2GLYA, HEL-S-51e, SHMT, P34897, gene:647215literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Amino acid metabolism > Serine metabolism;
Metabolism > Amino acid metabolism > Glycine metabolism;
Metabolism > Vitamin metabolism > Folate and 1-C metabolism		cerebrum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
MTHFD2methylenetetrahydrofolate ­dehydrogenase ­(NADP+ ­dependent) ­2, ­methenyltetrahydrofolate ­cyclohydrolaseNMDMC, P13995, gene:1079715literature, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, MS/MSMatrixMetabolism > Vitamin metabolism > Folate and 1-C metabolismspinalcord, placenta, testis
RMDN1regulator ­of ­microtubule ­dynamics ­1CGI-90, FAM82B, RMD-1, RMD1, Q96DB5, gene:5111515APEX_IMS, targetP signal+, induction, coexpression, MS/MSMOMnoneall 14
CPS1carbamoyl-phosphate ­synthase ­1CPSASE1, PHN, P31327, gene:137315literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, MS/MS++MatrixMetabolism > Amino acid metabolism > Urea cyclecerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta
MRPL14mitochondrial ­ribosomal ­protein ­L14L14mt, L32mt, MRP-L14, MRP-L32, MRPL32, RMPL32, RPML32, Q6P1L8, gene:6492815literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, coexpression+, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
MTRES1mitochondrial ­transcription ­rescue ­factor ­1C6orf203, HSPC230, PRED31, Q9P0P8, gene:5125015GFP, induction, coexpression++, MS/MS+MatrixMitochondrial central dogma > mtRNA metabolism > Transcription;
Mitochondrial central dogma > Translation		all 14
RDH13retinol ­dehydrogenase ­13SDR7C3, Q8NBN7, gene:11272415yeast mito homolog+, Rickettsial homolog, mito protein domain, induction, MS/MSMIMMetabolism > Vitamin metabolism > Vitamin A metabolismcerebrum, cerebellum, brainstem, spinalcord, adipose, largeintestine, stomach, placenta, testis
TRMT2BtRNA ­methyltransferase ­2 ­homolog ­BCXorf34, dJ341D10.3, Q96GJ1, gene:7997915GFP, targetP signal, Rickettsial homolog, mito protein domain+, induction, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications;
Mitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications		none
MRPS24mitochondrial ­ribosomal ­protein ­S24HSPC335, MRP-S24, S24mt, bMRP-47, bMRP47, Q96EL2, gene:6495115literature, APEX_matrix, induction, coexpression++, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
COA3cytochrome ­c ­oxidase ­assembly ­factor ­3CCDC56, COX25, HSPC009, MITRAC12, Q9Y2R0, gene:2895815APEX_IMS, APEX_matrix, mito protein domain+, coexpression, MS/MS++MIMMitochondrial central dogma > Translation > Translation factors;
OXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors		all 14
GSRglutathione-disulfide ­reductaseGR, GSRD, HEL-75, HEL-S-122m, P00390, gene:293615literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, MS/MS++MatrixMetabolism > Detoxification > ROS and glutathione metabolismspinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
ENDOGendonuclease ­GQ14249, gene:202115literature, APEX_IMS, mito protein domain, coexpression++, MS/MSIMSMitochondrial central dogma > mtDNA maintenance > mtDNA stability and decay;
Mitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay;
Mitochondrial dynamics and surveillance > Apoptosis		all 14
BCAT2branched ­chain ­amino ­acid ­transaminase ­2BCAM, BCATM, BCT2, HVLI, PP18, O15382, gene:58715literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Amino acid metabolism > BCAA metabolismcerebrum, cerebellum, brainstem, kidney, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
TEFMtranscription ­elongation ­factor, ­mitochondrialC17orf42, Q96QE5, gene:7973615APEX_matrix, targetP signal, induction, coexpression++, MS/MSMatrixMitochondrial central dogma > mtRNA metabolism > Transcriptionliver, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
ACSF2acyl-CoA ­synthetase ­family ­member ­2ACSMW, AVYV493, Q96CM8, gene:8022115APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, MS/MS++MatrixMetabolism > Lipid metabolism > Fatty acid oxidationcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
TRMUtRNA ­mitochondrial ­2-thiouridylaseLCAL3, MTO2, MTU1, TRMT, TRMT1, O75648, gene:5568715literature, APEX_matrix, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mt-tRNA modificationsadipose, largeintestine, placenta
MRPS22mitochondrial ­ribosomal ­protein ­S22C3orf5, COXPD5, GIBT, GK002, MRP-S22, ODG7, RPMS22, P82650, gene:5694515literature, APEX_matrix, mito protein domain+, induction, coexpression, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
LAP3leucine ­aminopeptidase ­3HEL-S-106, LAP, LAPEP, PEPS, P28838, gene:5105615APEX_matrix, targetP signal+, Rickettsial homolog, coexpression, MS/MS++MatrixProtein import, sorting and homeostasis > Protein homeostasis > Proteasesall 14
GLUD1glutamate ­dehydrogenase ­1GDH, GDH1, GLUD, P00367, gene:274615literature, APEX_matrix, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Amino acid metabolism > Glutamate metabolism;
Metabolism > Amino acid metabolism > GABA metabolism		all 14
HMGCL3-hydroxy-3-methylglutaryl-CoA ­lyaseHL, P35914, gene:315515literature, APEX_matrix, targetP signal+, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Carbohydrate metabolism > Ketone metabolism;
Metabolism > Amino acid metabolism > BCAA metabolism		all 14
FHITfragile ­histidine ­triad ­diadenosine ­triphosphataseAP3Aase, FRA3B, P49789, gene:227215yeast mito homolog, Rickettsial homolog, mito protein domain, MS/MS++MatrixMetabolism > Nucleotide metabolism > Nucleotide synthesis and processingcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
MRPS33mitochondrial ­ribosomal ­protein ­S33CGI-139, MRP-S33, PTD003, S33mt, Q9Y291, gene:5165015literature, targetP signal, mito protein domain+, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
NUBPLnucleotide ­binding ­protein ­likeC14orf127, IND1, MC1DN21, huInd1, Q8TB37, gene:8022414literature, targetP signal, Rickettsial homolog, mito protein domain, coexpression+, MS/MS+MatrixOXPHOS > Complex I > CI assembly factors;
Metabolism > Metals and cofactors > Fe-S cluster biosynthesis;
Metabolism > Metals and cofactors > Fe-S-containing proteins;
OXPHOS > OXPHOS assembly factors		spinalcord, adipose, largeintestine, stomach, placenta, testis
LYRM2LYR ­motif ­containing ­2DJ122O8.2, Q9NU23, gene:5722614APEX_matrix, GFP, targetP signal+, mito protein domain+, coexpression, MS/MSMatrixOXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors		heart, adipose, largeintestine, placenta
MPV17LMPV17 ­mitochondrial ­inner ­membrane ­protein ­likeM-LPH, MLPH1, MLPH2, MPV17L1, Q2QL34, gene:25502714targetP signal, yeast mito homolog+, mito protein domain, MS/MS++MIMSmall molecule transportcerebrum, cerebellum, brainstem, spinalcord, kidney, liver
GADD45GIP1GADD45G ­interacting ­protein ­1CKBBP2, CKbetaBP2, CRIF1, MRP-L59, PLINP, PLINP-1, PRG6, Plinp1, Q8TAE8, gene:9048014APEX_matrix, GFP, targetP signal, coexpression+, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
TFAMtranscription ­factor ­A, ­mitochondrialMTDPS15, MTTF1, MTTFA, TCF6, TCF6L1, TCF6L2, TCF6L3, Q00059, gene:701914literature, APEX_matrix, targetP signal, mito protein domain, induction, MS/MS++MatrixMitochondrial central dogma > mtDNA maintenance > mtDNA replication;
Mitochondrial central dogma > mtDNA maintenance > mtDNA nucleoid;
Mitochondrial central dogma > mtRNA metabolism > Transcription		all 14
ALDH18A1aldehyde ­dehydrogenase ­18 ­family ­member ­A1ADCL3, ARCL3A, GSAS, P5CS, PYCS, SPG9, SPG9A, SPG9B, P54886, gene:583214literature, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Amino acid metabolism > Proline metabolismcerebrum, cerebellum, brainstem, spinalcord, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
OXSM3-oxoacyl-ACP ­synthase, ­mitochondrialCEM1, FASN2D, KASI, KS, Q9NWU1, gene:5499514literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, MS/MS++MatrixMetabolism > Lipid metabolism > Type II fatty acid synthesisliver, heart, adipose, smallintestine, largeintestine, placenta, testis
KYAT3kynurenine ­aminotransferase ­3CCBL2, KAT3, KATIII, Q6YP21, gene:5626714APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, MS/MS++MatrixMetabolism > Amino acid metabolism > Kynurenine metabolism;
Metabolism > Metals and cofactors > NAD biosynthesis and metabolism		spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
TARS2threonyl-tRNA ­synthetase ­2, ­mitochondrialCOXPD21, TARSL1, thrRS, Q9BW92, gene:8022214APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, MS/MS++MatrixMitochondrial central dogma > Translation > mt-tRNA synthetasescerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
TBRG4transforming ­growth ­factor ­beta ­regulator ­4CPR2, FASTKD4, Q969Z0, gene:923814literature, APEX_matrix, targetP signal+, coexpression, MS/MS++MatrixMitochondrial central dogma > mtRNA metabolism > Polycistronic mtRNA processing;
Mitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay		all 14
TMEM65transmembrane ­protein ­65Q6PI78, gene:15737814literature, APEX_matrix, targetP signal+, coexpression, MS/MS++MIMnonecerebrum, cerebellum, brainstem, spinalcord, kidney, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
ROMO1reactive ­oxygen ­species ­modulator ­1C20orf52, MTGM, MTGMP, bA353C18.2, P60602, gene:14082314literature, APEX_IMS, yeast mito homolog++, mito protein domain+, MS/MSMIMProtein import, sorting and homeostasis > Protein import and sorting > TIM23 presequence pathway;
Small molecule transport		cerebrum, cerebellum, brainstem, spinalcord, liver, skeletalmuscle, adipose, smallintestine, stomach, placenta, testis
PRDX5peroxiredoxin ­5ACR1, AOEB166, B166, HEL-S-55, PLP, PMP20, PRDX6, PRXV, SBBI10, prx-V, P30044, gene:2582414literature, APEX_matrix, targetP signal, mito protein domain, induction, MS/MS++MatrixMetabolism > Detoxification > ROS and glutathione metabolismall 14
DNAJC15DnaJ ­heat ­shock ­protein ­family ­(Hsp40) ­member ­C15DNAJD1, HSD18, MCJ, Q9Y5T4, gene:2910314GFP, mito protein domain, induction, coexpression, MS/MS+MIMProtein import, sorting and homeostasis > Protein import and sorting > Import motorcerebrum, spinalcord, liver, adipose, smallintestine, largeintestine, placenta, testis
MAIP1matrix ­AAA ­peptidase ­interacting ­protein ­1C2orf47, Q8WWC4, gene:7956814GFP, targetP signal, induction, coexpression, MS/MS+MatrixProtein import, sorting and homeostasis > Protein homeostasis > Chaperonesall 14
HIGD1AHIG1 ­hypoxia ­inducible ­domain ­family ­member ­1AHIG1, RCF1a, Q9Y241, gene:2599414literature, APEX_IMS, mito protein domain+, induction, MS/MS++MIMOXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors;
OXPHOS > Respirasome assembly		all 14
C16orf91chromosome ­16 ­open ­reading ­frame ­91CCSMST1, URLC5, gs103, Q4G0I0, gene:28395114APEX_IMS, targetP signal+, induction, coexpression, MS/MSIMSnonebrainstem, spinalcord, adipose, largeintestine, placenta, testis
RHOT2ras ­homolog ­family ­member ­T2ARHT2, C16orf39, MIRO-2, MIRO2, RASL, Q8IXI1, gene:8994114literature, mito protein domain+, induction, coexpressionMOMSignaling > Calcium homeostasis > EF hand proteins;
Mitochondrial dynamics and surveillance > Trafficking		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
SLC25A44solute ­carrier ­family ­25 ­member ­44Q96H78, gene:967314yeast mito homolog+, mito protein domain+, induction, MS/MSMIMMetabolism > Amino acid metabolism > BCAA metabolism;
Small molecule transport > SLC25A family		placenta
HAO2hydroxyacid ­oxidase ­2GIG16, HAOX2, Q9NYQ3, gene:5117914yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpression, MS/MSunknownMetabolism > Lipid metabolismkidney, largeintestine, testis
OXA1LOXA1L ­mitochondrial ­inner ­membrane ­proteinOXA1, Q15070, gene:501814literature, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpressionMIMProtein import, sorting and homeostasis > Protein import and sorting > OXA;
Mitochondrial central dogma > Translation		cerebrum, brainstem, spinalcord, kidney, heart, adipose, smallintestine, largeintestine, placenta, testis
SLC25A46solute ­carrier ­family ­25 ­member ­46HMSN6B, Q96AG3, gene:9113714yeast mito homolog++, mito protein domain+, inductionMOMSmall molecule transport > SLC25A family;
Mitochondrial dynamics and surveillance > Fission;
Mitochondrial dynamics and surveillance > Intramitochondrial membrane interactions		cerebrum, spinalcord, adipose, placenta
RDH14retinol ­dehydrogenase ­14PAN2, SDR7C4, Q9HBH5, gene:5766514yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpression, MS/MSMembraneMetabolism > Vitamin metabolism > Vitamin A metabolismcerebrum, cerebellum, brainstem, spinalcord, smallintestine, largeintestine, stomach, placenta, testis
ATP5MPLATP ­synthase ­membrane ­subunit ­6.8PL6.8PL, C14orf2, MLQ, MP68, PLPM, P56378, gene:955614induction, coexpression++, MS/MS+MIMOXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta
CA5Bcarbonic ­anhydrase ­5BCA-VB, CAVB, Q9Y2D0, gene:1123814literature, targetP signal, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Amino acid metabolism > Urea cyclecerebellum, spinalcord, kidney, adipose, stomach, placenta, testis
COASYCoenzyme ­A ­synthaseDPCK, NBIA6, NBP, PCH12, PPAT, UKR1, pOV-2, Q13057, gene:8034714APEX_matrix, targetP signal, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Metals and cofactors > Coenzyme A metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
CISD3CDGSH ­iron ­sulfur ­domain ­3MiNT, Miner2, P0C7P0, gene:28410614APEX_matrix, targetP signal, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Metals and cofactors > Fe-S cluster biosynthesis;
Metabolism > Metals and cofactors > Fe-S-containing proteins		all 14
GSTZ1glutathione ­S-transferase ­zeta ­1GSTZ1-1, MAAI, MAAID, MAI, O43708, gene:295414APEX_matrix, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Detoxification > Xenobiotic metabolismall 14
COX6B2cytochrome ­c ­oxidase ­subunit ­6B2COXVIB2, CT59, Q6YFQ2, gene:12596514literature, yeast mito homolog+, mito protein domain+, MS/MS+MIMOXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits		testis
GLOD4glyoxalase ­domain ­containing ­4C17orf25, CGI-150, HC71, Q9HC38, gene:5103114APEX_matrix, targetP signal+, coexpression, MS/MS++Matrixnonecerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
ACAA1acetyl-CoA ­acyltransferase ­1ACAA, PTHIO, THIO, P09110, gene:3014yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpressionunknownMetabolism > Lipid metabolismkidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
CROTcarnitine ­O-octanoyltransferaseCOT, Q9UKG9, gene:5467714yeast mito homolog+, mito protein domain, induction, coexpression, MS/MSunknownMetabolism > Lipid metabolism > Fatty acid oxidationkidney, liver
PDFpeptide ­deformylase, ­mitochondrialQ9HBH1, gene:6414614APEX_matrix, Rickettsial homolog, mito protein domain+, coexpression, MS/MSMatrixMitochondrial central dogma > Translation > fMet processing;
Protein import, sorting and homeostasis > Protein homeostasis		cerebrum, heart, skeletalmuscle, adipose, largeintestine, placenta, testis
COX19cytochrome ­c ­oxidase ­assembly ­factor ­COX19Q49B96, gene:9063914yeast mito homolog, mito protein domain, induction, coexpression+, MS/MSIMSOXPHOS > Complex IV > CIV assembly factors;
Metabolism > Metals and cofactors > Copper metabolism;
OXPHOS > OXPHOS assembly factors		liver, placenta
AMTaminomethyltransferaseGCE, GCST, GCVT, NKH, P48728, gene:27514targetP signal+, mito protein domain, MS/MS++MatrixMetabolism > Amino acid metabolism > Glycine metabolism;
Metabolism > Amino acid metabolism > Glycine cleavage system;
Metabolism > Vitamin metabolism > Folate and 1-C metabolism		cerebrum, brainstem, spinalcord, kidney, liver, largeintestine, stomach, placenta
PDPRpyruvate ­dehydrogenase ­phosphatase ­regulatory ­subunitPDP3, Q8NCN5, gene:5506614APEX_matrix, targetP signal+, mito protein domain, MS/MS++MatrixMetabolism > Carbohydrate metabolism > Pyruvate metabolismcerebrum, cerebellum, brainstem, spinalcord, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
NLNneurolysinAGTBP, EP24.16, MEP, MOP, Q9BYT8, gene:5748614literature, APEX_matrix, targetP signal+, mito protein domain, MS/MS++MatrixProtein import, sorting and homeostasis > Protein homeostasis > Proteasescerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
BOLA1bolA ­family ­member ­1CGI-143, Q9Y3E2, gene:5102713GFP, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Metals and cofactors > Fe-S cluster biosynthesis;
Metabolism > Metals and cofactors > Fe-S-containing proteins		cerebrum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
ALAS25'-aminolevulinate ­synthase ­2ALAS-E, ALASE, ANH1, ASB, SIDBA1, XLDPP, XLEPP, XLSA, P22557, gene:21213literature, yeast mito homolog+, Rickettsial homolog, mito protein domain+, coexpressionMatrixMetabolism > Metals and cofactors > Heme synthesis and processingnone
GRPEL2GrpE ­like ­2, ­mitochondrialMt-GrpE#2, Q8TAA5, gene:13426613literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain+MatrixProtein import, sorting and homeostasis > Protein import and sorting > Import motor;
Metabolism > Metals and cofactors > Fe-S cluster biosynthesis		placenta
ALDH7A1aldehyde ­dehydrogenase ­7 ­family ­member ­A1ATQ1, EPD, PDE, P49419, gene:50113literature, APEX_matrix, targetP signal, yeast mito homolog, mito protein domain, MS/MS++MatrixMetabolism > Amino acid metabolism > Lysine metabolism;
Metabolism > Amino acid metabolism > Glycine metabolism;
Metabolism > Vitamin metabolism > Choline and betaine metabolism;
Metabolism > Vitamin metabolism > Folate and 1-C metabolism;
Metabolism > Detoxification > ROS and glutathione metabolism		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
HDHD5haloacid ­dehalogenase ­like ­hydrolase ­domain ­containing ­5CECR5, Q9BXW7, gene:2744013APEX_matrix, targetP signal, yeast mito homolog, mito protein domain, MS/MS++Matrixnonecerebellum, kidney, liver, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
GFERgrowth ­factor, ­augmenter ­of ­liver ­regenerationALR, ERV1, HERV1, HPO, HPO1, HPO2, HSS, MMCHD, MPMCD, P55789, gene:267113literature, GFP, yeast mito homolog++, mito protein domain, coexpression, MS/MSIMSProtein import, sorting and homeostasis > Protein import and sorting > MIA40cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
IMMP2Linner ­mitochondrial ­membrane ­peptidase ­subunit ­2IMMP2L-IT1, IMP2, IMP2-LIKE, Q96T52, gene:8394313literature, yeast mito homolog++, mito protein domain, coexpression, MS/MSMIMProtein import, sorting and homeostasis > Protein import and sorting > Preprotein cleavage;
Protein import, sorting and homeostasis > Protein homeostasis > Proteases		cerebellum, brainstem, liver, skeletalmuscle, adipose, largeintestine, testis
COX4I2cytochrome ­c ­oxidase ­subunit ­4I2COX4, COX4-2, COX4B, COX4L2, COXIV-2, dJ857M17.2, Q96KJ9, gene:8470113literature, targetP signal, yeast mito homolog++, mito protein domain+MIMOXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits		cerebrum, spinalcord, heart, adipose, largeintestine, stomach, placenta
MRPL39mitochondrial ­ribosomal ­protein ­L39C21orf92, L39mt, L5mt, MRP-L5, MRPL5, MSTP003, PRED22, PRED66, RPML5, Q9NYK5, gene:5414813literature, APEX_matrix, targetP signal, Rickettsial homolog, induction, coexpression, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, brainstem, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
AADATaminoadipate ­aminotransferaseKAT2, KATII, KYAT2, Q8N5Z0, gene:5116613targetP signal, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Amino acid metabolism > Lysine metabolismkidney, liver
FUNDC2FUN14 ­domain ­containing ­2DC44, HCBP6, HCC3, PD03104, Q9BWH2, gene:6599113mito protein domain+, MS/MS++MOMMitochondrial dynamics and surveillance > Organelle contact sites;
Signaling		cerebrum, cerebellum, brainstem, spinalcord, kidney, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
LYPLA1lysophospholipase ­1APT-1, APT1, LPL-I, LPL1, hAPT1, O75608, gene:1043413Rickettsial homolog, mito protein domain, coexpression, MS/MS++unknownMetabolism > Lipid metabolism;
Signaling		all 14
PDHA2pyruvate ­dehydrogenase ­E1 ­subunit ­alpha ­2PDHAL, P29803, gene:516113targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain+MatrixMetabolism > Carbohydrate metabolism > Pyruvate metabolismnone
MTRF1Lmitochondrial ­translational ­release ­factor ­1 ­likeHMRF1L, MRF1L, mtRF1a, Q9UGC7, gene:5451613literature, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domainMatrixMitochondrial central dogma > Translation > Translation factorstestis
GTPBP3GTP ­binding ­protein ­3, ­mitochondrialCOXPD23, GTPBG3, MSS1, MTGP1, THDF1, Q969Y2, gene:8470513literature, APEX_matrix, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domainMatrixMitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications;
Metabolism > Vitamin metabolism > Folate and 1-C metabolism		largeintestine
MTHFD2Lmethylenetetrahydrofolate ­dehydrogenase ­(NADP+ ­dependent) ­2 ­likeQ9H903, gene:44102413literature, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domainMatrixMetabolism > Vitamin metabolism > Folate and 1-C metabolismspinalcord, placenta
YME1L1YME1 ­like ­1 ­ATPaseFTSH, MEG4, OPA11, PAMP, YME1L, Q96TA2, gene:1073013literature, APEX_IMS, Rickettsial homolog, mito protein domain+, MS/MS+MIMProtein import, sorting and homeostasis > Protein homeostasis > Proteasescerebrum, cerebellum, brainstem, spinalcord, kidney, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
ACOT2acyl-CoA ­thioesterase ­2CTE-IA, CTE1A, MTE1, PTE2, PTE2A, ZAP128, P49753, gene:1096513literature, mito protein domain, induction, coexpression, MS/MS++MatrixMetabolism > Lipid metabolismall 14
COA8cytochrome ­c ­oxidase ­assembly ­factor ­8APOP, APOP1, APOPT1, C14orf153, Q96IL0, gene:8433413literature, targetP signal, mito protein domain+, coexpression+MIMOXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors		largeintestine, placenta
MMABmetabolism ­of ­cobalamin ­associated ­BATR, CFAP23, cblB, cob, Q96EY8, gene:32662513APEX_matrix, GFP, targetP signal, coexpression, MS/MS++MatrixMetabolism > Vitamin metabolism > Vitamin B12 metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
PPOXprotoporphyrinogen ­oxidasePPO, V290M, VP, P50336, gene:549813literature, APEX_matrix, yeast mito homolog++, mito protein domain, coexpression, MS/MSMIMMetabolism > Metals and cofactors > Heme synthesis and processingcerebrum, spinalcord, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
ABCD3ATP ­binding ­cassette ­subfamily ­D ­member ­3ABC43, CBAS5, PMP70, PXMP1, ZWS2, P28288, gene:582513GFP, targetP signal, Rickettsial homolog, mito protein domain, induction, coexpressionMembraneSmall molecule transport > ABC transporterskidney, liver, adipose, placenta, testis
SIRT3sirtuin ­3SIR2L3, Q9NTG7, gene:2341013literature, APEX_matrix, mito protein domain, induction, coexpression, MS/MS+MatrixMetabolism > Metals and cofactors > NAD biosynthesis and metabolism;
Signaling		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
LDHDlactate ­dehydrogenase ­DDLACD, DLD, Q86WU2, gene:19725713literature, yeast mito homolog+, mito protein domain, MS/MS++MIMMetabolism > Carbohydrate metabolismkidney, liver, heart, adipose, stomach, placenta, testis
MRPL45mitochondrial ­ribosomal ­protein ­L45L45mt, MRP-L45, B4DEF8, gene:8431113literature, APEX_matrix, mito protein domain, induction, coexpression, MS/MS+MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, brainstem, spinalcord, adipose, smallintestine, largeintestine, stomach, placenta, testis
C12orf65chromosome ­12 ­open ­reading ­frame ­65COXPD7, SPG55, Q9H3J6, gene:9157413literature, yeast mito homolog++, Rickettsial homolog, mito protein domain, MS/MSMatrixMitochondrial central dogma > Translation > Translation factorscerebrum, brainstem, spinalcord, adipose, largeintestine, stomach, placenta
IMMP1Linner ­mitochondrial ­membrane ­peptidase ­subunit ­1IMMP1, IMP1, IMP1-LIKE, Q96LU5, gene:19629413literature, yeast mito homolog++, Rickettsial homolog, mito protein domain, MS/MSMIMProtein import, sorting and homeostasis > Protein import and sorting > Preprotein cleavage;
Protein import, sorting and homeostasis > Protein homeostasis > Proteases		testis
POLGDNA ­polymerase ­gamma, ­catalytic ­subunitMDP1, MIRAS, MTDPS4A, MTDPS4B, PEO, POLG1, POLGA, SANDO, SCAE, P54098, gene:542813literature, APEX_matrix, targetP signal, yeast mito homolog++, mito protein domain, MS/MSMatrixMitochondrial central dogma > mtDNA maintenance > mtDNA replication;
Mitochondrial central dogma > mtDNA maintenance > mtDNA nucleoid;
Mitochondrial central dogma > mtDNA maintenance > mtDNA repair		cerebrum, cerebellum, spinalcord, liver, adipose, largeintestine, placenta, testis
ATPAF1ATP ­synthase ­mitochondrial ­F1 ­complex ­assembly ­factor ­1ATP11, ATP11p, Q5TC12, gene:6475613literature, APEX_matrix, yeast mito homolog++, mito protein domain+, MS/MSMatrixOXPHOS > Complex V > CV assembly factors;
OXPHOS > OXPHOS assembly factors		cerebrum, brainstem, spinalcord, adipose, smallintestine, largeintestine, placenta, testis
BLOC1S1biogenesis ­of ­lysosomal ­organelles ­complex ­1 ­subunit ­1BLOS1, BORCS1, GCN5L1, MICoA, RT14, P78537, gene:264713mito protein domain, coexpression++, MS/MSunknownSignalingplacenta, testis
ALDH1L2aldehyde ­dehydrogenase ­1 ­family ­member ­L2mtFDH, Q3SY69, gene:16042813literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, coexpression, MS/MSMatrixMetabolism > Vitamin metabolism > Folate and 1-C metabolismbrainstem, spinalcord, liver, skeletalmuscle, smallintestine, largeintestine, stomach, placenta
COX18cytochrome ­c ­oxidase ­assembly ­factor ­COX18COX18HS, Q8N8Q8, gene:28552113literature, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpressionMIMOXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors		none
CPOXcoproporphyrinogen ­oxidaseCOX, CPO, CPX, HARPO, HCP, P36551, gene:137113literature, APEX_IMS, APEX_matrix, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MIMMetabolism > Metals and cofactors > Heme synthesis and processingcerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
NNTnicotinamide ­nucleotide ­transhydrogenaseGCCD4, Q13423, gene:2353013literature, APEX_matrix, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS+MIMMetabolism > Metals and cofactors > NAD biosynthesis and metabolismcerebrum, spinalcord, skeletalmuscle, adipose, placenta
NDUFAF8NADH:ubiquinone ­oxidoreductase ­complex ­assembly ­factor ­8C17orf89, MC1DN34, A1L188, gene:28418413targetP signal, induction, coexpression+, MS/MSIMSOXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors		adipose, stomach
ATP23ATP23 ­metallopeptidase ­and ­ATP ­synthase ­assembly ­factor ­homologKUB3, XRCC6BP1, Q9Y6H3, gene:9141913yeast mito homolog++, mito protein domain+, coexpressionIMSProtein import, sorting and homeostasis > Protein homeostasis > Proteasescerebellum
DCAKDdephospho-CoA ­kinase ­domain ­containingQ8WVC6, gene:7987713GFP, yeast mito homolog, Rickettsial homolog, mito protein domain, coexpression, MS/MSMembranenoneall 14
ATP5MFATP ­synthase ­membrane ­subunit ­fATP5J2, ATP5JL, P56134, gene:955113literature, coexpression++, MS/MS++MIMOXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits		all 14
SFXN3sideroflexin ­3BA108L7.2, SFX3, SLC56A3, Q9BWM7, gene:8185513mito protein domain+, MS/MS++MIMMetabolism > Amino acid metabolism > Serine metabolism;
Metabolism > Vitamin metabolism > Folate and 1-C metabolism;
Small molecule transport > Sideroflexins		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
AASSaminoadipate-semialdehyde ­synthaseLKR/SDH, LKRSDH, LORSDH, Q9UDR5, gene:1015713APEX_matrix, targetP signal, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Amino acid metabolism > Lysine metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, largeintestine, stomach, placenta, testis
DIABLOdiablo ­IAP-binding ­mitochondrial ­proteinDFNA64, SMAC, Q9NR28, gene:5661613literature, APEX_IMS, targetP signal+, mito protein domain+, MS/MSIMSMitochondrial dynamics and surveillance > Apoptosiscerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
COX8Ccytochrome ­c ­oxidase ­subunit ­8CCOX8-3, Q7Z4L0, gene:34194713literature, targetP signal+, mito protein domain+, MS/MSMIMOXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits		testis
PXMP2peroxisomal ­membrane ­protein ­2MPV17L3, PMP22, Q9NR77, gene:582713GFP, targetP signal, yeast mito homolog+, mito protein domain, inductionMIMnonecerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, placenta, testis
AGXT2alanine--glyoxylate ­aminotransferase ­2AGT2, BAIBA, DAIBAT, Q9BYV1, gene:6490213literature, yeast mito homolog++, mito protein domain, MS/MS+MatrixMetabolism > Amino acid metabolism > Glyoxylate metabolismkidney, liver
GLS2glutaminase ­2GA, GLS, LGA, hLGA, Q9UI32, gene:2716513targetP signal, mito protein domain+, MS/MS++MatrixMetabolism > Amino acid metabolism > Glutamate metabolismcerebrum, cerebellum, brainstem, spinalcord, liver, skeletalmuscle, smallintestine, largeintestine
APOOapolipoprotein ­OFAM121B, MIC26, MICOS26, Mic23, My025, Q9BUR5, gene:7913513literature, APEX_IMS, mito protein domain+, MS/MS++MIMMitochondrial dynamics and surveillance > Cristae formation > MICOS complexall 14
ACAD11acyl-CoA ­dehydrogenase ­family ­member ­11ACAD-11, Q709F0, gene:8412913mito protein domain+, induction, coexpressionMIMMetabolism > Lipid metabolism > Fatty acid oxidationspinalcord, kidney, liver, adipose
C5orf63chromosome ­5 ­open ­reading ­frame ­63YDR286C, A6NC05, gene:40120713targetP signal+, coexpression+, MS/MSMatrixnonecerebrum, cerebellum, spinalcord, liver, heart, skeletalmuscle, adipose, placenta, testis
HOGA14-hydroxy-2-oxoglutarate ­aldolase ­1C10orf65, DHDPS2, DHDPSL, HP3, NPL2, Q86XE5, gene:11281713GFP, targetP signal, Rickettsial homolog, mito protein domain, coexpression, MS/MSMatrixMetabolism > Amino acid metabolism > Glyoxylate metabolismkidney, liver, adipose, largeintestine, placenta, testis
TTC19tetratricopeptide ­repeat ­domain ­192010204O13Rik, MC3DN2, Q6DKK2, gene:5490213literature, APEX_IMS, targetP signal+, mito protein domain, induction, MS/MS+MIMOXPHOS > Complex III > CIII assembly factors;
OXPHOS > OXPHOS assembly factors		cerebrum, cerebellum, spinalcord, adipose, largeintestine, stomach, testis
NGRNneugrin, ­neurite ­outgrowth ­associatedDSC92, Q9NPE2, gene:5133512APEX_matrix, yeast mito homolog++, mito protein domain+, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications;
Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly		none
TCAIMT ­cell ­activation ­inhibitor, ­mitochondrialC3orf23, TOAG-1, TOAG1, Q8N3R3, gene:28534312literature, APEX_matrix, targetP signal, mito protein domain+, coexpression++Matrixnonenone
GCSHglycine ­cleavage ­system ­protein ­HGCE, NKH, P23434, gene:265312literature, APEX_matrix, targetP signal, yeast mito homolog, mito protein domain+, MS/MSMatrixMetabolism > Lipid metabolism > Lipoate insertion;
Metabolism > Amino acid metabolism > Glycine metabolism;
Metabolism > Amino acid metabolism > Glycine cleavage system		spinalcord, liver, adipose, smallintestine, largeintestine, placenta, testis
SIRT5sirtuin ­5SIR2L5, Q9NXA8, gene:2340812literature, APEX_matrix, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MSMatrixMetabolism > Metals and cofactors > NAD biosynthesis and metabolismcerebrum, brainstem, spinalcord, liver, heart, adipose, largeintestine, stomach
GPT2glutamic--pyruvic ­transaminase ­2ALT2, GPT 2, MRT49, Q8TD30, gene:8470612APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, MS/MS++MatrixMetabolism > Amino acid metabolism > Glutamate metabolismcerebrum, brainstem, liver, skeletalmuscle, adipose, stomach, placenta, testis
TOMM6translocase ­of ­outer ­mitochondrial ­membrane ­6OBTP, TOM6, Q96B49, gene:10018889312GFP, mito protein domain+, coexpression, MS/MS+MOMProtein import, sorting and homeostasis > Protein import and sorting > TOMcerebrum, cerebellum, brainstem, spinalcord, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
FAM162Afamily ­with ­sequence ­similarity ­162 ­member ­AC3orf28, E2IG5, HGTD-P, Q96A26, gene:2635512APEX_IMS, APEX_matrix, targetP signal, induction, coexpression, MS/MS++MIMnoneall 14
ABCB6ATP ­binding ­cassette ­subfamily ­B ­member ­6 ­(Langereis ­blood ­group)ABC, LAN, MTABC3, PRP, umat, Q9NP58, gene:1005812literature, yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpression, MS/MSMOMMetabolism > Metals and cofactors > Heme synthesis and processing;
Small molecule transport > ABC transporters		cerebrum, brainstem, liver, largeintestine, stomach, placenta, testis
MACROD1mono-ADP ­ribosylhydrolase ­1LRP16, Q9BQ69, gene:2899212APEX_matrix, targetP signal+, coexpression, MS/MS++MatrixSignalingcerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
TSTthiosulfate ­sulfurtransferaseRDS, Q16762, gene:726312literature, APEX_matrix, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Detoxification > Xenobiotic metabolism;
Metabolism > Sulfur metabolism		all 14
MT-CO1cytochrome ­c ­oxidase ­subunit ­ICOI, MTCO1, P00395, gene:451212literature, APEX_IMS, yeast mito homolog++, Rickettsial homolog, mito protein domain, MS/MSMIMOXPHOS > Complex IV > CIV subunits;
Metabolism > Metals and cofactors > Heme-containing proteins;
OXPHOS > OXPHOS subunits		all 14
MTFR1Lmitochondrial ­fission ­regulator ­1 ­likeFAM54B, HYST1888, MST116, MSTP116, Q9H019, gene:5618112mito protein domain+, induction, coexpression, MS/MSunknownnonecerebrum, cerebellum, brainstem, spinalcord, placenta, testis
SDR39U1short ­chain ­dehydrogenase/reductase ­family ­39U ­member ­1C14orf124, HCDI, Q9NRG7, gene:5694812mito protein domain, coexpression, MS/MS++unknownnonecerebrum, cerebellum, brainstem, spinalcord, kidney, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
DMGDHdimethylglycine ­dehydrogenaseDMGDHD, ME2GLYDH, Q9UI17, gene:2995812literature, targetP signal, mito protein domain, MS/MS++MatrixMetabolism > Amino acid metabolism > Glycine metabolism;
Metabolism > Vitamin metabolism > Choline and betaine metabolism;
Metabolism > Vitamin metabolism > Folate and 1-C metabolism		spinalcord, kidney, liver, smallintestine, stomach, placenta
SLC25A14solute ­carrier ­family ­25 ­member ­14BMCP1, UCP5, O95258, gene:901612literature, yeast mito homolog+, mito protein domain+, MS/MSMIMMetabolism > Sulfur metabolism;
Small molecule transport > SLC25A family		cerebrum, cerebellum, brainstem, placenta, testis
PRELID3APRELI ­domain ­containing ­3AC18orf43, HFL-EDDG1, SLMO1, Q96N28, gene:1065012yeast mito homolog+, mito protein domain+, MS/MSIMSMetabolism > Lipid metabolism > Cardiolipin synthesis;
Metabolism > Lipid metabolism > Phospholipid metabolism		cerebrum, cerebellum, spinalcord
CHCHD5coiled-coil-helix-coiled-coil-helix ­domain ­containing ­5C2orf9, CHTM1, MIC14, MIX14, Q9BSY4, gene:8426912APEX_IMS, yeast mito homolog++, mito protein domain, coexpression, MS/MSIMSnoneadipose, placenta, testis
TOMM70translocase ­of ­outer ­mitochondrial ­membrane ­70TOMM70A, Tom70, O94826, gene:986812literature, yeast mito homolog+, mito protein domain, induction, coexpressionMOMProtein import, sorting and homeostasis > Protein import and sorting > TOMcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
NIT1nitrilase ­1Q86X76, gene:481712APEX_matrix, targetP signal, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Detoxification > ROS and glutathione metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
FAHD1fumarylacetoacetate ­hydrolase ­domain ­containing ­1C16orf36, YISKL, Q6P587, gene:8188912literature, APEX_matrix, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Carbohydrate metabolism > TCA-associatedall 14
MCUmitochondrial ­calcium ­uniporterC10orf42, CCDC109A, HsMCU, Q8NE86, gene:9055012literature, GFP, mito protein domain+, MS/MS++MIMSignaling > Calcium homeostasis > Calcium cycle;
Small molecule transport > Calcium uniporter		cerebrum, cerebellum, brainstem, spinalcord, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta
CYP27A1cytochrome ­P450 ­family ­27 ­subfamily ­A ­member ­1CP27, CTX, CYP27, Q02318, gene:159312literature, targetP signal+, mito protein domain, MS/MS++MIMMetabolism > Lipid metabolism > Cholesterol, bile acid, steroid synthesis;
Metabolism > Metals and cofactors > Heme-containing proteins		kidney, liver, skeletalmuscle, adipose, smallintestine, placenta, testis
MT-ND2MTND2MTND2, P03891, gene:453612literature, Rickettsial homolog, mito protein domain+, MS/MS+MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		cerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
MT-ND4NADH ­dehydrogenase, ­subunit ­4 ­(complex ­I)MTND4, P03905, gene:453812literature, APEX_IMS, APEX_matrix, Rickettsial homolog, mito protein domain+, MS/MS+MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		all 14
MT-ND5NADH ­dehydrogenase, ­subunit ­5 ­(complex ­I)MTND5, P03915, gene:454012literature, APEX_IMS, APEX_matrix, Rickettsial homolog, mito protein domain+, MS/MS+MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		all 14
DELE1DAP3 ­binding ­cell ­death ­enhancer ­1DELE, DELE1(L), KIAA0141, Q14154, gene:981212targetP signal+, mito protein domain, induction, coexpressionMIMSignalingnone
FASTKD1FAST ­kinase ­domains ­1Q53R41, gene:7967512APEX_matrix, induction, coexpression++MatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > Polycistronic mtRNA processing;
Mitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay		liver, largeintestine
MRPS31mitochondrial ­ribosomal ­protein ­S31IMOGN38, MRP-S31, S31mt, Q92665, gene:1024012literature, APEX_matrix, targetP signal, induction, coexpression, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
ALDH1L1aldehyde ­dehydrogenase ­1 ­family ­member ­L110-FTHFDH, 10-fTHF, FDH, FTHFD, O75891, gene:1084012APEX_matrix, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MSMatrixMetabolism > Vitamin metabolism > Folate and 1-C metabolismcerebrum, liver, smallintestine, largeintestine, stomach, placenta, testis
PTCD1pentatricopeptide ­repeat ­domain ­1O75127, gene:2602411targetP signal+, mito protein domain+, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granulescerebellum, largeintestine, placenta
HADHBhydroxyacyl-CoA ­dehydrogenase ­trifunctional ­multienzyme ­complex ­subunit ­betaECHB, MSTP029, MTPB, TP-BETA, P55084, gene:303211literature, targetP signal, Rickettsial homolog, mito protein domain, MS/MS++MIMMetabolism > Carbohydrate metabolism > Ketone metabolism;
Metabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Amino acid metabolism > Lysine metabolism		all 14
TRIT1tRNA ­isopentenyltransferase ­1COXPD35, GRO1, IPPT, IPT, IPTase, MOD5, hGRO1, Q9H3H1, gene:5480211literature, APEX_matrix, targetP signal, yeast mito homolog, Rickettsial homolog, mito protein domain, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications;
Mitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications		cerebellum
CRLS1cardiolipin ­synthase ­1C20orf155, CLS, CLS1, GCD10, dJ967N21.6, Q9UJA2, gene:5467511literature, targetP signal, yeast mito homolog, Rickettsial homolog, mito protein domain, coexpressionMIMMetabolism > Lipid metabolism > Cardiolipin synthesisnone
GLYCTKglycerate ­kinaseHBEBP2, HBEBP4, HBeAgBP4A, Q8IVS8, gene:13215811targetP signal+, coexpression, MS/MS+MatrixMetabolism > Carbohydrate metabolismkidney, liver, placenta
SLC25A32solute ­carrier ­family ­25 ­member ­32MFT, MFTC, RREI, Q9H2D1, gene:8103411literature, targetP signal, yeast mito homolog+, mito protein domain+MIMMetabolism > Vitamin metabolism > Vitamin B2 metabolism;
Metabolism > Vitamin metabolism > Folate and 1-C metabolism;
Small molecule transport > SLC25A family		adipose, placenta
AARS2alanyl-tRNA ­synthetase ­2, ­mitochondrialAARSL, COXPD8, LKENP, MT-ALARS, MTALARS, Q5JTZ9, gene:5750511literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, MS/MSMatrixMitochondrial central dogma > Translation > mt-tRNA synthetasescerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
EXOGexo/endonuclease ­GENDOGL1, ENDOGL2, ENGL, ENGL-a, ENGL-b, ENGLA, ENGLB, Q9Y2C4, gene:994111APEX_IMS, targetP signal, mito protein domain, MS/MS++MIMMitochondrial central dogma > mtDNA maintenance > mtDNA replication;
Mitochondrial central dogma > mtDNA maintenance > mtDNA repair;
Mitochondrial central dogma > mtDNA maintenance > mtDNA stability and decay		cerebrum, cerebellum, brainstem, spinalcord, adipose, largeintestine, placenta
CHCHD2coiled-coil-helix-coiled-coil-helix ­domain ­containing ­2C7orf17, MIX17B, MNRR1, NS2TP, PARK22, Q9Y6H1, gene:5114211APEX_IMS, targetP signal, yeast mito homolog+, mito protein domain, coexpressionIMSMitochondrial dynamics and surveillance > Apoptosisnone
KMOkynurenine ­3-monooxygenasedJ317G22.1, O15229, gene:856411yeast mito homolog, Rickettsial homolog, mito protein domain, MS/MS++MOMMetabolism > Amino acid metabolism > Kynurenine metabolism;
Metabolism > Metals and cofactors > NAD biosynthesis and metabolism		kidney, liver
SFXN5sideroflexin ­5BBG-TCC, SLC56A5, Q8TD22, gene:9409711literature, mito protein domain+, MS/MS++MIMMetabolism > Carbohydrate metabolism > TCA-associated;
Small molecule transport > Sideroflexins		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, largeintestine
ELAC2elaC ­ribonuclease ­Z ­2COXPD17, ELC2, HPC2, Q9BQ52, gene:6052811APEX_matrix, GFP, targetP signal, Rickettsial homolog, mito protein domain, MS/MSMatrixMitochondrial central dogma > mtRNA metabolism > Polycistronic mtRNA processingcerebellum, skeletalmuscle, adipose, largeintestine, stomach
GPX1glutathione ­peroxidase ­1GPXD, GSHPX1, P07203, gene:287611literature, APEX_matrix, targetP signal, mito protein domain, coexpression, MS/MS+MatrixMetabolism > Detoxification > ROS and glutathione metabolism;
Metabolism > Detoxification > Selenoproteins		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
CPT1Bcarnitine ­palmitoyltransferase ­1BCPT1-M, CPT1M, CPTI, CPTI-M, M-CPT1, MCCPT1, MCPT1, Q92523, gene:137511literature, yeast mito homolog+, mito protein domain, MS/MS++MOMMetabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Metals and cofactors > Carnitine synthesis and transport;
Metabolism > Metals and cofactors > Carnitine shuttle		cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
FIS1fission, ­mitochondrial ­1CGI-135, TTC11, Q9Y3D6, gene:5102411literature, yeast mito homolog++, mito protein domain+MOMMitochondrial dynamics and surveillance > Fissionall 14
PRELID1PRELI ­domain ­containing ­1CGI-106, PRELI, PX19, SBBI12, Q9Y255, gene:2716611literature, APEX_IMS, GFP, targetP signal, yeast mito homolog++, mito protein domain+IMSMetabolism > Lipid metabolism > Cardiolipin synthesis;
Metabolism > Lipid metabolism > Phospholipid metabolism		none
UCP3uncoupling ­protein ­3SLC25A9, P55916, gene:735211literature, yeast mito homolog+, mito protein domain+, MS/MSMIMSmall molecule transport > SLC25A familyspinalcord, heart, skeletalmuscle, adipose
SLC25A27solute ­carrier ­family ­25 ­member ­27UCP4, O95847, gene:948111literature, yeast mito homolog+, mito protein domain+, MS/MSMIMSmall molecule transport > SLC25A familycerebrum, cerebellum, brainstem, spinalcord
SLC25A40solute ­carrier ­family ­25 ­member ­40MCFP, Q8TBP6, gene:5597211literature, yeast mito homolog+, mito protein domain+, MS/MSMIMSmall molecule transport > SLC25A familycerebrum, cerebellum, brainstem, spinalcord, adipose, largeintestine, stomach, placenta, testis
SLC25A23solute ­carrier ­family ­25 ­member ­23APC2, MCSC2, SCaMC-3, Q9BV35, gene:7908511literature, yeast mito homolog+, mito protein domain+, MS/MSMIMMetabolism > Nucleotide metabolism > Nucleotide import;
Signaling > Calcium homeostasis > EF hand proteins;
Small molecule transport > SLC25A family		cerebrum, brainstem, spinalcord, liver, adipose, smallintestine, largeintestine, stomach, placenta
HEMK1HemK ­methyltransferase ­family ­member ­1HEMK, MPRMC, MTQ1, Q9Y5R4, gene:5140911GFP, targetP signal, yeast mito homolog, Rickettsial homolog, mito protein domain+MatrixMitochondrial central dogma > Translation > Translation factorsnone
SLC25A36solute ­carrier ­family ­25 ­member ­36PNC2, Q96CQ1, gene:5518611GFP, yeast mito homolog+, mito protein domain+, coexpressionMIMMetabolism > Nucleotide metabolism > Nucleotide import;
Small molecule transport > SLC25A family		brainstem, spinalcord, placenta, testis
OMA1OMA1 ­zinc ­metallopeptidase2010001O09Rik, DAB1, MPRP-1, MPRP1, YKR087C, ZMPOMA1, peptidase, Q96E52, gene:11520911APEX_IMS, GFP, targetP signal+, yeast mito homolog++, coexpressionMIMProtein import, sorting and homeostasis > Protein homeostasis > Proteases;
Mitochondrial dynamics and surveillance > Fusion;
Mitochondrial dynamics and surveillance > Fission		skeletalmuscle, adipose, largeintestine
LETM2leucine ­zipper ­and ­EF-hand ­containing ­transmembrane ­protein ­2SLC55A2, Q2VYF4, gene:13799411targetP signal, yeast mito homolog+, mito protein domain+MIMnonenone
DHRS1dehydrogenase/reductase ­1SDR19C1, Q96LJ7, gene:11581711Rickettsial homolog, mito protein domain, MS/MS++MIMMetabolism > Lipid metabolism > Cholesterol, bile acid, steroid synthesiscerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
STOMstomatinBND7, EPB7, EPB72, P27105, gene:204011yeast mito homolog+, Rickettsial homolog, mito protein domain, MS/MSMembranenoneadipose, placenta, testis
METTL5methyltransferase ­like ­5HSPC133, MRT72, Q9NRN9, gene:2908110Rickettsial homolog, mito protein domain+, induction, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mt-rRNA modificationsnone
MSRAmethionine ­sulfoxide ­reductase ­APMSR, Q9UJ68, gene:448210mito protein domain, coexpression, MS/MS++MatrixMetabolism > Detoxification > ROS and glutathione metabolism;
Metabolism > Sulfur metabolism		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta
NUDT8nudix ­hydrolase ­8Q8WV74, gene:25455210APEX_matrix, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Metals and cofactors > Coenzyme A metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
NMNAT3nicotinamide ­nucleotide ­adenylyltransferase ­3FKSG76, PNAT-3, PNAT3, Q96T66, gene:34956510literature, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Metals and cofactors > NAD biosynthesis and metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, placenta, testis
SLC25A38solute ­carrier ­family ­25 ­member ­38SIDBA2, Q96DW6, gene:5497710yeast mito homolog++, mito protein domain+, coexpressionMIMMetabolism > Amino acid metabolism > Glycine metabolism;
Metabolism > Metals and cofactors > Heme synthesis and processing;
Metabolism > Vitamin metabolism > Folate and 1-C metabolism;
Small molecule transport > SLC25A family		none
MPV17L2MPV17 ­mitochondrial ­inner ­membrane ­protein ­like ­2FKSG24, Q567V2, gene:8476910GFP, targetP signal, yeast mito homolog+, mito protein domain, coexpressionMIMMitochondrial central dogma > Translation > Mitochondrial ribosome assemblylargeintestine, placenta
ACLYATP ­citrate ­lyaseACL, ATPCL, CLATP, P53396, gene:4710APEX_matrix, yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpressionMatrixMetabolism > Carbohydrate metabolism > TCA-associatedcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, largeintestine, stomach, placenta, testis
ABCD2ATP ­binding ­cassette ­subfamily ­D ­member ­2ABC39, ALDL1, ALDR, ALDRP, hALDR, Q9UBJ2, gene:22510targetP signal, Rickettsial homolog, mito protein domain, coexpression, MS/MS+MembraneSmall molecule transport > ABC transportersadipose
FLAD1flavin ­adenine ­dinucleotide ­synthetase ­1FAD1, FADS, LSMFLAD, PP591, Q8NFF5, gene:8030810APEX_matrix, targetP signal, mito protein domain, induction, coexpressionMatrixMetabolism > Vitamin metabolism > Vitamin B2 metabolismspinalcord, adipose, stomach
LIPT1lipoyltransferase ­1LIPT1D, Q9Y234, gene:5160110GFP, yeast mito homolog++, mito protein domain, coexpressionMatrixMetabolism > Lipid metabolism > Lipoate insertionadipose, placenta
DGLUCYD-glutamate ­cyclaseC14orf159, Q7Z3D6, gene:8001710literature, APEX_matrix, targetP signal, coexpression, MS/MS++MatrixMetabolism > Amino acid metabolismall 14
PCK2phosphoenolpyruvate ­carboxykinase ­2, ­mitochondrialPEPCK, PEPCK-M, PEPCK2, Q16822, gene:510610literature, targetP signal, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Carbohydrate metabolism > Gluconeogenesis;
Metabolism > Carbohydrate metabolism > TCA-associated		cerebrum, cerebellum, brainstem, spinalcord, adipose, smallintestine, largeintestine, stomach, placenta, testis
NADK2NAD ­kinase ­2, ­mitochondrialC5orf33, DECRD, MNADK, NADKD1, Q4G0N4, gene:13368610APEX_matrix, targetP signal, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Metals and cofactors > NAD biosynthesis and metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
MPSTmercaptopyruvate ­sulfurtransferaseMST, TST2, TUM1, P25325, gene:435710APEX_matrix, mito protein domain, coexpression, MS/MS++MIMMetabolism > Detoxification > Xenobiotic metabolism;
Metabolism > Sulfur metabolism		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
NUDT13nudix ­hydrolase ­13Q86X67, gene:2596110GFP, targetP signal, mito protein domain, induction, coexpression, MS/MSMatrixMetabolism > Metals and cofactors > NAD biosynthesis and metabolismcerebellum, brainstem, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta
ACACAacetyl-CoA ­carboxylase ­alphaACAC, ACACAD, ACC, ACC1, ACCA, Q13085, gene:3110targetP signal, Rickettsial homolog, mito protein domain, coexpressionMatrixMetabolism > Lipid metabolism > Type II fatty acid synthesis;
Metabolism > Vitamin metabolism > Biotin utilizing proteins		brainstem, spinalcord, liver, adipose, largeintestine, stomach, placenta, testis
ABAT4-aminobutyrate ­aminotransferaseGABA-AT, GABAT, NPD009, P80404, gene:1810literature, APEX_matrix, targetP signal, mito protein domain, MS/MS++MatrixMetabolism > Amino acid metabolism > GABA metabolism;
Metabolism > Metals and cofactors > Fe-S-containing proteins		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
HMGCS23-hydroxy-3-methylglutaryl-CoA ­synthase ­2P54868, gene:315810literature, targetP signal, mito protein domain, MS/MS++MatrixMetabolism > Carbohydrate metabolism > Ketone metabolismcerebellum, spinalcord, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
ALDH5A1aldehyde ­dehydrogenase ­5 ­family ­member ­A1SSADH, SSDH, P51649, gene:791510APEX_matrix, targetP signal, mito protein domain, MS/MS++MatrixMetabolism > Amino acid metabolism > GABA metabolismall 14
CKMT1Acreatine ­kinase, ­mitochondrial ­1ACKMT1, U-MtCK, mia-CK, 0, gene:54859610literature, targetP signal, mito protein domain, MS/MS++MIMMetabolism > Nucleotide metabolism > Creatine metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta
PISDphosphatidylserine ­decarboxylaseDJ858B16, LIBF, PSD, PSDC, PSSC, dJ858B16.2, Q9UG56, gene:2376110yeast mito homolog++, mito protein domain, MS/MSMIMMetabolism > Lipid metabolism > Phospholipid metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
NDUFB4NADH:ubiquinone ­oxidoreductase ­subunit ­B4B15, CI-B15, O95168, gene:471010literature, mito protein domain+, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		all 14
UQCR11ubiquinol-cytochrome ­c ­reductase, ­complex ­III ­subunit ­XI0710008D09Rik, QCR10, UQCR, O14957, gene:1097510literature, mito protein domain+, MS/MS++MIMOXPHOS > Complex III > CIII subunits;
OXPHOS > OXPHOS subunits		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, stomach, placenta, testis
FAM136Afamily ­with ­sequence ­similarity ­136 ­member ­AQ96C01, gene:8490810APEX_IMS, mito protein domain+, MS/MS++IMSnoneall 14
TOMM5translocase ­of ­outer ­mitochondrial ­membrane ­5C9orf105, Tom5, bA613M10.3, Q8N4H5, gene:40150510literature, mito protein domain+, MS/MS++MOMProtein import, sorting and homeostasis > Protein import and sorting > TOMcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
HTRA2HtrA ­serine ­peptidase ­2MGCA8, OMI, PARK13, PRSS25, O43464, gene:2742910literature, APEX_IMS, targetP signal, Rickettsial homolog, mito protein domain, MS/MS+MIMProtein import, sorting and homeostasis > Protein homeostasis > Proteases;
Mitochondrial dynamics and surveillance > Apoptosis		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
NDUFA1NADH:ubiquinone ­oxidoreductase ­subunit ­A1CI-MWFE, MC1DN12, MWFE, ZNF183, O15239, gene:469410literature, coexpression+, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		all 14
MT-CYBcytochrome ­bMTCYB, P00156, gene:451910literature, yeast mito homolog++, Rickettsial homolog, mito protein domainMIMOXPHOS > Complex III > CIII subunits;
Metabolism > Metals and cofactors > Heme-containing proteins;
Metabolism > Electron carriers > Cytochromes;
OXPHOS > OXPHOS subunits		all 14
GUK1guanylate ­kinase ­1GMK, Q16774, gene:298710APEX_matrix, targetP signal, Rickettsial homolog, induction, coexpression, MS/MSMatrixMetabolism > Nucleotide metabolism > Nucleotide synthesis and processingcerebrum, cerebellum, brainstem, spinalcord, adipose, largeintestine, stomach
D2HGDHD-2-hydroxyglutarate ­dehydrogenaseD2HGD, Q8N465, gene:72829410literature, APEX_matrix, targetP signal, yeast mito homolog++, mito protein domainMatrixMetabolism > Carbohydrate metabolism > TCA-associatedadipose, largeintestine
OPA3outer ­mitochondrial ­membrane ­lipid ­metabolism ­regulator ­OPA3MGA3, Q9H6K4, gene:8020710literature, targetP signal, coexpression, MS/MS++MOMnoneall 14
AKR1B10aldo-keto ­reductase ­family ­1 ­member ­B10AKR1B11, AKR1B12, ALDRLn, ARL-1, ARL1, HIS, HSI, O60218, gene:5701610induction, coexpression, MS/MS++unknownMetabolism > Vitamin metabolism > Vitamin A metabolism;
Metabolism > Detoxification > Xenobiotic metabolism		cerebrum, brainstem, spinalcord, kidney, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
PHYHphytanoyl-CoA ­2-hydroxylaseLN1, LNAP1, PAHX, PHYH1, RD, O14832, gene:526410targetP signal, induction, MS/MS++MatrixMetabolism > Lipid metabolismkidney, liver, smallintestine
NDUFAF2NADH:ubiquinone ­oxidoreductase ­complex ­assembly ­factor ­2B17.2L, MC1DN10, MMTN, NDUFA12L, mimitin, Q8N183, gene:9194210literature, APEX_matrix, induction, coexpression, MS/MS++MIMOXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors		all 14
ECSITECSIT ­signaling ­integratorSITPEC, Q9BQ95, gene:5129510literature, APEX_matrix, targetP signal, mito protein domain, induction, coexpression+MIMOXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors		brainstem, spinalcord, kidney, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
PARLpresenilin ­associated ­rhomboid ­likePRO2207, PSARL, PSARL1, PSENIP2, RHBDS1, Q9H300, gene:5548610literature, yeast mito homolog++, mito protein domain, MS/MSMIMProtein import, sorting and homeostasis > Protein homeostasis > Proteases;
Mitochondrial dynamics and surveillance > Mitophagy;
Mitochondrial dynamics and surveillance > Autophagy		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
MRPL42mitochondrial ­ribosomal ­protein ­L42HSPC204, L31MT, L42MT, MRP-L31, MRP-L42, MRP-S32, MRPL31, MRPS32, PTD007, RPML31, S32MT, Q9Y6G3, gene:2897710APEX_matrix, GFP, mito protein domain+, induction, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeliver, adipose, largeintestine, testis
DNAJC4DnaJ ­heat ­shock ­protein ­family ­(Hsp40) ­member ­C4DANJC4, HSPF2, MCG18, Q9NNZ3, gene:333810GFP, targetP signal+, Rickettsial homolog, mito protein domain, coexpressionMembraneProtein import, sorting and homeostasis > Protein homeostasislargeintestine
PAM16presequence ­translocase ­associated ­motor ­16CGI-136, MAGMAS, SMDMDM, TIM16, TIMM16, Q9Y3D7, gene:5102510literature, APEX_matrix, yeast mito homolog, MS/MS++MIMProtein import, sorting and homeostasis > Protein import and sorting > Import motorall 14
ALDH3A2aldehyde ­dehydrogenase ­3 ­family ­member ­A2ALDH10, FALDH, SLS, P51648, gene:22410targetP signal+, mito protein domain, coexpressionMembraneMetabolism > Detoxification > ROS and glutathione metabolismspinalcord, kidney, liver, smallintestine, largeintestine, stomach, placenta, testis
ACSL6acyl-CoA ­synthetase ­long ­chain ­family ­member ­6ACS2, FACL6, LACS 6, LACS2, LACS5, Q9UKU0, gene:2330510Rickettsial homolog, mito protein domain, MS/MS++MOMMetabolism > Lipid metabolism > Fatty acid oxidationcerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, largeintestine, stomach, testis
MRS2magnesium ­transporter ­MRS2HPT, MRS2L, Q9HD23, gene:5738010APEX_matrix, GFP, targetP signal, yeast mito homolog++, coexpressionMIMSmall molecule transport;
Signaling		cerebrum, cerebellum, brainstem, spinalcord, skeletalmuscle, smallintestine, largeintestine, stomach, placenta, testis
ABHD10abhydrolase ­domain ­containing ­10, ­depalmitoylaseQ9NUJ1, gene:553479literature, APEX_IMS, APEX_matrix, Rickettsial homolog, mito protein domain, MS/MS++MatrixMetabolism > Detoxification > Xenobiotic metabolismcerebrum, cerebellum, brainstem, spinalcord, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
HINT3histidine ­triad ­nucleotide ­binding ­protein ­3HINT4, Q9NQE9, gene:1351149Rickettsial homolog, mito protein domain, induction, coexpression+unknownnonebrainstem, heart, skeletalmuscle
PXMP4peroxisomal ­membrane ­protein ­4PMP24, Q9Y6I8, gene:112649targetP signal, mito protein domain+, coexpressionunknownnonekidney, liver, adipose
OSGEPL1O-sialoglycoprotein ­endopeptidase ­like ­1OSGEPL, Qri7, Q9H4B0, gene:641729APEX_matrix, targetP signal, yeast mito homolog, Rickettsial homolog, mito protein domain, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mt-tRNA modificationsnone
NUDT2nudix ­hydrolase ­2APAH1, P50583, gene:3189mito protein domain, coexpression, MS/MS++MatrixMetabolism > Nucleotide metabolism > Nucleotide synthesis and processingall 14
CYB5Bcytochrome ­b5 ­type ­BCYB5-M, CYPB5M, OMB5, O43169, gene:807779literature, mito protein domain, coexpression, MS/MS++MOMMetabolism > Metals and cofactors > Heme-containing proteins;
Metabolism > Detoxification > Xenobiotic metabolism;
Metabolism > Detoxification > Amidoxime reducing complex;
Metabolism > Electron carriers > Cytochromes		all 14
GRSF1G-rich ­RNA ­sequence ­binding ­factor ­1Q12849, gene:29269APEX_matrix, mito protein domain, induction, coexpression, MS/MSMatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > Polycistronic mtRNA processing;
Mitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay;
Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly		adipose, smallintestine, largeintestine, stomach, placenta, testis
MRPS18Bmitochondrial ­ribosomal ­protein ­S18BC6orf14, HSPC183, HumanS18a, MRP-S18-2, MRPS18-2, PTD017, S18amt, Q9Y676, gene:289739literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, brainstem, spinalcord, liver, heart, adipose, smallintestine, largeintestine, placenta, testis
DNAJC30DnaJ ­heat ­shock ­protein ­family ­(Hsp40) ­member ­C30WBSCR18, Q96LL9, gene:842779APEX_matrix, GFP, targetP signal+, Rickettsial homolog, mito protein domain, MS/MSMIMnonecerebrum, cerebellum, brainstem, spinalcord, heart, skeletalmuscle, adipose, largeintestine, stomach
CATcatalaseP04040, gene:8479yeast mito homolog++, mito protein domain, coexpressionIMSMetabolism > Detoxification > ROS and glutathione metabolismspinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
DGUOKdeoxyguanosine ­kinaseMTDPS3, NCPH, PEOB4, dGK, Q16854, gene:17169literature, APEX_matrix, targetP signal, mito protein domain, MS/MS++MatrixMetabolism > Nucleotide metabolism > Nucleotide synthesis and processingcerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
ACOT9acyl-CoA ­thioesterase ­9ACATE2, CGI-16, MT-ACT48, MTACT48, Q9Y305, gene:235979literature, targetP signal, mito protein domain, MS/MS++MIMMetabolism > Lipid metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
ACSS1acyl-CoA ­synthetase ­short ­chain ­family ­member ­1ACAS2L, ACECS1, AceCS2L, Q9NUB1, gene:845329literature, APEX_matrix, targetP signal, mito protein domain, MS/MS++MIMMetabolism > Lipid metabolism > Fatty acid oxidationcerebrum, cerebellum, brainstem, spinalcord, kidney, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
RFKriboflavin ­kinaseRIFK, Q969G6, gene:553129targetP signal, yeast mito homolog++, MS/MSMatrixMetabolism > Vitamin metabolism > Vitamin B2 metabolismbrainstem, spinalcord, kidney, adipose, smallintestine, largeintestine
STARD7StAR ­related ­lipid ­transfer ­domain ­containing ­7FAME2, GTT1, Q9NQZ5, gene:569109APEX_IMS, targetP signal, mito protein domain, induction, coexpression+IMSMetabolism > Lipid metabolism > Phospholipid metabolism;
Small molecule transport		none
CHPT1choline ­phosphotransferase ­1CPT, CPT1, Q8WUD6, gene:569949targetP signal, mito protein domain, induction, coexpression+MIMMetabolism > Lipid metabolism > Phospholipid metabolismnone
MT-ATP8ATP ­synthase ­F0 ­subunit ­8ATPase8, MTATP8, P03928, gene:45099literature, mito protein domain+, MS/MS++MIMOXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits		all 14
TIMMDC1translocase ­of ­inner ­mitochondrial ­membrane ­domain ­containing ­1C3orf1, MC1DN31, Q9NPL8, gene:513009literature, APEX_IMS, APEX_matrix, mito protein domain+, MS/MS++MIMOXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors		cerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
SPATA19spermatogenesis ­associated ­19CT132, SPAS1, spergen1, Q7Z5L4, gene:2199389mito protein domain+, MS/MS++unknownnonetestis
HTATIP2HIV-1 ­Tat ­interactive ­protein ­2CC3, SDR44U1, TIP30, Q9BUP3, gene:105539yeast mito homolog, mito protein domain+, MS/MSMembranenonecerebrum, cerebellum, brainstem, liver, adipose, placenta, testis
AKR7A2aldo-keto ­reductase ­family ­7 ­member ­A2AFAR, AFAR1, AFB1-AR1, AKR7, O43488, gene:85749APEX_matrix, targetP signal, coexpression, MS/MS++MatrixMetabolism > Amino acid metabolism > GABA metabolism;
Metabolism > Detoxification > Xenobiotic metabolism		all 14
DUTdeoxyuridine ­triphosphatasedUTPase, P33316, gene:18549literature, targetP signal, Rickettsial homolog, MS/MS++MatrixMetabolism > Nucleotide metabolism > Nucleotide synthesis and processingcerebrum, spinalcord, liver, heart, adipose, largeintestine, stomach, placenta
MT-CO3cytochrome ­c ­oxidase ­IIICOIII, MTCO3, P00414, gene:45149literature, yeast mito homolog++, Rickettsial homolog, mito protein domainMIMOXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits		all 14
DHRS7Bdehydrogenase/reductase ­7BCGI-93, SDR32C1, Q6IAN0, gene:259799Rickettsial homolog, mito protein domain, induction, coexpressionMembranenonecerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
MTRF1mitochondrial ­translation ­release ­factor ­1MRF1, MTTRF1, RF1, O75570, gene:96179literature, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domainMatrixMitochondrial central dogma > Translation > Translation factorsadipose
TMEM143transmembrane ­protein ­143Q96AN5, gene:552609targetP signal, coexpression+, MS/MSMIMnonecerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta
NT5M5',3'-nucleotidase, ­mitochondrialdNT-2, dNT2, mdN, Q9NPB1, gene:569539literature, targetP signal, mito protein domain, induction, MS/MSMatrixMetabolism > Nucleotide metabolism > Nucleotide synthesis and processingcerebrum, cerebellum, brainstem, spinalcord, skeletalmuscle, adipose, testis
RHOT1ras ­homolog ­family ­member ­T1ARHT1, MIRO-1, MIRO1, Q8IXI2, gene:552889literature, mito protein domain+, MS/MS+MOMSignaling > Calcium homeostasis > EF hand proteins;
Mitochondrial dynamics and surveillance > Trafficking		all 14
NME4NME/NM23 ­nucleoside ­diphosphate ­kinase ­4NDPK-D, NM23H4, nm23-H4, O00746, gene:48339APEX_matrix, GFP, targetP signal, Rickettsial homolog, mito protein domain, MS/MSMIMMetabolism > Nucleotide metabolism > Nucleotide synthesis and processingadipose, largeintestine, placenta, testis
MTHFSmethenyltetrahydrofolate ­synthetaseHsT19268, NEDMEHM, P49914, gene:105889targetP signal, Rickettsial homolog, mito protein domain, MS/MSMatrixMetabolism > Vitamin metabolism > Folate and 1-C metabolismcerebrum, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta
TIMM29translocase ­of ­inner ­mitochondrial ­membrane ­29C19orf52, TIM29, Q9BSF4, gene:905809targetP signal+, coexpression, MS/MSMIMProtein import, sorting and homeostasis > Protein import and sorting > TIM22 carrier pathwaycerebrum, cerebellum, brainstem, spinalcord, liver, heart, adipose, largeintestine, placenta, testis
PNPOpyridoxamine ­5'-phosphate ­oxidaseHEL-S-302, PDXPO, Q9NVS9, gene:551639APEX_matrix, yeast mito homolog++, coexpression, MS/MSMatrixMetabolism > Vitamin metabolism > Vitamin B6 metabolismbrainstem, liver
MICU2mitochondrial ­calcium ­uptake ­21110008L20Rik, EFHA1, Q8IYU8, gene:2211549literature, APEX_IMS, GFP, targetP signal+, mito protein domain, MS/MS+MIMSignaling > Calcium homeostasis > Calcium cycle;
Signaling > Calcium homeostasis > EF hand proteins;
Small molecule transport > Calcium uniporter		smallintestine, largeintestine, placenta, testis
PCBD2pterin-4 ­alpha-carbinolamine ­dehydratase ­2DCOH2, DCOHM, PHS2, Q9H0N5, gene:841059targetP signal+, induction, MS/MSunknownMetabolism > Metals and cofactors > Tetrahydrobiopterin synthesiscerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
QRSL1glutaminyl-tRNA ­amidotransferase ­subunit ­QRSL1COXPD40, GatA, Q9H0R6, gene:552789literature, APEX_matrix, GFP, yeast mito homolog, Rickettsial homolog, mito protein domain, coexpression, MS/MSMatrixMitochondrial central dogma > Translation > mt-tRNA synthetasesbrainstem, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
ADCK1aarF ­domain ­containing ­kinase ­1Q86TW2, gene:571439yeast mito homolog+, Rickettsial homolog, mito protein domain, MS/MSunknownnonecerebrum, spinalcord, kidney, heart, skeletalmuscle, adipose, stomach, placenta
PRDX6peroxiredoxin ­61-Cys, AOP2, HEL-S-128m, NSGPx, PRX, aiPLA2, p29, P30041, gene:95889yeast mito homolog++, Rickettsial homolog, mito protein domainunknownMetabolism > Lipid metabolism > Phospholipid metabolism;
Metabolism > Detoxification > ROS and glutathione metabolism		cerebrum, cerebellum, brainstem, spinalcord, liver, adipose, smallintestine, largeintestine, placenta, testis
NIPSNAP1nipsnap ­homolog ­1Q9BPW8, gene:85089APEX_matrix, targetP signal, coexpression, MS/MS++MatrixMitochondrial dynamics and surveillance > Mitophagy;
Mitochondrial dynamics and surveillance > Autophagy		all 14
GARS1glycyl-tRNA ­synthetase ­1CMT2D, DSMAV, GARS, GlyRS, HMN5, SMAD1, P41250, gene:26179targetP signal, yeast mito homolog, mito protein domain, inductionMatrixMitochondrial central dogma > Translation > mt-tRNA synthetasescerebrum, cerebellum, brainstem, spinalcord, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
ACSM2Aacyl-CoA ­synthetase ­medium ­chain ­family ­member ­2AA-923A4.1, ACSM2, Q08AH3, gene:1238769targetP signal, mito protein domain, MS/MS+MatrixMetabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Detoxification > Xenobiotic metabolism		kidney, liver, heart
SLC25A33solute ­carrier ­family ­25 ­member ­33BMSC-MCP, PNC1, Q9BSK2, gene:842759literature, yeast mito homolog+, mito protein domain+MIMMetabolism > Nucleotide metabolism > Nucleotide import;
Small molecule transport > SLC25A family		brainstem, spinalcord, placenta, testis
LYRM9LYR ­motif ­containing ­9C17orf108, HSD24, A8MSI8, gene:2012299targetP signal, mito protein domain+, MS/MSunknownnonecerebellum, heart, adipose, testis
RAB24RAB24, ­member ­RAS ­oncogene ­familyQ969Q5, gene:539179mito protein domain, induction, coexpression, MS/MSMOMMitochondrial dynamics and surveillance > Fission;
Mitochondrial dynamics and surveillance > Autophagy		cerebrum, cerebellum, brainstem, spinalcord, skeletalmuscle, adipose, stomach, testis
PARS2prolyl-tRNA ­synthetase ­2, ­mitochondrialEIEE75, MT-PRORS, proRS, Q7L3T8, gene:259738APEX_matrix, GFP, targetP signal, yeast mito homolog, Rickettsial homolog, mito protein domainMatrixMitochondrial central dogma > Translation > mt-tRNA synthetasessmallintestine, largeintestine, stomach
MRPL52mitochondrial ­ribosomal ­protein ­L52Q86TS9, gene:1227048literature, APEX_matrix, targetP signal, coexpression, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, spinalcord, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
PUS1pseudouridine ­synthase ­1MLASA1, Q9Y606, gene:803248APEX_matrix, targetP signal, yeast mito homolog+, mito protein domain, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications		none
GOT2glutamic-oxaloacetic ­transaminase ­2KAT4, KATIV, KYAT4, mitAAT, P00505, gene:28068literature, APEX_IMS, APEX_matrix, targetP signal, mito protein domain, MS/MS++MIMMetabolism > Carbohydrate metabolism > Malate-aspartate shuttle;
Metabolism > Amino acid metabolism > Glutamate metabolism;
Metabolism > Amino acid metabolism > Glyoxylate metabolism;
Metabolism > Amino acid metabolism > Kynurenine metabolism;
Metabolism > Sulfur metabolism		all 14
NAXDNAD(P)HX ­dehydrataseCARKD, LP3298, PEBEL2, Q8IW45, gene:557398GFP, targetP signal, mito protein domain, MS/MS++MIMMetabolism > Metals and cofactors > NAD biosynthesis and metabolismall 14
MT-ND3NADH ­dehydrogenase, ­subunit ­3 ­(complex ­I)MTND3, P03897, gene:45378literature, Rickettsial homolog, mito protein domain+, MS/MSMIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		cerebrum, cerebellum, brainstem, spinalcord, kidney, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta
ACCS1-aminocyclopropane-1-carboxylate ­synthase ­homolog ­(inactive)ACS, PHACS, Q96QU6, gene:846808yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpressionunknownnonenone
PTRH2peptidyl-tRNA ­hydrolase ­2BIT1, CFAP37, CGI-147, IMNEPD, PTH, PTH 2, PTH2, Q9Y3E5, gene:516518literature, yeast mito homolog, mito protein domain, MS/MS++MOMnoneall 14
PGS1phosphatidylglycerophosphate ­synthase ­1Q32NB8, gene:94898targetP signal, yeast mito homolog++, mito protein domainMIMMetabolism > Lipid metabolism > Cardiolipin synthesis;
Metabolism > Lipid metabolism > Phospholipid metabolism		cerebrum, spinalcord, placenta
OXR1oxidation ­resistance ­1CHEGDD, Nbla00307, TLDC3, Q8N573, gene:550748literature, yeast mito homolog+, mito protein domain, MS/MSMOMMetabolism > Detoxification > ROS and glutathione metabolismcerebrum, cerebellum, brainstem
LYPLAL1lysophospholipase ­like ­1Q96AV0, Q5VWZ2, gene:1270188mito protein domain, MS/MS++unknownMetabolism > Lipid metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, largeintestine, placenta, testis
DNAJC28DnaJ ­heat ­shock ­protein ­family ­(Hsp40) ­member ­C28C21orf55, C21orf78, Q9NX36, gene:549438Rickettsial homolog, mito protein domain, coexpression+unknownnonestomach
FPGSfolylpolyglutamate ­synthaseQ05932, gene:23568APEX_matrix, GFP, targetP signal, yeast mito homolog, Rickettsial homolog, mito protein domainMatrixMetabolism > Vitamin metabolism > Folate and 1-C metabolismnone
TMEM205transmembrane ­protein ­205UNQ501, Q6UW68, gene:3748828yeast mito homolog, mito protein domain+, coexpressionMembranenoneliver, adipose
SLC25A53solute ­carrier ­family ­25 ­member ­53MCART6, Q5H9E4, gene:4016128yeast mito homolog+, mito protein domain+MIMSmall molecule transport > SLC25A familytestis
AIFM3apoptosis ­inducing ­factor ­mitochondria ­associated ­3AIFL, Q96NN9, gene:1502098literature, yeast mito homolog, Rickettsial homolog, mito protein domain+MIMMetabolism > Metals and cofactors > Fe-S-containing proteins;
Mitochondrial dynamics and surveillance > Apoptosis		none
MGST1microsomal ­glutathione ­S-transferase ­1GST12, MGST, MGST-I, P10620, gene:42578mito protein domain, coexpression, MS/MS++MembraneMetabolism > Detoxification > Xenobiotic metabolism;
Metabolism > Detoxification > ROS and glutathione metabolism		cerebrum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
MTFP1mitochondrial ­fission ­process ­1HSPC242, MTP18, Q9UDX5, gene:515378literature, induction, coexpression, MS/MS+MembraneMitochondrial dynamics and surveillance > Fissionall 14
COA4cytochrome ­c ­oxidase ­assembly ­factor ­4 ­homologCHCHD8, CMC3, E2IG2, Q9NYJ1, gene:512878yeast mito homolog++, mito protein domain, MS/MSIMSOXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors		placenta
NIF3L1NGG1 ­interacting ­factor ­3 ­like ­1ALS2CR1, CALS-7, MDS015, Q9GZT8, gene:604918APEX_matrix, targetP signal, yeast mito homolog, coexpression, MS/MS+Matrixnonecerebrum, cerebellum, brainstem, spinalcord, skeletalmuscle, adipose, largeintestine, placenta, testis
PUSL1pseudouridine ­synthase ­like ­1Q8N0Z8, gene:1267898APEX_matrix, yeast mito homolog+, Rickettsial homolog, mito protein domainMatrixMitochondrial central dogma > Translationcerebrum, spinalcord, largeintestine, placenta
SDSLserine ­dehydratase ­likeSDH 2, SDS-RS1, TDH, cSDH, Q96GA7, gene:1136758yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpressionunknownMetabolism > Amino acid metabolism > Serine metabolismnone
BAXBCL2 ­associated ­X, ­apoptosis ­regulatorBCL2L4, Q07812, gene:5818literature, mito protein domain, coexpression, MS/MS++MOMMitochondrial dynamics and surveillance > Apoptosiscerebrum, cerebellum, brainstem, adipose, largeintestine, placenta, testis
MAVSmitochondrial ­antiviral ­signaling ­proteinCARDIF, IPS-1, IPS1, VISA, Q7Z434, gene:575068literature, induction, coexpression+, MS/MSMOMSignaling > Immune responsebrainstem, skeletalmuscle, adipose, placenta, testis
METTL17methyltransferase ­like ­17METT11D1, Q9H7H0, gene:647458GFP, yeast mito homolog++, mito protein domain+MatrixMitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications;
Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly		none
ACACBacetyl-CoA ­carboxylase ­betaACC2, ACCB, HACC275, O00763, gene:328Rickettsial homolog, mito protein domain, coexpression+MOMMetabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Vitamin metabolism > Biotin utilizing proteins		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, largeintestine, stomach, placenta, testis
ARG2arginase ­2P78540, gene:3848literature, APEX_matrix, targetP signal+, mito protein domain, MS/MSMatrixMetabolism > Amino acid metabolismkidney, adipose, smallintestine, largeintestine, placenta
MTPAPmitochondrial ­poly(A) ­polymerasePAPD1, SPAX4, TENT6, Q9NVV4, gene:551497literature, APEX_matrix, targetP signal+, mito protein domain, inductionMatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications;
Mitochondrial central dogma > mtRNA metabolism > mt-mRNA modifications;
Mitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay		cerebrum, cerebellum, spinalcord, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
CCDC51coiled-coil ­domain ­containing ­51MITOK, Q96ER9, gene:797147APEX_matrix, targetP signal, MS/MS++MIMSmall molecule transportall 14
SUGCTsuccinyl-CoA:glutarate-CoA ­transferaseC7orf10, DERP13, GA3, ORF19, Q9HAC7, gene:797837APEX_matrix, targetP signal, mito protein domain, coexpression, MS/MS+MatrixMetabolism > Amino acid metabolismkidney, liver, testis
DNM1Ldynamin ­1 ­likeDLP1, DRP1, DVLP, DYMPLE, EMPF, EMPF1, HDYNIV, OPA5, O00429, gene:100597literature, yeast mito homolog+, mito protein domain, inductionMOMMitochondrial dynamics and surveillance > Fissioncerebrum, cerebellum, brainstem, spinalcord
NRDCnardilysin ­convertaseNRD1, hNRD1, hNRD2, O43847, gene:48987targetP signal, Rickettsial homolog, mito protein domain+MatrixProtein import, sorting and homeostasis > Protein homeostasis > Chaperones;
Protein import, sorting and homeostasis > Protein import and sorting		testis
TMEM126Btransmembrane ­protein ­126BHT007, MC1DN29, Q8IUX1, gene:558637targetP signal, mito protein domain+, MS/MSMIMOXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors		cerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
NSUN4NOP2/Sun ­RNA ­methyltransferase ­4SHTAP, Q96CB9, gene:3873387APEX_matrix, targetP signal, mito protein domain, induction, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications;
Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly		placenta
ACSS3acyl-CoA ­synthetase ­short ­chain ­family ­member ­3Q9H6R3, gene:796117Rickettsial homolog, mito protein domain, MS/MSMatrixMetabolism > Lipid metabolismkidney, liver, adipose, placenta, testis
FAM210Bfamily ­with ­sequence ­similarity ­210 ­member ­B5A3, C20orf108, dJ1167H4.1, Q96KR6, gene:1161517targetP signal, yeast mito homolog, coexpression, MS/MSMIMMetabolism > Metals and cofactors > Iron homeostasiscerebrum, cerebellum, brainstem, spinalcord, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
RBFAribosome ­binding ­factor ­AC18orf22, HsT169, Q8N0V3, gene:798637APEX_matrix, targetP signal+, Rickettsial homolog, coexpressionMatrixMitochondrial central dogma > Translation > Mitochondrial ribosome assemblynone
METAP1Dmethionyl ­aminopeptidase ­type ­1D, ­mitochondrialMAP 1D, MAP1D, MetAP 1D, Metap1l, Q6UB28, gene:2540427literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, coexpressionMatrixMitochondrial central dogma > Translation > fMet processing;
Protein import, sorting and homeostasis > Protein homeostasis > Proteases		none
AHCYL1adenosylhomocysteinase ­like ­1DCAL, IRBIT, PPP1R78, PRO0233, XPVKONA, O43865, gene:107687targetP signal, mito protein domain+, inductionMOMMitochondrial dynamics and surveillance > Organelle contact sitesbrainstem, spinalcord, smallintestine, stomach
NIPSNAP3Anipsnap ­homolog ­3AHSPC299, NIPSNAP4, TASSC, Q9UFN0, gene:259347APEX_matrix, targetP signal+, MS/MS++Matrixnonecerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
MTARC1mitochondrial ­amidoxime ­reducing ­component ­1MARC1, MOSC1, Q5VT66, gene:647577mito protein domain+, MS/MSMOMMetabolism > Metals and cofactors > Molybdenum cofactor synthesis and proteins;
Metabolism > Detoxification > Xenobiotic metabolism;
Metabolism > Detoxification > Amidoxime reducing complex		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
FUNDC1FUN14 ­domain ­containing ­1Q8IVP5, gene:1393417yeast mito homolog, mito protein domain+, MS/MSMOMMitochondrial dynamics and surveillance > Mitophagy;
Mitochondrial dynamics and surveillance > Autophagy		cerebrum, cerebellum, brainstem, spinalcord, kidney, skeletalmuscle, adipose, largeintestine, stomach, testis
ACSL1acyl-CoA ­synthetase ­long ­chain ­family ­member ­1ACS1, FACL1, FACL2, LACS, LACS1, LACS2, P33121, gene:21807Rickettsial homolog, mito protein domain, coexpressionMOMMetabolism > Lipid metabolism > Fatty acid oxidationall 14
COX20cytochrome ­c ­oxidase ­assembly ­factor ­COX20FAM36A, Q5RI15, gene:1162287literature, APEX_IMS, GFP, yeast mito homolog++, MS/MSMIMOXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors		all 14
HSDL1hydroxysteroid ­dehydrogenase ­like ­1SDR12C3, Q3SXM5, gene:836937GFP, Rickettsial homolog, mito protein domain, MS/MS++unknownnonecerebrum, cerebellum, brainstem, spinalcord, adipose, smallintestine, largeintestine, stomach, placenta, testis
CBR3carbonyl ­reductase ­3HEL-S-25, SDR21C2, hCBR3, O75828, gene:8747targetP signal, Rickettsial homolog, mito protein domain, MS/MSMatrixMetabolism > Detoxification > Xenobiotic metabolismcerebrum, spinalcord, adipose, smallintestine, largeintestine, stomach
MTERF4mitochondrial ­transcription ­termination ­factor ­4MTERFD2, Q7Z6M4, gene:1309167APEX_matrix, targetP signal, mito protein domain+, coexpressionMatrixMitochondrial central dogma > Translation > Mitochondrial ribosome assemblynone
NUDT19nudix ­hydrolase ­19RP2, A8MXV4, gene:3909167APEX_matrix, targetP signal, mito protein domain, inductionMatrixMetabolism > Metals and cofactors > Coenzyme A metabolismkidney, liver, largeintestine, placenta
HEBP1heme ­binding ­protein ­1HBP, HEBP, Q9NRV9, gene:508657APEX_IMS, mito protein domain, coexpression, MS/MS++IMSnonecerebrum, cerebellum, liver, adipose, placenta
PTPMT1protein ­tyrosine ­phosphatase ­mitochondrial ­1DUSP23, MOSP, PLIP, PNAS-129, Q8WUK0, gene:1149717literature, APEX_matrix, mito protein domain, coexpression, MS/MS++MIMMetabolism > Lipid metabolism > Cardiolipin synthesis;
Metabolism > Lipid metabolism > Phospholipid metabolism		all 14
PGAM5PGAM ­family ­member ­5, ­mitochondrial ­serine/threonine ­protein ­phosphataseBXLBV68, Q96HS1, gene:1921117APEX_IMS, mito protein domain, coexpression, MS/MS++MOMMitochondrial dynamics and surveillance > Mitophagy;
Mitochondrial dynamics and surveillance > Autophagy		cerebrum, cerebellum, brainstem, spinalcord, skeletalmuscle, adipose, largeintestine, placenta, testis
DTYMKdeoxythymidylate ­kinaseCDC8, PP3731, TMPK, TYMK, P23919, gene:18417Rickettsial homolog, MS/MS+MatrixMetabolism > Nucleotide metabolism > Nucleotide synthesis and processingcerebrum, cerebellum, brainstem, spinalcord, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
KARS1lysyl-tRNA ­synthetase ­1CMTRIB, DFNB89, KARS, KARS2, KRS, Q15046, gene:37357literature, GFP, targetP signal, Rickettsial homolog, mito protein domain, coexpressionMatrixMitochondrial central dogma > Translation > mt-tRNA synthetasescerebrum, cerebellum, brainstem, spinalcord, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
STARsteroidogenic ­acute ­regulatory ­proteinSTARD1, P49675, gene:67707literature, targetP signal, mito protein domain, coexpression, MS/MSMOM, IMSMetabolism > Lipid metabolism > Cholesterol, bile acid, steroid synthesistestis
DNAJC19DnaJ ­heat ­shock ­protein ­family ­(Hsp40) ­member ­C19PAM18, TIM14, TIMM14, Q96DA6, gene:1311187literature, APEX_matrix, mito protein domain, MS/MS++MIMProtein import, sorting and homeostasis > Protein import and sorting > Import motorall 14
OTCornithine ­carbamoyltransferaseOCTD, P00480, gene:50097literature, targetP signal, mito protein domain, MS/MS+MatrixMetabolism > Amino acid metabolism > Urea cyclecerebellum, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta
COX7A2Lcytochrome ­c ­oxidase ­subunit ­7A2 ­likeCOX7AR, COX7RP, EB1, SIG81, O14548, gene:91677literature, APEX_IMS, APEX_matrix, mito protein domain+, MS/MS+MIMOXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits;
OXPHOS > OXPHOS assembly factors;
OXPHOS > Respirasome assembly		all 14
MTERF3mitochondrial ­transcription ­termination ­factor ­3CGI-12, MTERFD1, Q96E29, gene:510017literature, APEX_matrix, GFP, targetP signal, mito protein domain+, coexpressionMatrixMitochondrial central dogma > Translation > Mitochondrial ribosome assemblynone
SFXN2sideroflexin ­2SLC56A2, Q96NB2, gene:1189807mito protein domain+, coexpressionMIMSmall molecule transport > Sideroflexinskidney, liver, adipose, largeintestine, stomach, placenta, testis
FASNfatty ­acid ­synthaseFAS, OA-519, SDR27X1, P49327, gene:21947Rickettsial homolog, mito protein domain, coexpressionIMSMetabolism > Lipid metabolismcerebrum, cerebellum, brainstem, spinalcord, liver, skeletalmuscle, adipose, smallintestine, stomach, placenta, testis
PINK1PTEN ­induced ­kinase ­1BRPK, PARK6, Q9BXM7, gene:650187literature, targetP signal+, mito protein domain, coexpressionMOMMitochondrial dynamics and surveillance > Mitophagy;
Mitochondrial dynamics and surveillance > Autophagy;
Signaling		none
TMEM177transmembrane ­protein ­177Q53S58, gene:807757targetP signal, coexpression, MS/MSMIMOXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors		cerebrum, adipose, stomach, placenta, testis
TXNRD1thioredoxin ­reductase ­1GRIM-12, TR, TR1, TRXR1, TXNR, Q16881, gene:72966GFP, Rickettsial homolog, mito protein domain, coexpression, MS/MSunknownMetabolism > Detoxification > ROS and glutathione metabolism;
Metabolism > Detoxification > Selenoproteins		skeletalmuscle, smallintestine, placenta, testis
THNSL1threonine ­synthase ­like ­1TSH1, Q8IYQ7, gene:798966APEX_matrix, mito protein domain, MS/MS++Matrixnonecerebrum, cerebellum, brainstem, spinalcord, skeletalmuscle, adipose, largeintestine, stomach, testis
FDPSfarnesyl ­diphosphate ­synthaseFPPS, FPS, POROK9, P14324, gene:22246targetP signal, mito protein domain, MS/MS+MIMMetabolism > Lipid metabolism > Cholesterol-associatedliver, adipose, smallintestine, largeintestine, placenta
IDI1isopentenyl-diphosphate ­delta ­isomerase ­1IPP1, IPPI1, Q13907, gene:34226targetP signal, mito protein domain, MS/MS+MatrixMetabolism > Lipid metabolism > Cholesterol-associatedcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
TOP3ADNA ­topoisomerase ­III ­alphaMGRISCE2, PEOB5, TOP3, ZGRF7, Q13472, gene:71566APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domainMatrixMitochondrial central dogma > mtDNA maintenance > mtDNA replicationtestis
FTMTferritin ­mitochondrialMTF, Q8N4E7, gene:940336literature, targetP signal+, mito protein domain, MS/MSMatrixMetabolism > Metals and cofactors > Iron homeostasistestis
LDHAL6Blactate ­dehydrogenase ­A ­like ­6BLDH6B, LDHAL6, LDHL, Q9BYZ2, gene:924836targetP signal, Rickettsial homolog, mito protein domain, MS/MSMatrixMetabolism > Carbohydrate metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
MTFR1mitochondrial ­fission ­regulator ­1CHPPR, FAM54A2, Q15390, gene:96506literature, targetP signal, mito protein domain+, coexpressionMembraneMitochondrial dynamics and surveillance > Fissionnone
BCL2L13BCL2 ­like ­13BCL-RAMBO, Bcl2-L-13, MIL1, Q9BXK5, gene:237866literature, mito protein domain, coexpression, MS/MS+MOMMitochondrial dynamics and surveillance > Mitophagy;
Mitochondrial dynamics and surveillance > Autophagy;
Mitochondrial dynamics and surveillance > Apoptosis		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
TRMT10CtRNA ­methyltransferase ­10C, ­mitochondrial ­RNase ­P ­subunitCOXPD30, HNYA, MRPP1, RG9MTD1, Q7L0Y3, gene:549316literature, APEX_matrix, GFP, targetP signal+, mito protein domain, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > Polycistronic mtRNA processing;
Mitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications		largeintestine, placenta
ACOT7acyl-CoA ­thioesterase ­7ACH1, ACT, BACH, CTE-II, LACH, LACH1, hBACH, O00154, gene:113326targetP signal+, mito protein domain, inductionMatrixMetabolism > Lipid metabolismbrainstem, spinalcord, adipose, largeintestine
TFB1Mtranscription ­factor ­B1, ­mitochondrialCGI-75, CGI75, mtTFB, mtTFB1, Q8WVM0, gene:511066literature, APEX_matrix, Rickettsial homolog, mito protein domain, MS/MS+MatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications;
Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly		brainstem, largeintestine, placenta, testis
ARF5ADP ­ribosylation ­factor ­5P84085, gene:3816targetP signal, mito protein domain, coexpression, MS/MSunknownnonecerebrum, cerebellum, brainstem, spinalcord, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
MCUBmitochondrial ­calcium ­uniporter ­dominant ­negative ­subunit ­betaCCDC109B, Q9NWR8, gene:550136literature, targetP signal, mito protein domain+MIMSignaling > Calcium homeostasis > Calcium cycle;
Small molecule transport > Calcium uniporter		placenta
TWNKtwinkle ­mtDNA ­helicaseATXN8, C10orf2, IOSCA, MTDPS7, PEO, PEO1, PEOA3, PRLTS5, SANDO, SCA8, TWINL, Q96RR1, gene:566526literature, APEX_matrix, targetP signal, induction, coexpressionMatrixMitochondrial central dogma > mtDNA maintenance > mtDNA replication;
Mitochondrial central dogma > mtDNA maintenance > mtDNA nucleoid		none
MMADHCmetabolism ­of ­cobalamin ­associated ­DC2orf25, CL25022, cblD, Q9H3L0, gene:272496APEX_matrix, targetP signal, induction, coexpressionMatrixMetabolism > Vitamin metabolism > Vitamin B12 metabolismnone
PYCR2pyrroline-5-carboxylate ­reductase ­2HLD10, P5CR2, Q96C36, gene:299206APEX_matrix, mito protein domain, MS/MS++MatrixMetabolism > Amino acid metabolism > Proline metabolismcerebrum, cerebellum, brainstem, spinalcord, adipose, largeintestine, stomach, placenta, testis
TSTD1thiosulfate ­sulfurtransferase ­like ­domain ­containing ­1KAT, TST, Q8NFU3, gene:1001311876Rickettsial homolog, mito protein domain, MS/MSunknownMetabolism > Sulfur metabolismkidney, smallintestine, largeintestine
LYRM1LYR ­motif ­containing ­1A211C6.1, O43325, gene:571496GFP, mito protein domain+, MS/MSunknownnonebrainstem, testis
OSBPL1Aoxysterol ­binding ­protein ­like ­1AORP-1, ORP1, OSBPL1B, Q9BXW6, gene:1148766Rickettsial homolog, mito protein domain, induction, MS/MSunknownMetabolism > Lipid metabolism > Cholesterol, bile acid, steroid synthesis;
Metabolism > Lipid metabolism > Phospholipid metabolism		cerebrum, liver, largeintestine, placenta
RECQL4RecQ ­like ­helicase ­4RECQ4, O94761, gene:94016targetP signal, Rickettsial homolog, mito protein domain, coexpressionMatrixMitochondrial central dogma > mtDNA maintenance > mtDNA repairnone
PET100PET100 ­cytochrome ­c ­oxidase ­chaperoneC19orf79, P0DJ07, gene:1001318016literature, yeast mito homolog++, MS/MSMIMOXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors		cerebrum, spinalcord, kidney, liver, heart, smallintestine, largeintestine, placenta
GDAP1ganglioside ­induced ­differentiation ­associated ­protein ­1CMT4, CMT4A, CMTRIA, Q8TB36, gene:543326APEX_IMS, mito protein domain, MS/MS++MOMnonecerebrum, cerebellum, brainstem, spinalcord
MTIF3mitochondrial ­translational ­initiation ­factor ­3IF3mt, Q9H2K0, gene:2194026literature, APEX_matrix, Rickettsial homolog, mito protein domain, coexpressionMatrixMitochondrial central dogma > Translation > Translation factorslargeintestine
UNGuracil ­DNA ­glycosylaseDGU, HIGM4, HIGM5, UDG, UNG1, UNG15, UNG2, P13051, gene:73746literature, targetP signal, yeast mito homolog, mito protein domain, inductionMatrixMitochondrial central dogma > mtDNA maintenance > mtDNA repairnone
GLYATglycine-N-acyltransferaseACGNAT, CAT, GAT, Q6IB77, gene:102496literature, mito protein domain, coexpression, MS/MS+MatrixMetabolism > Amino acid metabolism > Glycine metabolism;
Metabolism > Detoxification > Xenobiotic metabolism		spinalcord, kidney, liver, placenta
ISOC2isochorismatase ­domain ­containing ­2Q96AB3, gene:797636APEX_matrix, targetP signal, MS/MS++Matrixnoneall 14
ATP5MC1ATP ­synthase ­membrane ­subunit ­c ­locus ­1ATP5A, ATP5G, ATP5G1, P05496, gene:5166literature, yeast mito homolog+, Rickettsial homolog, mito protein domainMIMOXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits		none
QDPRquinoid ­dihydropteridine ­reductaseDHPR, HDHPR, PKU2, SDR33C1, P09417, gene:58606APEX_matrix, targetP signal, mito protein domain, inductionMatrixMetabolism > Metals and cofactors > Tetrahydrobiopterin synthesis;
Metabolism > Vitamin metabolism > Folate and 1-C metabolism		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, stomach, placenta, testis
COQ8Bcoenzyme ­Q8BADCK4, NPHS9, Q96D53, gene:799346APEX_matrix, GFP, yeast mito homolog+, Rickettsial homolog, mito protein domainMIMMetabolism > Metals and cofactors > Coenzyme Q metabolismadipose, smallintestine, largeintestine, placenta
GTPBP10GTP ­binding ­protein ­10ObgH2, UG0751c10, A4D1E9, gene:858656APEX_matrix, yeast mito homolog+, Rickettsial homolog, mito protein domainMatrixMitochondrial central dogma > Translation > Mitochondrial ribosome assemblylargeintestine, placenta
TSPOtranslocator ­proteinBPBS, BZRP, DBI, IBP, MBR, PBR, PBS, PKBS, PTBR, mDRC, pk18, P30536, gene:7066literature, mito protein domain, MS/MS++MOMMetabolism > Lipid metabolism > Cholesterol, bile acid, steroid synthesisspinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
FTH1ferritin ­heavy ­chain ­1FHC, FTH, FTHL6, HFE5, PIG15, PLIF, P02794, gene:24956mito protein domain, MS/MS++unknownMetabolism > Metals and cofactors > Iron homeostasiscerebrum, cerebellum, brainstem, spinalcord, liver, adipose, smallintestine, largeintestine, stomach, placenta
TRMT1tRNA ­methyltransferase ­1MRT68, TRM1, Q9NXH9, gene:556216targetP signal+, mito protein domain, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mt-tRNA modificationsnone
ADCK2aarF ­domain ­containing ­kinase ­2AARF, Q7Z695, gene:909566GFP, targetP signal, yeast mito homolog, Rickettsial homolog, mito protein domainMIMnonenone
TSTD3thiosulfate ­sulfurtransferase ­like ­domain ­containing ­3H0UI37, gene:1001308906mito protein domain, induction, MS/MSunknownnonecerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
MRPS36mitochondrial ­ribosomal ­protein ­S36DC47, MRP-S36, P82909, gene:922596literature, APEX_matrix, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosome;
Metabolism > Carbohydrate metabolism > TCA cycle		all 14
PDP2pyruvate ­dehyrogenase ­phosphatase ­catalytic ­subunit ­2PDPC 2, PPM2B, PPM2C2, Q9P2J9, gene:575466APEX_matrix, yeast mito homolog+, mito protein domain, coexpressionMatrixMetabolism > Carbohydrate metabolism > Pyruvate metabolismadipose
AGPAT51-acylglycerol-3-phosphate ­O-acyltransferase ­51AGPAT5, LPAATE, Q9NUQ2, gene:553266literature, Rickettsial homolog, mito protein domain, induction, MS/MSMOMMetabolism > Lipid metabolism > Cardiolipin synthesis;
Metabolism > Lipid metabolism > Phospholipid metabolism		cerebellum, brainstem, spinalcord, adipose, smallintestine, largeintestine, placenta, testis
MCRIP2MAPK ­regulated ­corepressor ­interacting ­protein ­2C16orf14, FAM195A, c349E10.1, Q9BUT9, gene:843315induction, coexpression+unknownSignalingnone
UCP2uncoupling ­protein ­2BMIQ4, SLC25A8, UCPH, P55851, gene:73515literature, yeast mito homolog+, mito protein domain+MIMSmall molecule transport > SLC25A familynone
SLC25A37solute ­carrier ­family ­25 ­member ­37HT015, MFRN, MFRN1, MSC, MSCP, PRO1278, PRO1584, PRO2217, Q9NYZ2, gene:513125literature, yeast mito homolog+, mito protein domain+MIMMetabolism > Metals and cofactors > Iron homeostasis;
Small molecule transport > SLC25A family		none
SLC25A28solute ­carrier ­family ­25 ­member ­28MFRN2, MRS3/4, MRS4L, NPD016, Q96A46, gene:818945literature, yeast mito homolog+, mito protein domain+MIMMetabolism > Metals and cofactors > Iron homeostasis;
Small molecule transport > SLC25A family		none
SLC25A48solute ­carrier ­family ­25 ­member ­48Q6ZT89, gene:1533285yeast mito homolog+, mito protein domain+MIMSmall molecule transport > SLC25A familynone
SLC25A43solute ­carrier ­family ­25 ­member ­43Q8WUT9, gene:2034275yeast mito homolog+, mito protein domain+MIMSmall molecule transport > SLC25A familynone
SLC25A47solute ­carrier ­family ­25 ­member ­47C14orf68, HDMCP, HMFN1655, Q6Q0C1, gene:2836005literature, yeast mito homolog+, mito protein domain+MIMSmall molecule transport > SLC25A familynone
SLC25A41solute ­carrier ­family ­25 ­member ­41APC4, Q8N5S1, gene:2844275yeast mito homolog+, mito protein domain+MIMMetabolism > Nucleotide metabolism > Nucleotide import;
Small molecule transport > SLC25A family		none
SLC25A34solute ­carrier ­family ­25 ­member ­34Q6PIV7, gene:2847235yeast mito homolog+, mito protein domain+MIMSmall molecule transport > SLC25A familynone
EPHX2epoxide ­hydrolase ­2ABHD20, CEH, SEH, P34913, gene:20535Rickettsial homolog, mito protein domain, coexpressionunknownMetabolism > Detoxification > Xenobiotic metabolism;
Metabolism > Lipid metabolism		kidney, liver, heart, skeletalmuscle, adipose, smallintestine, stomach
NUDT5nudix ­hydrolase ­5YSA1, YSA1H, YSAH1, hNUDT5, Q9UKK9, gene:111645yeast mito homolog++, mito protein domain, coexpressionunknownMetabolism > Nucleotide metabolism > Nucleotide synthesis and processingnone
MICU1mitochondrial ­calcium ­uptake ­1CALC, CBARA1, EFHA3, MPXPS, ara CALC, Q9BPX6, gene:103675literature, APEX_IMS, GFP, targetP signal, mito protein domain, MS/MS+MIMSignaling > Calcium homeostasis > Calcium cycle;
Signaling > Calcium homeostasis > EF hand proteins;
Small molecule transport > Calcium uniporter		cerebrum, cerebellum, brainstem, spinalcord, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
SOD1superoxide ­dismutase ­1ALS, ALS1, HEL-S-44, IPOA, SOD, STAHP, hSod1, homodimer, P00441, gene:66475literature, mito protein domain, induction, coexpressionIMSMetabolism > Detoxification > ROS and glutathione metabolismall 14
SERHL2serine ­hydrolase ­like ­2dJ222E13.1, Q9H4I8, gene:2531905Rickettsial homolog, mito protein domain, MS/MSunknownnonekidney, adipose, smallintestine, largeintestine, stomach, placenta, testis
NDUFAF3NADH:ubiquinone ­oxidoreductase ­complex ­assembly ­factor ­32P1, C3orf60, E3-3, MC1DN18, Q9BU61, gene:259155literature, targetP signal, mito protein domain, MS/MSMIMOXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors		all 14
BOLA3bolA ­family ­member ­3MMDS2, Q53S33, gene:3889625targetP signal, mito protein domain, MS/MSMatrixMetabolism > Metals and cofactors > Fe-S cluster biosynthesis;
Metabolism > Metals and cofactors > Fe-S-containing proteins		cerebrum, spinalcord, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
OCIAD2OCIA ­domain ­containing ­2Q56VL3, gene:1322995MS/MS++unknownSignalingall 14
RSAD1radical ­S-adenosyl ­methionine ­domain ­containing ­1HemW, Q9HA92, gene:553165APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, coexpressionMIMMetabolism > Metals and cofactors > Fe-S-containing proteinsnone
CHCHD6coiled-coil-helix-coiled-coil-helix ­domain ­containing ­6CHCM1, MICOS25, Mic25, PPP1R23, Q9BRQ6, gene:843035literature, APEX_IMS, APEX_matrix, coexpression, MS/MS++MIMMitochondrial dynamics and surveillance > Cristae formation > MICOS complexcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
FASTKD2FAST ­kinase ­domains ­2COXPD44, KIAA0971, Q9NYY8, gene:228685literature, APEX_matrix, induction, coexpression, MS/MSMatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > Polycistronic mtRNA processing;
Mitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay;
Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly		skeletalmuscle, adipose, smallintestine, largeintestine, placenta, testis
PEX11Bperoxisomal ­biogenesis ­factor ­11 ­betaPEX11-BETA, PEX14B, O96011, gene:87995targetP signal, coexpression, MS/MSMembranenonecerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, placenta, testis
TMEM186transmembrane ­protein ­186C16orf51, Q96B77, gene:258805targetP signal, coexpression, MS/MSMIMOXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors		cerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
PIF1PIF1 ­5'-to-3' ­DNA ­helicaseC15orf20, PIF, Q9H611, gene:801195literature, targetP signal+, yeast mito homolog, mito protein domainMatrixMitochondrial central dogma > mtDNA maintenancenone
FABP1fatty ­acid ­binding ­protein ­1FABPL, L-FABP, P07148, gene:21685mito protein domain, MS/MS++unknownMetabolism > Metals and cofactors > Heme synthesis and processing;
Metabolism > Lipid metabolism		liver, smallintestine
ABCD1ATP ­binding ­cassette ­subfamily ­D ­member ­1ABC42, ALD, ALDP, AMN, P33897, gene:2155Rickettsial homolog, mito protein domain, inductionMembraneSmall molecule transport > ABC transportersadipose
PLPBPpyridoxal ­phosphate ­binding ­proteinEPVB6D, PROSC, O94903, gene:112125APEX_matrix, coexpression, MS/MS++MatrixMetabolism > Vitamin metabolism > Vitamin B6 metabolismall 14
DBIdiazepam ­binding ­inhibitor, ­acyl-CoA ­binding ­proteinACBD1, ACBP, CCK-RP, EP, P07108, gene:16225mito protein domain, induction, coexpressionunknownMetabolism > Lipid metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
PNPT1polyribonucleotide ­nucleotidyltransferase ­1COXPD13, DFNB70, OLD35, PNPASE, old-35, Q8TCS8, gene:871785literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domainMatrix, IMSMitochondrial central dogma > mtRNA metabolism > mtRNA stability and decayall 14
TOP1MTDNA ­topoisomerase ­I ­mitochondrialQ969P6, gene:1164475literature, APEX_matrix, targetP signal, mito protein domain, coexpressionMatrixMitochondrial central dogma > mtDNA maintenance > mtDNA replication;
Mitochondrial central dogma > mtRNA metabolism > Transcription		none
RNASEH1ribonuclease ­H1H1RNA, PEOB2, RNH1, O60930, gene:2462435literature, APEX_matrix, targetP signal, Rickettsial homolog, coexpressionMatrixMitochondrial central dogma > mtDNA maintenance > mtDNA replication;
Mitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay		none
ABCA9ATP ­binding ­cassette ­subfamily ­A ­member ­9EST640918, Q8IUA7, gene:103505GFP, yeast mito homolog, Rickettsial homolog, mito protein domainMembraneSmall molecule transport > ABC transporterscerebrum, placenta
SCP2sterol ­carrier ­protein ­2NLTP, NSL-TP, SCP-2, SCP-CHI, SCP-X, SCPX, P22307, gene:63425Rickettsial homolog, mito protein domain+MIMMetabolism > Lipid metabolism > Cholesterol, bile acid, steroid synthesisbrainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, placenta, testis
NT5DC35'-nucleotidase ­domain ­containing ­3TU12B1-TY, TU12B1TY, Q86UY8, gene:515595APEX_matrix, targetP signal, MS/MS++Matrixnonecerebrum, cerebellum, brainstem, spinalcord, skeletalmuscle, stomach, placenta, testis
CMC4C-X9-C ­motif ­containing ­4C6.1B, MTCP1, MTCP1B, MTCP1NB, p8, p8MTCP1, P56277, gene:1002721475yeast mito homolog++, mito protein domain+IMSnonenone
LDHBlactate ­dehydrogenase ­BHEL-S-281, LDH-B, LDH-H, LDHBD, TRG-5, P07195, gene:39455Rickettsial homolog, mito protein domain, inductionunknownMetabolism > Amino acid metabolism > Glyoxylate metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
YBEYybeY ­metalloendoribonucleaseC21orf57, P58557, gene:540595APEX_matrix, targetP signal, Rickettsial homolog, MS/MSMatrixMitochondrial central dogma > mtRNA metabolism > Polycistronic mtRNA processing;
Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly		largeintestine, placenta, testis
HINT1histidine ­triad ­nucleotide ­binding ­protein ­1HINT, NMAN, PKCI-1, PRKCNH1, P49773, gene:30945Rickettsial homolog, mito protein domain, coexpressionunknownMetabolism > Nucleotide metabolism > Nucleotide synthesis and processingcerebrum, cerebellum, brainstem, spinalcord, kidney, smallintestine, placenta, testis
MUL1mitochondrial ­E3 ­ubiquitin ­protein ­ligase ­1C1orf166, GIDE, MAPL, MULAN, RNF218, Q969V5, gene:795945literature, mito protein domain+, inductionMOMProtein import, sorting and homeostasis > Protein homeostasis;
Mitochondrial dynamics and surveillance > Fission;
Signaling		skeletalmuscle, largeintestine
TK2thymidine ­kinase ­2MTDPS2, MTTK, PEOB3, SCA31, O00142, gene:70845literature, targetP signal, mito protein domain, MS/MSMatrixMetabolism > Nucleotide metabolism > Nucleotide synthesis and processingcerebrum, cerebellum, brainstem, adipose
AGMATagmatinaseQ9BSE5, gene:798145literature, targetP signal, mito protein domain, MS/MS+MatrixMetabolism > Amino acid metabolismliver, smallintestine
CMPK2cytidine/uridine ­monophosphate ­kinase ­2NDK, TMPK2, TYKi, UMP-CMPK2, Q5EBM0, gene:1296074literature, targetP signal+, MS/MSMatrixMetabolism > Nucleotide metabolism > Nucleotide synthesis and processingspinalcord, smallintestine
BIDBH3 ­interacting ­domain ­death ­agonistFP497, P55957, gene:6374literature, targetP signal+, mito protein domain, coexpressionMOMMitochondrial dynamics and surveillance > Apoptosisnone
ARL2ADP ­ribosylation ­factor ­like ­GTPase ­2ARFL2, P36404, gene:4024literature, mito protein domain, coexpression, MS/MS+IMSMitochondrial dynamics and surveillance > Fusioncerebrum, brainstem, spinalcord, adipose, largeintestine
SYNJ2BPsynaptojanin ­2 ­binding ­proteinARIP2, OMP25, P57105, gene:553334APEX_IMS, mito protein domain, coexpression, MS/MS+MOMMitochondrial dynamics and surveillance > Organelle contact sitescerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
MFFmitochondrial ­fission ­factorC2orf33, EMPF2, GL004, Q9GZY8, gene:569474literature, induction, MS/MSMOMMitochondrial dynamics and surveillance > Fissioncerebrum, cerebellum, brainstem, spinalcord, adipose, largeintestine, placenta, testis
DCXRdicarbonyl ­and ­L-xylulose ­reductaseDCR, HCR2, HCRII, KIDCR, P34H, PNTSU, SDR20C1, XR, Q7Z4W1, gene:511814APEX_matrix, targetP signal, Rickettsial homolog, mito protein domainMatrixMetabolism > Carbohydrate metabolismcerebrum, cerebellum, spinalcord, heart, adipose, largeintestine, stomach, placenta, testis
NUDT9nudix ­hydrolase ­9NUDT10, Q9BW91, gene:533434APEX_matrix, mito protein domain, coexpression, MS/MSMatrixMetabolism > Nucleotide metabolism > Nucleotide synthesis and processingcerebrum, cerebellum, placenta, testis
AMACRalpha-methylacyl-CoA ­racemaseAMACRD, CBAS4, P504S, RACE, RM, Q9UHK6, gene:236004literature, mito protein domain, coexpression, MS/MSMatrixMetabolism > Lipid metabolism > Fatty acid oxidationkidney, liver, adipose, smallintestine, testis
VPS13Dvacuolar ­protein ­sorting ­13 ­homolog ­DSCAR4, Q5THJ4, gene:551874manual, mito protein domain, induction, coexpressionMOMMitochondrial dynamics and surveillance > Mitophagy;
Mitochondrial dynamics and surveillance > Autophagy		cerebrum, cerebellum, brainstem, spinalcord, kidney, skeletalmuscle
NUDT6nudix ­hydrolase ­6ASFGF2, FGF-AS, FGF2AS, GFG-1, GFG1, P53370, gene:111624APEX_matrix, targetP signal, mito protein domain, coexpressionMatrixnonenone
DMAC2distal ­membrane ­arm ­assembly ­complex ­2ATP5SL, Q9NW81, gene:551014APEX_matrix, GFP, targetP signal, induction, MS/MSMIMOXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors		cerebrum, cerebellum, brainstem, stomach
MRM2mitochondrial ­rRNA ­methyltransferase ­2FJH1, FTSJ2, HEL97, MTDPS17, RRMJ2, Q9UI43, gene:299604literature, Rickettsial homolog, mito protein domain, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications;
Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly		cerebrum
BAK1BCL2 ­antagonist/killer ­1BAK, BAK-LIKE, BCL2L7, CDN1, Q16611, gene:5784literature, mito protein domain, MS/MS++MOMMitochondrial dynamics and surveillance > Apoptosisbrainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
DHODHdihydroorotate ­dehydrogenase ­(quinone)DHOdehase, POADS, URA1, Q02127, gene:17234literature, mito protein domain, MS/MS++MIMMetabolism > Nucleotide metabolism > Nucleotide synthesis and processing;
Metabolism > Electron carriers > Q-linked reactions, other		cerebrum, spinalcord, kidney, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
MAOBmonoamine ­oxidase ­BP27338, gene:41294literature, mito protein domain, MS/MS++MOMMetabolism > Amino acid metabolism > Catechol metabolism;
Metabolism > Detoxification > Xenobiotic metabolism		cerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
MGST3microsomal ­glutathione ­S-transferase ­3GST-III, O14880, gene:42594APEX_IMS, mito protein domain, MS/MS++MOMMetabolism > Lipid metabolism > Eicosanoid metabolismall 14
ACP6acid ­phosphatase ­6, ­lysophosphatidicACPL1, LPAP, PACPL1, Q9NPH0, gene:512054literature, APEX_IMS, mito protein domain, MS/MS++IMSMetabolism > Lipid metabolism > Phospholipid metabolismcerebrum, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
MRPL48mitochondrial ­ribosomal ­protein ­L48CGI-118, HSPC290, L48MT, MRP-L48, Q96GC5, gene:516424literature, APEX_matrix, mito protein domain, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
AGXTalanine--glyoxylate ­and ­serine--pyruvate ­aminotransferaseAGT, AGT1, AGXT1, PH1, SPAT, SPT, TLH6, P21549, gene:1894literature, yeast mito homolog, mito protein domain, MS/MSMatrixMetabolism > Amino acid metabolism > Serine metabolism;
Metabolism > Amino acid metabolism > Glyoxylate metabolism		liver
CYB5R3cytochrome ­b5 ­reductase ­3B5R, DIA1, P00387, gene:17274literature, mito protein domain, MS/MS+MOMMetabolism > Detoxification > Xenobiotic metabolism;
Metabolism > Detoxification > Amidoxime reducing complex;
Metabolism > Lipid metabolism		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
DDX28DEAD-box ­helicase ­28MDDX28, Q9NUL7, gene:557944literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domainMatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly		spinalcord, adipose
PNKDPNKD ­metallo-beta-lactamase ­domain ­containingBRP17, DYT8, FKSG19, FPD1, KIPP1184, MR-1, MR-1S, MR1, PDC, PKND1, PNKD1, R1, TAHCCP2, Q8N490, gene:259534literature, targetP signal, mito protein domain, MS/MSMIMOXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors		cerebrum, cerebellum, spinalcord, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
MICU3mitochondrial ­calcium ­uptake ­family ­member ­3EFHA2, Q86XE3, gene:2860974manual, targetP signal, mito protein domain, MS/MSMIMSignaling > Calcium homeostasis > Calcium cycle;
Signaling > Calcium homeostasis > EF hand proteins;
Small molecule transport > Calcium uniporter		cerebrum, cerebellum, brainstem, spinalcord, skeletalmuscle, stomach
BNIP3BCL2 ­interacting ­protein ­3NIP3, Q12983, gene:6644literature, mito protein domain+, MS/MSMOMMitochondrial dynamics and surveillance > Apoptosiscerebrum, cerebellum, brainstem, heart, skeletalmuscle, adipose
MTX3metaxin ­3Q5HYI7, gene:3457784manual, mito protein domain+, MS/MSMOMMitochondrial dynamics and surveillance > Intramitochondrial membrane interactionscerebrum, cerebellum, brainstem, spinalcord
MUTYHmutY ­DNA ­glycosylaseMYH, Q9UIF7, gene:45954GFP, targetP signal+, Rickettsial homolog, mito protein domainMatrixMitochondrial central dogma > mtDNA maintenancenone
GTPBP6GTP ­binding ­protein ­6 ­(putative)PGPL, O43824, gene:82254APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domainMatrixnonenone
CYP11A1cytochrome ­P450 ­family ­11 ­subfamily ­A ­member ­1CYP11A, CYPXIA1, P450SCC, P05108, gene:15834literature, mito protein domain, MS/MS+MIMMetabolism > Lipid metabolism > Cholesterol, bile acid, steroid synthesis;
Metabolism > Metals and cofactors > Heme-containing proteins		adipose, placenta, testis
OCIAD1OCIA ­domain ­containing ­1ASRIJ, OCIA, TPA018, Q9NX40, gene:549404APEX_IMS, GFP, MS/MS++MOMnoneall 14
METTL8methyltransferase ­like ­8TIP, Q9H825, gene:798284APEX_matrix, targetP signal, Rickettsial homolog, mito protein domainMatrixMitochondrial central dogma > mtRNA metabolismnone
SMIM20small ­integral ­membrane ­protein ­20C4orf52, MITRAC7, PNX, Q8N5G0, gene:3892034literature, coexpression, MS/MSMIMOXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, placenta, testis
RPUSD4RNA ­pseudouridine ­synthase ­D4Q96CM3, gene:848814APEX_matrix, GFP, Rickettsial homolog, mito protein domain, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications;
Mitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications		placenta
SMIM8small ­integral ­membrane ­protein ­8C6orf162, dJ102H19.2, Q96KF7, gene:571504manual, induction, coexpression, MS/MSMOMnonecerebrum, cerebellum, brainstem, spinalcord, kidney, adipose, placenta, testis
NME6NME/NM23 ­nucleoside ­diphosphate ­kinase ­6IPIA-ALPHA, NDK 6, NM23-H6, O75414, gene:102013literature, APEX_matrix, GFP, Rickettsial homolog, mito protein domain, coexpressionMatrixMetabolism > Nucleotide metabolism > Nucleotide synthesis and processinglargeintestine
FKBP8FKBP ­prolyl ­isomerase ­8FKBP38, FKBPr38, Q14318, gene:237703literature, mito protein domain, coexpression, MS/MSMOMMitochondrial dynamics and surveillance > Organelle contact sites;
Mitochondrial dynamics and surveillance > Mitophagy;
Mitochondrial dynamics and surveillance > Autophagy		cerebrum, brainstem, spinalcord, largeintestine, placenta, testis
ACSM4acyl-CoA ­synthetase ­medium ­chain ­family ­member ­4P0C7M7, gene:3413923manual, targetP signal, Rickettsial homolog, mito protein domainMatrixMetabolism > Lipid metabolism > Fatty acid oxidationnone
NLRX1NLR ­family ­member ­X1CLR11.3, DLNB26, NOD26, NOD5, NOD9, Q86UT6, gene:796713APEX_matrix, targetP signal, MS/MSMatrixSignaling > Immune responseall 14
FOXRED1FAD ­dependent ­oxidoreductase ­domain ­containing ­1FP634, H17, MC1DN19, Q96CU9, gene:555723literature, mito protein domain, coexpression+MIMOXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors		adipose, largeintestine, placenta, testis
TFB2Mtranscription ­factor ­B2, ­mitochondrialHkp1, mtTFB2, Q9H5Q4, gene:642163literature, APEX_matrix, targetP signal+, mito protein domainMatrixMitochondrial central dogma > mtDNA maintenance > mtDNA replication;
Mitochondrial central dogma > mtRNA metabolism > Transcription		placenta
PPM1Kprotein ­phosphatase, ­Mg2+/Mn2+ ­dependent ­1KBDP, MSUDMV, PP2Ckappa, PP2Cm, PTMP, UG0882E07, Q8N3J5, gene:1529263literature, targetP signal+, mito protein domainMatrixMetabolism > Amino acid metabolism > BCAA metabolism;
Metabolism > Amino acid metabolism > BCAA dehydrogenase complex		cerebellum, liver, heart, adipose
ATP5MC2ATP ­synthase ­membrane ­subunit ­c ­locus ­2ATP5A, ATP5G2, Q06055, gene:5173literature, yeast mito homolog+, Rickettsial homolog, mito protein domainMIMOXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits		none
MT-ND1NADH ­dehydrogenase, ­subunit ­1 ­(complex ­I)MTND1, P03886, gene:45353literature, Rickettsial homolog, mito protein domain, MS/MSMIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		all 14
TAZtafazzinBTHS, CMD3A, EFE, EFE2, G4.5, LVNCX, Taz1, Q16635, gene:69013literature, yeast mito homolog++, mito protein domainIMSMetabolism > Lipid metabolism > Cardiolipin synthesisnone
MSRB3methionine ­sulfoxide ­reductase ­B3DFNB74, Q8IXL7, gene:2538273literature, yeast mito homolog+, mito protein domainunknownMetabolism > Detoxification > ROS and glutathione metabolismnone
ACOT11acyl-CoA ­thioesterase ­11BFIT, STARD14, THEA, THEM1, Q8WXI4, gene:260273literature, targetP signal, mito protein domain, coexpressionMatrixMetabolism > Lipid metabolism > Fatty acid oxidationnone
NIPSNAP3Bnipsnap ­homolog ­3BFP944, NIPSNAP3, SNAP1, Q9BS92, gene:553353GFP, targetP signal, MS/MS+Matrixnonecerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
NTHL1nth ­like ­DNA ­glycosylase ­1FAP3, NTH1, OCTS3, hNTH1, P78549, gene:49133literature, yeast mito homolog, Rickettsial homolog, mito protein domainunknownMetabolism > Metals and cofactors > Fe-S-containing proteinsnone
MARCHF5membrane ­associated ­ring-CH-type ­finger ­5MARCH-V, MARCH5, MITOL, RNF153, Q9NX47, gene:547083literature, mito protein domain, coexpression, MS/MSMOMMitochondrial dynamics and surveillance > Mitophagy;
Mitochondrial dynamics and surveillance > Autophagy		cerebrum, cerebellum, brainstem, spinalcord, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
SFXN4sideroflexin ­4BCRM1, COXPD18, SLC56A4, Q6P4A7, gene:1195593literature, mito protein domain+MIMSmall molecule transport > Sideroflexinscerebellum, spinalcord
TRMT5tRNA ­methyltransferase ­5COXPD26, KIAA1393, TRM5, Q32P41, gene:575703literature, yeast mito homolog, mito protein domain, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mt-tRNA modificationsnone
SLC25A6solute ­carrier ­family ­25 ­member ­6AAC3, ANT, ANT 2, ANT 3, ANT3, ANT3Y, P12236, gene:2933literature, yeast mito homolog+, mito protein domain+MIMMetabolism > Nucleotide metabolism > Nucleotide import;
Signaling > Calcium homeostasis > Mitochondrial permeability transition pore;
Small molecule transport > SLC25A family		none
SLC25A52solute ­carrier ­family ­25 ­member ­52MCART2, Q3SY17, gene:1474073manual, yeast mito homolog+, mito protein domain+MIMMetabolism > Metals and cofactors > NAD biosynthesis and metabolism;
Small molecule transport > SLC25A family		none
OGG18-oxoguanine ­DNA ­glycosylaseHMMH, HOGG1, MUTM, OGH1, O15527, gene:49683literature, targetP signal, yeast mito homolog, mito protein domainMatrixMitochondrial central dogma > mtDNA maintenance > mtDNA repairnone
ACOD1aconitate ­decarboxylase ­1CAD, IRG1, A6NK06, gene:7302493literature, yeast mito homolog, mito protein domain+MatrixMetabolism > Carbohydrate metabolism > Itaconate metabolism;
Signaling > Immune response		none
QTRT1queuine ­tRNA-ribosyltransferase ­catalytic ­subunit ­1FP3235, TGT, TGUT, Q9BXR0, gene:818903APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mt-tRNA modificationsnone
AIFM2apoptosis ­inducing ­factor ­mitochondria ­associated ­2AMID, FSP1, PRG3, Q9BRQ8, gene:848833literature, Rickettsial homolog, mito protein domain, coexpressionMembraneMetabolism > Detoxification > ROS and glutathione metabolism;
Mitochondrial dynamics and surveillance > Apoptosis		none
PRDX2peroxiredoxin ­2HEL-S-2a, NKEF-B, NKEFB, PRP, PRX2, PRXII, PTX1, TDPX1, TPX1, TSA, P32119, gene:70013GFP, Rickettsial homolog, mito protein domain, coexpressionunknownMetabolism > Detoxification > ROS and glutathione metabolismcerebrum, cerebellum, brainstem, spinalcord, adipose, smallintestine, stomach, placenta, testis
FBXL4F-box ­and ­leucine ­rich ­repeat ­protein ­4FBL4, FBL5, MTDPS13, Q9UKA2, gene:262352literature, mito protein domain, coexpressionIMSnoneadipose
GPAMglycerol-3-phosphate ­acyltransferase, ­mitochondrialGPAT, GPAT1, Q9HCL2, gene:576782literature, mito protein domain, coexpressionMembraneMetabolism > Lipid metabolism > Cardiolipin synthesis;
Metabolism > Lipid metabolism > Phospholipid metabolism		cerebellum, spinalcord, kidney, liver, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
DMAC2Ldistal ­membrane ­arm ­assembly ­complex ­2 ­likeATP5S, ATPW, FB, HSU79253, Q99766, gene:271092literature, APEX_matrix, MS/MS++MIMOXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
RMDN3regulator ­of ­microtubule ­dynamics ­3FAM82A2, FAM82C, RMD-3, RMD3, ptpip51, Q96TC7, gene:551772literature, APEX_IMS, GFP, MS/MS++MOMMitochondrial dynamics and surveillance > Organelle contact sitescerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, largeintestine, stomach, placenta, testis
PRXL2Aperoxiredoxin ­like ­2AAdrx, C10orf58, FAM213A, PAMM, Q9BRX8, gene:842932GFP, MS/MS++MembraneMetabolism > Detoxification > ROS and glutathione metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, placenta, testis
ATP5MDATP ­synthase ­membrane ­subunit ­DAPITDAPIT, HCVFTP2, MC5DN6, USMG5, bA792D24.4, Q96IX5, gene:848332GFP, MS/MS++MIMOXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits;
Mitochondrial dynamics and surveillance > Cristae formation		all 14
PET117PET117 ­cytochrome ­c ­oxidase ­chaperoneCSRP2BP, Q6UWS5, gene:1003037552literature, yeast mito homolog, MS/MSMIMOXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors		cerebrum, cerebellum, liver, adipose, largeintestine, stomach, placenta
MAOAmonoamine ­oxidase ­ABRNRS, MAO-A, P21397, gene:41282literature, mito protein domain, MS/MS+MOMMetabolism > Amino acid metabolism > Catechol metabolism;
Metabolism > Detoxification > Xenobiotic metabolism		all 14
C15orf61chromosome ­15 ­open ­reading ­frame ­61A6NNL5, gene:1458532APEX_matrix, targetP signal, MS/MSMatrixnonebrainstem, spinalcord, skeletalmuscle, adipose, largeintestine, stomach, testis
POLQDNA ­polymerase ­thetaPRO0327, O75417, gene:107212literature, targetP signal, Rickettsial homolog, mito protein domainMatrixMitochondrial central dogma > mtDNA maintenancebrainstem, testis
BCO2beta-carotene ­oxygenase ­2B-DIOX-II, BCDO2, Q9BYV7, gene:838752literature, targetP signal, mito protein domain, MS/MSMIMMetabolism > Vitamin metabolism > Vitamin A metabolismsmallintestine
IFI27interferon ­alpha ­inducible ­protein ­27FAM14D, ISG12, ISG12A, P27, P40305, gene:34292literature, induction, coexpressionMembraneSignaling > Immune response;
Mitochondrial dynamics and surveillance > Apoptosis		none
CRYZcrystallin ­zetaQ08257, gene:14292APEX_matrix, mito protein domain, MS/MSMatrixnonespinalcord, kidney, liver, adipose, placenta, testis
PYCR1pyrroline-5-carboxylate ­reductase ­1ARCL2B, ARCL3B, P5C, P5CR, PIG45, PP222, PRO3, PYCR, P32322, gene:58312literature, mito protein domain, MS/MSMatrixMetabolism > Amino acid metabolism > Proline metabolismcerebrum, cerebellum, brainstem, spinalcord, adipose, largeintestine, stomach, placenta, testis
TMLHEtrimethyllysine ­hydroxylase, ­epsilonAUTSX6, BBOX2, TMLD, TMLH, TMLHED, XAP130, Q9NVH6, gene:552172literature, APEX_matrix, mito protein domain, MS/MSMatrixMetabolism > Metals and cofactors > Carnitine synthesis and transportkidney, adipose, smallintestine, largeintestine, stomach, placenta, testis
TOMM20translocase ­of ­outer ­mitochondrial ­membrane ­20MAS20, MOM19, TOM20, Q15388, gene:98042literature, yeast mito homolog+MOMProtein import, sorting and homeostasis > Protein import and sorting > TOM;
Mitochondrial dynamics and surveillance		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
YRDCyrdC ­N6-threonylcarbamoyltransferase ­domain ­containingDRIP3, IRIP, SUA5, Q86U90, gene:796932manual, targetP signal+, Rickettsial homologMatrixMitochondrial central dogma > mtRNA metabolism > mt-tRNA modificationsnone
COA7cytochrome ­c ­oxidase ­assembly ­factor ­7 ­(putative)C1orf163, RESA1, SCAN3, SELRC1, Q96BR5, gene:652602literature, APEX_IMS, mito protein domain, coexpression, MS/MSIMSOXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
DHRS2dehydrogenase/reductase ­2HEP27, SDR25C1, Q13268, gene:102022literature, targetP signal, Rickettsial homolog, mito protein domainMatrixMetabolism > Detoxification > Xenobiotic metabolismnone
MMAAmetabolism ­of ­cobalamin ­associated ­AcblA, Q8IVH4, gene:1667852manual, coexpression+MatrixMetabolism > Vitamin metabolism > Vitamin B12 metabolismcerebrum, cerebellum, brainstem, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
MIEF1mitochondrial ­elongation ­factor ­1AltMIEF1, HSU79252, MID51, MIEF1-MP, SMCR7L, dJ1104E15.3, Q9NQG6, gene:544712literature, mito protein domain, induction, coexpressionMOM, MatrixMitochondrial central dogma > Translation > Mitochondrial ribosome assembly;
Mitochondrial dynamics and surveillance > Fission		none
C15orf48chromosome ­15 ­open ­reading ­frame ­48COXFA4L3, FOAP-11, MIR147BHG, NMES1, Q9C002, gene:844191GFP, mito protein domain, MS/MS+Membranenonesmallintestine, largeintestine, stomach, placenta, testis
LIG3DNA ­ligase ­3LIG2, LIG3alpha, P49916, gene:39801literature, targetP signal, mito protein domain, coexpressionMatrixMitochondrial central dogma > mtDNA maintenance > mtDNA replication;
Mitochondrial central dogma > mtDNA maintenance > mtDNA repair		none
BNIP3LBCL2 ­interacting ­protein ­3 ­likeBNIP3a, NIX, O60238, gene:6651literature, mito protein domain+, coexpressionMOMMitochondrial dynamics and surveillance > Apoptosisnone
FASTKD5FAST ­kinase ­domains ­5dJ1187M17.5, Q7L8L6, gene:604931APEX_matrix, targetP signal, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > Polycistronic mtRNA processing;
Mitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay		skeletalmuscle, largeintestine, placenta
PARK7Parkinsonism ­associated ­deglycaseDJ-1, DJ1, GATD2, HEL-S-67p, Q99497, gene:113151literature, mito protein domain, coexpressionMOMProtein import, sorting and homeostasis > Protein homeostasis;
Mitochondrial dynamics and surveillance > Mitophagy;
Mitochondrial dynamics and surveillance > Autophagy		all 14
COA1cytochrome ­c ­oxidase ­assembly ­factor ­1 ­homologC7orf44, MITRAC15, Q9GZY4, gene:557441APEX_IMS, targetP signal, mito protein domain+MIMOXPHOS > Complex I > CI assembly factors;
OXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors		none
COX7B2cytochrome ­c ­oxidase ­subunit ­7B2Q8TF08, gene:1707121literature, targetP signal, mito protein domain+MIMOXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits		none
MT-ND4LNADH ­dehydrogenase, ­subunit ­4L ­(complex ­I)MTND4L, P03901, gene:45391literature, Rickettsial homolog, mito protein domainMIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		heart, skeletalmuscle
SIRT4sirtuin ­4SIR2L4, Q9Y6E7, gene:234091literature, targetP signal, mito protein domainMatrixMetabolism > Metals and cofactors > NAD biosynthesis and metabolismlargeintestine
UQCC2ubiquinol-cytochrome ­c ­reductase ­complex ­assembly ­factor ­2C6orf125, C6orf126, Cbp6, M19, MC3DN7, MNF1, bA6B20.2, Q9BRT2, gene:843001APEX_matrix, GFP, coexpression, MS/MSMIMOXPHOS > Complex III > CIII assembly factors;
OXPHOS > OXPHOS assembly factors		all 14
USP30ubiquitin ­specific ­peptidase ­30Q70CQ3, gene:847491literature, targetP signal, mito protein domainMOMProtein import, sorting and homeostasis > Protein homeostasis > Proteases;
Mitochondrial dynamics and surveillance > Mitophagy;
Mitochondrial dynamics and surveillance > Autophagy		adipose, placenta, testis
PDE12phosphodiesterase ­122'-PDE, 2-PDE, Q6L8Q7, gene:2016261APEX_matrix, targetP signal, mito protein domainMatrixMitochondrial central dogma > mtRNA metabolism > mt-mRNA modifications;
Mitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay		brainstem, smallintestine, largeintestine, testis
NME3NME/NM23 ­nucleoside ­diphosphate ­kinase ­3DR-nm23, NDPK-C, NDPKC, NM23-H3, NM23H3, c371H6.2, Q13232, gene:48321GFP, Rickettsial homolog, mito protein domainMOMMetabolism > Nucleotide metabolism > Nucleotide synthesis and processingcerebrum, cerebellum, brainstem, kidney, adipose, placenta, testis
DHX30DExH-box ­helicase ­30DDX30, NEDMIAL, RETCOR, Q7L2E3, gene:229071literature, mito protein domainMatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
FASTKFas ­activated ­serine/threonine ­kinaseFAST, Q14296, gene:109221GFP, induction, coexpressionMIMMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > Polycistronic mtRNA processing;
Mitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay		none
METTL15methyltransferase ­like ­15METT5D1, A6NJ78, gene:1960741APEX_matrix, Rickettsial homolog, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mt-rRNA modificationslargeintestine, placenta
CYP24A1cytochrome ­P450 ­family ­24 ­subfamily ­A ­member ­1CP24, CYP24, HCAI, HCINF1, P450-CC24, Q07973, gene:15911literature, targetP signal+, mito protein domainMIMMetabolism > Metals and cofactors > Heme-containing proteins;
Metabolism > Vitamin metabolism > Vitamin D metabolism		none
NOCTnocturninCCR4L, CCRN4L, Ccr4c, NOC, Q9UK39, gene:258191literature, targetP signal+, mito protein domainMatrixMetabolism > Metals and cofactors > NAD biosynthesis and metabolismnone
PANK2pantothenate ­kinase ­2C20orf48, HARP, HSS, NBIA1, PKAN, Q9BZ23, gene:800251literature, targetP signal+, mito protein domainIMSMetabolism > Metals and cofactors > Coenzyme A metabolismnone
CA5Acarbonic ­anhydrase ­5ACA5, CA5AD, CAV, CAVA, GS1-21A4.1, P35218, gene:7631literature, mito protein domain, MS/MSMatrixMetabolism > Amino acid metabolism > Urea cycleliver
NT5DC25'-nucleotidase ­domain ­containing ­2Q9H857, gene:649431APEX_matrix, targetP signal, MS/MSMatrixMetabolism > Nucleotide metabolism > Nucleotide synthesis and processingspinalcord, largeintestine, placenta
ATAD3BATPase ­family ­AAA ­domain ­containing ­3BAAA-TOB3, TOB3, Q5T9A4, gene:838581APEX_IMS, APEX_matrix, GFP, Rickettsial homolog, mito protein domain+MIMMitochondrial central dogma > mtDNA maintenance > mtDNA nucleoid;
Mitochondrial dynamics and surveillance > Organelle contact sites;
Mitochondrial dynamics and surveillance > Intramitochondrial membrane interactions		none
TRUB2TruB ­pseudouridine ­synthase ­family ­member ­2CLONE24922, O95900, gene:269951APEX_matrix, Rickettsial homolog, mito protein domainMatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications		none
PRKACAprotein ­kinase ­cAMP-activated ­catalytic ­subunit ­alphaPKACA, PPNAD4, P17612, gene:55660literature, mito protein domain, coexpressionMIMSignaling > cAMP-PKA signalingcerebrum, brainstem, spinalcord, adipose, testis
COMTD1catechol-O-methyltransferase ­domain ­containing ­1Q86VU5, gene:1188810GFP, MS/MS++Membranenoneall 14
CPT1Ccarnitine ­palmitoyltransferase ­1CCATL1, CPT1-B, CPT1P, CPTI-B, CPTIC, SPG73, Q8TCG5, gene:1261290literature, yeast mito homolog+, mito protein domainMOMMetabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Metals and cofactors > Carnitine synthesis and transport;
Metabolism > Metals and cofactors > Carnitine shuttle		none
C3orf33chromosome ­3 ­open ­reading ­frame ­33AC3-33, Q6P1S2, gene:2853150APEX_matrix, targetP signal, MS/MSMatrixnoneadipose, testis
MIGA2mitoguardin ­2C9orf54, FAM73B, Q7L4E1, gene:848950literature, coexpressionMOMMitochondrial dynamics and surveillance > Fusion;
Mitochondrial dynamics and surveillance > Organelle contact sites		cerebrum, cerebellum, brainstem, spinalcord, liver, testis
SND1staphylococcal ­nuclease ­and ­tudor ­domain ­containing ­1TDRD11, Tudor-SN, p100, Q7KZF4, gene:270440GFP, yeast mito homolog, mito protein domainunknownnonecerebrum, cerebellum, brainstem, liver, smallintestine, placenta, testis
GLUD2glutamate ­dehydrogenase ­2GDH2, GLUDP1, P49448, gene:27470literature, targetP signal+, mito protein domainMatrixMetabolism > Amino acid metabolism > Glutamate metabolism;
Metabolism > Amino acid metabolism > GABA metabolism		none
HPDL4-hydroxyphenylpyruvate ­dioxygenase ­like4-HPPD-L, GLOXD1, Q96IR7, gene:848420manual, targetP signal, inductionunknownnonenone
ANGEL2angel ­homolog ­2Ccr4d, KIAA0759L, Q5VTE6, gene:908060APEX_matrix, mito protein domain, MS/MSMatrixMitochondrial central dogma > mtRNA metabolism > mt-mRNA modificationsadipose, smallintestine, placenta, testis
ANTKMTadenine ­nucleotide ­translocase ­lysine ­methyltransferaseANT-KMT, C16orf24, FAM173A, Q9BQD7, gene:659900literature, coexpression, MS/MSMIMMetabolism > Nucleotide metabolismcerebrum, cerebellum, brainstem, spinalcord, adipose, largeintestine, placenta
UQCC3ubiquinol-cytochrome ­c ­reductase ­complex ­assembly ­factor ­3C11orf83, CCDS41658.1, MC3DN9, UNQ655, Q6UW78, gene:7909550literature, coexpression, MS/MSMIMOXPHOS > Complex III > CIII assembly factors;
OXPHOS > OXPHOS assembly factors		liver, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
PRDX4peroxiredoxin ­4AOE37-2, AOE372, HEL-S-97n, PRX-4, Q13162, gene:105490APEX_IMS, Rickettsial homolog, mito protein domainIMSMetabolism > Detoxification > ROS and glutathione metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
CDK5RAP1CDK5 ­regulatory ­subunit ­associated ­protein ­1C20orf34, C42, CGI-05, HSPC167, Q96SZ6, gene:516540literature, targetP signal, Rickettsial homolog, mito protein domainMatrixMitochondrial central dogma > mtRNA metabolism > mt-tRNA modificationsnone
PAICSphosphoribosylaminoimidazole ­carboxylase ­and ­phosphoribosylaminoimidazolesuccinocarboxamide ­synthaseADE2, ADE2H1, AIRC, PAIS, P22234, gene:106060APEX_matrix, Rickettsial homolog, coexpressionMatrixMetabolism > Nucleotide metabolism > Nucleotide synthesis and processingnone
MGARPmitochondria ­localized ­glutamic ­acid ­rich ­proteinC4orf49, CESP-1, HUMMR, OSAP, Q8TDB4, gene:847090literature, APEX_IMS, targetP signal, coexpressionMembraneMitochondrial dynamics and surveillance > Traffickingnone
HDHD3haloacid ­dehalogenase ­like ­hydrolase ­domain ­containing ­32810435D12Rik, C9orf158, Q9BSH5, gene:819320APEX_matrix, MS/MS++Matrixnonecerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
CYP11B2cytochrome ­P450 ­family ­11 ­subfamily ­B ­member ­2ALDOS, CPN2, CYP11B, CYP11BL, CYPXIB2, P-450C18, P450C18, P450aldo, P19099, gene:15850literature, targetP signal, mito protein domain, coexpressionMIMMetabolism > Lipid metabolism > Cholesterol, bile acid, steroid synthesis;
Metabolism > Metals and cofactors > Heme-containing proteins		none
AGPAT41-acylglycerol-3-phosphate ­O-acyltransferase ­41-AGPAT4, LPAAT-delta, dJ473J16.2, Q9NRZ5, gene:568950literature, mito protein domain, inductionMOMMetabolism > Lipid metabolism > Cardiolipin synthesis;
Metabolism > Lipid metabolism > Phospholipid metabolism		testis
RCC1LRCC1 ­likeWBSCR16, Q96I51, gene:81554-0literature, APEX_matrix, targetP signal, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mt-rRNA modificationsplacenta
FAM185Afamily ­with ­sequence ­similarity ­185 ­member ­AQ8N0U4, gene:222234-0APEX_matrix, targetP signal, coexpressionMatrixnonenone
PMAIP1phorbol-12-myristate-13-acetate-induced ­protein ­1APR, NOXA, Q13794, gene:5366-0literature, mito protein domain+MOMMitochondrial dynamics and surveillance > Apoptosisnone
TDRKHtudor ­and ­KH ­domain ­containingTDRD2, Q9Y2W6, gene:11022-0GFP, mito protein domain, MS/MSMembranenonecerebrum, cerebellum, brainstem, spinalcord, adipose, testis
SLC8B1solute ­carrier ­family ­8 ­member ­B1NCKX6, NCLX, SLC24A6, Q6J4K2, gene:80024-0literature, mito protein domain, inductionMIMSignaling > Calcium homeostasis > Calcium cycle;
Small molecule transport		placenta
CYP27B1cytochrome ­P450 ­family ­27 ­subfamily ­B ­member ­1CP2B, CYP1, CYP1alpha, CYP27B, P450c1, PDDR, VDD1, VDDR, VDDRI, VDR, O15528, gene:1594-1literature, targetP signal, mito protein domainMIMMetabolism > Metals and cofactors > Heme-containing proteins;
Metabolism > Vitamin metabolism > Vitamin D metabolism		none
POLG2DNA ­polymerase ­gamma ­2, ­accessory ­subunitHP55, MTDPS16, MTPOLB, PEOA4, POLB, POLG-BETA, POLGB, Q9UHN1, gene:11232-1literature, APEX_matrix, targetP signal, mito protein domainMatrixMitochondrial central dogma > mtDNA maintenance > mtDNA replication;
Mitochondrial central dogma > mtDNA maintenance > mtDNA nucleoid		none
THEM5thioesterase ­superfamily ­member ­5ACOT15, Q8N1Q8, gene:284486-1literature, targetP signal, mito protein domainMatrixMetabolism > Lipid metabolismnone
BIKBCL2 ­interacting ­killerBIP1, BP4, NBK, Q13323, gene:638-1manual, mito protein domain+MOMMitochondrial dynamics and surveillance > Apoptosisnone
MTERF1mitochondrial ­transcription ­termination ­factor ­1MTERF, Q99551, gene:7978-1literature, APEX_matrix, mito protein domain+MatrixMitochondrial central dogma > mtDNA maintenance > mtDNA nucleoid;
Mitochondrial central dogma > mtRNA metabolism > Transcription		none
MTFR2mitochondrial ­fission ­regulator ­2DUFD1, FAM54A, Q6P444, gene:113115-1manual, mito protein domain+MembraneMitochondrial dynamics and surveillance > Fissionnone
GPX4glutathione ­peroxidase ­4GPx-4, GSHPx-4, MCSP, PHGPx, SMDS, snGPx, snPHGPx, P36969, gene:2879-1literature, mito protein domainMIMMetabolism > Detoxification > ROS and glutathione metabolism;
Metabolism > Detoxification > Selenoproteins		all 14
TOMM20Ltranslocase ­of ­outer ­mitochondrial ­membrane ­20 ­likeUNQ9438, Q6UXN7, gene:387990-1manual, yeast mito homolog+MOMProtein import, sorting and homeostasis > Protein import and sortingnone
COMTcatechol-O-methyltransferaseHEL-S-98n, P21964, gene:1312-1GFP, induction, coexpressionMembraneMetabolism > Amino acid metabolism > Catechol metabolismliver
MYG1MYG1 ­exonucleaseC12orf10, Gamm1, MST024, MSTP024, MYG, Q86UA3, gene:60314-1literature, mito protein domain, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications;
Mitochondrial central dogma > mtRNA metabolism > mt-mRNA modifications		none
SPATA20spermatogenesis ­associated ­20HEL-S-98, SSP411, Tisp78, Q8TB22, gene:64847-1APEX_matrix, targetP signalMatrixnonetestis
OXCT23-oxoacid ­CoA-transferase ­2FKSG25, SCOTT, Q9BYC2, gene:64064-1manual, targetP signal+, mito protein domainMatrixMetabolism > Carbohydrate metabolism > Ketone metabolismnone
CCDC127coiled-coil ­domain ­containing ­127Q96BQ5, gene:133957-1APEX_IMS, MS/MSMembranenonecerebrum, cerebellum, brainstem, spinalcord, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
CRY1cryptochrome ­circadian ­regulator ­1DSPD, PHLL1, Q16526, gene:1407-1literature, mito protein domain, coexpressionunknownnonenone
ACSM2Bacyl-CoA ­synthetase ­medium ­chain ­family ­member ­2BACSM2, HXMA, HYST1046, Q68CK6, gene:348158-1manual, targetP signal, mito protein domainMatrixMetabolism > Lipid metabolism > Fatty acid oxidationnone
MYO19myosin ­XIXMYOHD1, Q96H55, gene:80179-1literature, mito protein domainMOMMitochondrial dynamics and surveillance > Traffickingadipose
MGME1mitochondrial ­genome ­maintenance ­exonuclease ­1C20orf72, DDK1, MTDPS11, bA504H3.4, Q9BQP7, gene:92667-2literature, APEX_matrix, GFP, targetP signalMatrixMitochondrial central dogma > mtDNA maintenance > mtDNA replication;
Mitochondrial central dogma > mtDNA maintenance > mtDNA stability and decay		largeintestine, placenta
SEPTIN4septin ­4ARTS, BRADEION, C17orf47, CE5B3, H5, MART, PNUTL2, SEP4, SEPT4, hCDCREL-2, hucep-7, O43236, gene:5414-2literature, mito protein domainMOMMitochondrial dynamics and surveillance > Apoptosisbrainstem, spinalcord
EFHD1EF-hand ­domain ­family ­member ­D1MST133, MSTP133, PP3051, SWS2, Q9BUP0, gene:80303-2literature, mito protein domain, inductionMIMSignaling > Calcium homeostasis > EF hand proteinsnone
RAB5IFRAB5 ­interacting ­factorC20orf24, PNAS-11, RCAF1, RIP5, Q9BUV8, gene:55969-2GFP, induction, coexpressionMIMOXPHOS > OXPHOS assembly factors;
OXPHOS > Respirasome assembly		none
APEX1apurinic/apyrimidinic ­endodeoxyribonuclease ­1APE, APE1, APEN, APEX, APX, HAP1, REF1, P27695, gene:328-2literature, Rickettsial homolog, mito protein domainMatrixMitochondrial central dogma > mtDNA maintenance > mtDNA repairnone
SPTLC2serine ­palmitoyltransferase ­long ­chain ­base ­subunit ­2HSN1C, LCB2, LCB2A, NSAN1C, SPT2, hLCB2a, O15270, gene:9517-2GFP, Rickettsial homolog, mito protein domainMembraneMetabolism > Lipid metabolism > Phospholipid metabolismnone
DMAC1distal ­membrane ­arm ­assembly ­complex ­1C9orf123, TMEM261, Q96GE9, gene:90871-2literature, MS/MSMIMOXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors		cerebrum, spinalcord, kidney, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
ATPSCKMTATP ­synthase ­c ­subunit ­lysine ­N-methyltransferaseFAM173B, JS-2, hFAM173B, Q6P4H8, gene:134145-2literature, coexpressionMIMOXPHOS > Complex V > CV assembly factors;
OXPHOS > OXPHOS assembly factors		placenta
NEU4neuraminidase ­4Q8WWR8, gene:129807-2GFP, targetP signalMOMMetabolism > Carbohydrate metabolismnone
CASP9caspase ­9APAF-3, APAF3, ICE-LAP6, MCH6, PPP1R56, P55211, gene:842-2literature, targetP signal, mito protein domainIMSProtein import, sorting and homeostasis > Protein homeostasis > Proteases;
Mitochondrial dynamics and surveillance > Apoptosis		none
AGKacylglycerol ­kinaseCATC5, CTRCT38, MTDPS10, MULK, Q53H12, gene:55750-2literature, APEX_IMS, mito protein domainMIMProtein import, sorting and homeostasis > Protein import and sorting > TIM22 carrier pathway;
Metabolism > Lipid metabolism > Phospholipid metabolism		cerebrum, cerebellum, brainstem, spinalcord, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
MCL1MCL1 ­apoptosis ­regulator, ­BCL2 ­family ­memberBCL2L3, EAT, MCL1-ES, MCL1L, MCL1S, Mcl-1, TM, bcl2-L-3, mcl1/EAT, Q07820, gene:4170-3manual, mito protein domainMOMMitochondrial dynamics and surveillance > Apoptosisadipose, largeintestine, placenta, testis
DUS2dihydrouridine ­synthase ­2DUS2L, SMM1, URLC8, Q9NX74, gene:54920-3GFP, Rickettsial homolog, mito protein domainMatrixMitochondrial central dogma > mtRNA metabolism > mt-tRNA modificationsnone
PLD6phospholipase ­D ­family ­member ­6ZUC, Q8N2A8, gene:201164-3literature, Rickettsial homolog, mito protein domainMOMMetabolism > Lipid metabolism > Cardiolipin synthesis;
Mitochondrial dynamics and surveillance > Fusion		none
TOMM34translocase ­of ­outer ­mitochondrial ­membrane ­34HTOM34P, TOM34, URCC3, Q15785, gene:10953-3literature, mito protein domainMOMProtein import, sorting and homeostasis > Protein import and sorting > TOMtestis
PRORPprotein ­only ­RNase ­P ­catalytic ­subunitKIAA0391, MRPP3, O15091, gene:9692-3APEX_matrixMatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > Polycistronic mtRNA processing		spinalcord, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
PLSCR3phospholipid ­scramblase ­3Q9NRY6, gene:57048-3literature, mito protein domainMIMMetabolism > Lipid metabolism > Cardiolipin synthesisnone
CKMT1Bcreatine ­kinase, ­mitochondrial ­1BCKMT, CKMT1, UMTCK, 0, gene:1159-3literature, APEX_IMS, targetP signal, mito protein domainMIMMetabolism > Nucleotide metabolism > Creatine metabolismnone
NDUFB1NADH:ubiquinone ­oxidoreductase ­subunit ­B1CI-MNLL, CI-SGDH, MNLL, O75438, gene:4707-3literature, mito protein domain+MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		none
ATP5MF-PTCD1ATP5MF-PTCD1 ­readthroughATP5J2-PTCD1, B4DJ38, gene:100526740-3APEX_matrix, mito protein domain+unknownnonenone
CASP8caspase ­8ALPS2B, CAP4, Casp-8, FLICE, MACH, MCH5, Q14790, gene:841-3literature, mito protein domainMOMProtein import, sorting and homeostasis > Protein homeostasis;
Mitochondrial dynamics and surveillance > Apoptosis		none
BCL2L1BCL2 ­like ­1BCL-XL/S, BCL2L, BCLX, Bcl-X, PPP1R52, Q07817, gene:598-3literature, mito protein domainMOMMitochondrial dynamics and surveillance > Apoptosiscerebrum, cerebellum, placenta
EXD2exonuclease ­3'-5' ­domain ­containing ­2C14orf114, EXDL2, Q9NVH0, gene:55218-3literature, mito protein domainMatrixMitochondrial central dogma > Translationcerebrum, cerebellum, testis
ARMC10armadillo ­repeat ­containing ­10PNAS-112, PNAS112, PSEC0198, SVH, Q8N2F6, gene:83787-4GFP, MS/MSMOMMitochondrial dynamics and surveillance > Fission;
Mitochondrial dynamics and surveillance > Trafficking		cerebrum, cerebellum, brainstem, spinalcord, liver, adipose, largeintestine, stomach, placenta, testis
MIEF2mitochondrial ­elongation ­factor ­2MID49, SMCR7, Q96C03, gene:125170-4literature, mito protein domainMOMMitochondrial dynamics and surveillance > Fissionnone
THG1LtRNA-histidine ­guanylyltransferase ­1 ­likeICF45, IHG-1, IHG1, SCAR28, THG1, hTHG1, Q9NWX6, gene:54974-4literature, targetP signal, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mt-tRNA modificationsnone
GOLPH3golgi ­phosphoprotein ­3GOPP1, GPP34, MIDAS, Vps74, Q9H4A6, gene:64083-4literature, inductionIMSnonenone
PREPLprolyl ­endopeptidase ­likeCMS22, Q4J6C6, gene:9581-4APEX_matrix, Rickettsial homologMatrixnonespinalcord, largeintestine
FASTKD3FAST ­kinase ­domains ­3Q14CZ7, gene:79072-4APEX_matrix, targetP signalMatrixMitochondrial central dogma > mtRNA metabolism > Polycistronic mtRNA processing;
Mitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay		none
CEP89centrosomal ­protein ­89CCDC123, CEP123, Q96ST8, gene:84902-4literature, targetP signalIMSOXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors		none
GPAT2glycerol-3-phosphate ­acyltransferase ­2, ­mitochondrialCT123, Q6NUI2, gene:150763-5manual MOMMetabolism > Lipid metabolism > Cardiolipin synthesis;
Metabolism > Lipid metabolism > Phospholipid metabolism		testis
NBR1NBR1 ­autophagy ­cargo ­receptor1A1-3B, IAI3B, M17S2, MIG19, Q14596, gene:4077-5APEX_IMS, coexpressionIMSMitochondrial dynamics and surveillance > Autophagycerebellum, liver
BOKBCL2 ­family ­apoptosis ­regulator ­BOKBCL2L9, BOKL, Q9UMX3, gene:666-5manual, mito protein domainMOMMitochondrial dynamics and surveillance > Apoptosisnone
FKBP10FKBP ­prolyl ­isomerase ­10BRKS1, FKBP65, OI11, OI6, PPIASE, hFKBP65, Q96AY3, gene:60681-5APEX_IMS, mito protein domainIMSProtein import, sorting and homeostasis > Protein homeostasis > Chaperonesnone
SETD9SET ­domain ­containing ­9C5orf35, Q8NE22, gene:133383-5APEX_matrix, targetP signalMatrixnonenone
CYP11B1cytochrome ­P450 ­family ­11 ­subfamily ­B ­member ­1CPN1, CYP11B, FHI, P450C11, P15538, gene:1584-5manual, targetP signal, mito protein domainMIMMetabolism > Lipid metabolism > Cholesterol, bile acid, steroid synthesis;
Metabolism > Metals and cofactors > Heme-containing proteins		none
CSKMTcitrate ­synthase ­lysine ­methyltransferaseCS-KMT, METTL12, U99HG, A8MUP2, gene:751071-5literature, targetP signal, mito protein domainMatrixSignalingnone
MIGA1mitoguardin ­1FAM73A, Q8NAN2, gene:374986-5literatureMOMMitochondrial dynamics and surveillance > Fusioncerebrum, cerebellum, brainstem, spinalcord, smallintestine, testis
RPIAribose ­5-phosphate ­isomerase ­ARPI, RPIAD, P49247, gene:22934-5APEX_matrixMatrixMetabolism > Carbohydrate metabolismplacenta
BBC3BCL2 ­binding ­component ­3JFY-1, JFY1, PUMA, Q96PG8, gene:27113-6manual MOMMitochondrial dynamics and surveillance > Apoptosisnone
BADBCL2 ­associated ­agonist ­of ­cell ­deathBBC2, BCL2L8, Q92934, gene:572-6literature, mito protein domainMOMMitochondrial dynamics and surveillance > Apoptosisnone
BCL2BCL2 ­apoptosis ­regulatorBcl-2, PPP1R50, P10415, gene:596-6literature, mito protein domainMOMMitochondrial dynamics and surveillance > Apoptosisnone
BCL2A1BCL2 ­related ­protein ­A1ACC-1, ACC-2, ACC1, ACC2, BCL2L5, BFL1, GRS, HBPA1, Q16548, gene:597-6manual, mito protein domainMOMMitochondrial dynamics and surveillance > Apoptosisnone
BCL2L2BCL2 ­like ­2BCL-W, BCL2-L-2, BCLW, PPP1R51, Q92843, gene:599-6literature, mito protein domainMOMMitochondrial dynamics and surveillance > Apoptosisnone
CASP3caspase ­3CPP32, CPP32B, SCA-1, P42574, gene:836-6literature, mito protein domainIMSProtein import, sorting and homeostasis > Protein homeostasis > Proteases;
Mitochondrial dynamics and surveillance > Apoptosis		none
DMPKDM1 ­protein ­kinaseDM, DM1, DM1PK, DMK, MDPK, MT-PK, Q09013, gene:1760-6literature, mito protein domainMOMnonenone
DNA2DNA ­replication ­helicase/nuclease ­2DNA2L, hDNA2, P51530, gene:1763-6literature, mito protein domainMatrixMitochondrial central dogma > mtDNA maintenance > mtDNA replication;
Metabolism > Metals and cofactors > Fe-S-containing proteins		none
MT-ND6NADH ­dehydrogenase, ­subunit ­6 ­(complex ­I)MTND6, P03923, gene:4541-6literature, mito protein domainMIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		none
PRKNparkin ­RBR ­E3 ­ubiquitin ­protein ­ligaseAR-JP, LPRS2, PARK2, PDJ, O60260, gene:5071-6manual, mito protein domainMOMMitochondrial dynamics and surveillance > Mitophagy;
Mitochondrial dynamics and surveillance > Autophagy		none
ALKBH1alkB ­homolog ­1, ­histone ­H2A ­dioxygenaseABH, ABH1, ALKBH, alkB, hABH, Q13686, gene:8846-6literature, APEX_matrix, mito protein domainMatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications		none
PICK1protein ­interacting ­with ­PRKCA ­1PICK, PRKCABP, Q9NRD5, gene:9463-6literature, mito protein domainunknownnonenone
BCL2L10BCL2 ­like ­10BCL-B, Boo, Diva, bcl2-L-10, Q9HD36, gene:10017-6manual, mito protein domainMOMMitochondrial dynamics and surveillance > Apoptosisnone
AKAP10A-kinase ­anchoring ­protein ­10AKAP-10, D-AKAP-2, D-AKAP2, PRKA10, O43572, gene:11216-6literature, mito protein domainMOMSignaling > cAMP-PKA signalingnone
STYXL1serine/threonine/tyrosine ­interacting ­like ­1DUSP24, MK-STYX, MKSTYX, Q9Y6J8, gene:51657-6literature, mito protein domainMatrixMitochondrial dynamics and surveillance > Apoptosisnone
NSUN2NOP2/Sun ­RNA ­methyltransferase ­2MISU, MRT5, SAKI, TRM4, Q08J23, gene:54888-6literature, mito protein domainMatrixMitochondrial central dogma > mtRNA metabolism > mt-tRNA modificationsnone
ADCY10adenylate ­cyclase ­10HCA2, HEL-S-7a, SAC, SACI, Sacy, hsAC, Q96PN6, gene:55811-6literature, mito protein domainMembraneSignaling > cAMP-PKA signalingnone
SPHK2sphingosine ­kinase ­2SK 2, SK-2, SPK 2, SPK-2, Q9NRA0, gene:56848-6literature, mito protein domainMIMMetabolism > Lipid metabolism > Phospholipid metabolism;
Mitochondrial dynamics and surveillance > Apoptosis		none
NSUN3NOP2/Sun ­RNA ­methyltransferase ­3MST077, MSTP077, Q9H649, gene:63899-6APEX_matrix, mito protein domainMatrixMitochondrial central dogma > mtRNA metabolism > mt-tRNA modificationsnone
PABPC5poly(A) ­binding ­protein ­cytoplasmic ­5PABP5, Q96DU9, gene:140886-6literature, mito protein domainMatrixnonenone
PRSS35serine ­protease ­35C6orf158, dJ223E3.1, Q8N3Z0, gene:167681-6GFP, mito protein domainunknownProtein import, sorting and homeostasis > Protein homeostasisnone
PRIMPOLprimase ­and ­DNA ­directed ­polymeraseCCDC111, MYP22, Primpol1, Q96LW4, gene:201973-6literature, mito protein domainMatrixMitochondrial central dogma > mtDNA maintenance > mtDNA repairnone
SERAC1serine ­active ­site ­containing ­1Q96JX3, gene:84947-6literature, APEX_IMSMOMMetabolism > Lipid metabolism > Phospholipid metabolismcerebrum, cerebellum, skeletalmuscle, stomach, testis
STX17syntaxin ­17P56962, gene:55014-6APEX_IMS, coexpressionMOMMitochondrial dynamics and surveillance > Fission;
Mitochondrial dynamics and surveillance > Organelle contact sites;
Mitochondrial dynamics and surveillance > Autophagy		testis
TRMT61BtRNA ­methyltransferase ­61BQ9BVS5, gene:55006-7APEX_matrix, mito protein domainMatrixMitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications;
Mitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications;
Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly		none
PDE2Aphosphodiesterase ­2ACGS-PDE, PDE2A1, PED2A4, cGSPDE, O00408, gene:5138-7literatureMIMSignaling > cAMP-PKA signalingcerebrum, cerebellum, brainstem, largeintestine
PLGRKTplasminogen ­receptor ­with ­a ­C-terminal ­lysineAD025, C9orf46, MDS030, PLG-RKT, Plg-R(KT), Q9HBL7, gene:55848-7GFPMembranenonecerebrum, cerebellum, brainstem, spinalcord, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
ETFBKMTelectron ­transfer ­flavoprotein ­subunit ­beta ­lysine ­methyltransferaseC12orf72, ETFB-KMT, METTL20, Q8IXQ9, gene:254013-7literature, targetP signalMatrixMetabolism > Electron carriers > Q-linked reactions, othernone
RTL10retrotransposon ­Gag ­like ­10BOP, C22orf29, Q7L3V2, gene:79680-7manual, targetP signalMOMMitochondrial dynamics and surveillance > Apoptosisnone
BCL2L11BCL2 ­like ­11BAM, BIM, BOD, O43521, gene:10018-8manual MOMMitochondrial dynamics and surveillance > Apoptosisnone
SPIRE1spire ­type ­actin ­nucleation ­factor ­1Spir-1, Q08AE8, gene:56907-8literatureMOMMitochondrial dynamics and surveillance > Fission;
Mitochondrial dynamics and surveillance > Organelle contact sites		none
C2orf69chromosome ­2 ­open ­reading ­frame ­69Q8N8R5, gene:205327-8APEX_matrixMatrixnonenone
ARMCX2armadillo ­repeat ­containing ­X-linked ­2ALEX2, GASP9, Q7L311, gene:9823-10literatureMembranenonenone
ARMCX1armadillo ­repeat ­containing ­X-linked ­1ALEX1, GASP7, Q9P291, gene:51309-10literatureMOMMitochondrial dynamics and surveillance > Traffickingnone
POLBDNA ­polymerase ­betaP06746, gene:5423-10literatureMatrixMitochondrial central dogma > mtDNA maintenance > mtDNA repairnone
SNAP29synaptosome ­associated ­protein ­29CEDNIK, SNAP-29, O95721, gene:9342-10APEX_IMSIMSMitochondrial dynamics and surveillance > Autophagynone
ARMCX3armadillo ­repeat ­containing ­X-linked ­3ALEX3, GASP6, dJ545K15.2, Q9UH62, gene:51566-10literatureMOMMitochondrial dynamics and surveillance > Traffickingnone
ARMCX6armadillo ­repeat ­containing ­X-linked ­6GASP10, Q7L4S7, gene:54470-10literatureMembranenonenone
METTL4methyltransferase ­like ­4HsT661, Q8N3J2, gene:64863-10literatureMatrixMitochondrial central dogma > mtDNA maintenance > mtDNA modificationsnone
SPHKAPSPHK1 ­interactor, ­AKAP ­domain ­containingSKIP, Q2M3C7, gene:80309-10manual MIMSignaling > cAMP-PKA signalingnone
NAT8LN-acetyltransferase ­8 ­likeCML3, NACED, NAT8-LIKE, Q8N9F0, gene:339983-10literatureMembraneMetabolism > Amino acid metabolism > Glutamate metabolismnone
MCCD1mitochondrial ­coiled-coil ­domain ­1P59942, gene:401250-10manual unknownnonenone
PIGBOS1PIGB ­opposite ­strand ­1HP06981, A0A0B4J2F0, gene:101928527NAliteratureMOMMitochondrial dynamics and surveillance > Organelle contact sitesNA
HTD2hydroxyacyl-thioester ­dehydratase ­type ­2P86397, gene:109703458NAliteratureMatrixMetabolism > Lipid metabolism > Type II fatty acid synthesisNA
RP11_469A15.2RP11_469A15.20, gene:ENSG00000230623NAliteratureMIM NA
SymbolDescriptionSynonymsMaestro scoreEvidenceSub-compartmentMitoPathwaysTissues
CYC1cytochrome ­c1MC3DN6, UQCR4, P08574, gene:153743literature, APEX_IMS, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MS++MIMOXPHOS > Complex III > CIII subunits;
Metabolism > Metals and cofactors > Heme-containing proteins;
Metabolism > Electron carriers > Cytochromes;
OXPHOS > OXPHOS subunits		all 14
SDHBsuccinate ­dehydrogenase ­complex ­iron ­sulfur ­subunit ­BCWS2, IP, PGL4, SDH, SDH1, SDH2, SDHIP, P21912, gene:639043literature, APEX_matrix, targetP signal+, yeast mito homolog, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MS++MIMOXPHOS > Complex II > CII subunits;
Metabolism > Carbohydrate metabolism > TCA cycle;
Metabolism > Metals and cofactors > Fe-S-containing proteins;
OXPHOS > OXPHOS subunits		all 14
COQ7coenzyme ­Q7, ­hydroxylaseCAT5, CLK-1, CLK1, COQ10D8, Q99807, gene:1022943literature, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain+, coexpression++, MS/MS++MIMMetabolism > Metals and cofactors > Coenzyme Q metabolismall 14
SDHAsuccinate ­dehydrogenase ­complex ­flavoprotein ­subunit ­ACMD1GG, FP, PGL5, SDH1, SDH2, SDHF, P31040, gene:638942literature, APEX_matrix, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++MIMOXPHOS > Complex II > CII subunits;
Metabolism > Carbohydrate metabolism > TCA cycle;
OXPHOS > OXPHOS subunits		all 14
UQCRC1ubiquinol-cytochrome ­c ­reductase ­core ­protein ­1D3S3191, QCR1, UQCR1, P31930, gene:738442literature, APEX_matrix, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MS++MIMProtein import, sorting and homeostasis > Protein import and sorting > Preprotein cleavage;
OXPHOS > Complex III > CIII subunits;
OXPHOS > OXPHOS subunits		all 14
COQ5coenzyme ­Q5, ­methyltransferaseQ5HYK3, gene:8427442APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MS++MIMMetabolism > Metals and cofactors > Coenzyme Q metabolismall 14
PDHA1pyruvate ­dehydrogenase ­E1 ­subunit ­alpha ­1PDHA, PDHAD, PDHCE1A, PHE1A, P08559, gene:516042literature, APEX_matrix, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MS++MatrixMetabolism > Carbohydrate metabolism > Pyruvate metabolismall 14
COQ9coenzyme ­Q9C16orf49, COQ10D5, O75208, gene:5701742APEX_matrix, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain+, induction, coexpression, MS/MS++MIMMetabolism > Metals and cofactors > Coenzyme Q metabolismall 14
MRPL12mitochondrial ­ribosomal ­protein ­L125c5-2, L12mt, MRP-L31/34, MRPL7, MRPL7/L12, RPML12, P52815, gene:618242literature, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++MatrixMitochondrial central dogma > mtRNA metabolism > Transcription;
Mitochondrial central dogma > Translation > Mitochondrial ribosome		all 14
ATP5F1DATP ­synthase ­F1 ­subunit ­deltaATP5D, MC5DN5, P30049, gene:51342literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MS+MIMOXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits		all 14
COX5Acytochrome ­c ­oxidase ­subunit ­5ACOX, COX-VA, VA, P20674, gene:937742literature, APEX_IMS, APEX_matrix, targetP signal+, yeast mito homolog++, mito protein domain+, induction, coexpression++, MS/MS++MIMOXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits		all 14
ISCA2iron-sulfur ­cluster ­assembly ­2HBLD1, ISA2, MMDS4, c14_5557, Q86U28, gene:12296142APEX_matrix, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain+, induction, coexpression, MS/MS++MatrixMetabolism > Metals and cofactors > Fe-S cluster biosynthesis;
Metabolism > Metals and cofactors > Fe-S-containing proteins		all 14
PMPCBpeptidase, ­mitochondrial ­processing ­subunit ­betaBeta-MPP, MPP11, MPPB, MPPP52, P-52, O75439, gene:951241literature, APEX_matrix, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MS++MatrixProtein import, sorting and homeostasis > Protein import and sorting > Preprotein cleavage;
Protein import, sorting and homeostasis > Protein homeostasis > Proteases		all 14
UQCRFS1ubiquinol-cytochrome ­c ­reductase, ­Rieske ­iron-sulfur ­polypeptide ­1MC3DN10, RIP1, RIS1, RISP, UQCR5, P47985, gene:738640literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain+, coexpression++, MS/MS++MIMOXPHOS > Complex III > CIII subunits;
Metabolism > Metals and cofactors > Fe-S-containing proteins;
OXPHOS > OXPHOS subunits		all 14
ATP5F1AATP ­synthase ­F1 ­subunit ­alphaATP5A, ATP5A1, ATP5AL2, ATPM, COXPD22, HEL-S-123m, MC5DN4, MOM2, OMR, ORM, hATP1, P25705, gene:49840literature, APEX_matrix, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++MIMOXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits		all 14
OGDHoxoglutarate ­dehydrogenaseAKGDH, E1k, KGD1, OGDC, OGDH2, Q02218, gene:496740literature, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MS++MatrixMetabolism > Carbohydrate metabolism > TCA cycleall 14
PDHBpyruvate ­dehydrogenase ­E1 ­subunit ­betaPDHBD, PDHE1-B, PDHE1B, PHE1B, P11177, gene:516240literature, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MS++MatrixMetabolism > Carbohydrate metabolism > Pyruvate metabolismall 14
UQCRC2ubiquinol-cytochrome ­c ­reductase ­core ­protein ­2MC3DN5, QCR2, UQCR2, P22695, gene:738540literature, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MS++MIMProtein import, sorting and homeostasis > Protein import and sorting > Preprotein cleavage;
OXPHOS > Complex III > CIII subunits;
OXPHOS > OXPHOS subunits		all 14
SDHDsuccinate ­dehydrogenase ­complex ­subunit ­DCBT1, CII-4, CWS3, PGL, PGL1, QPs3, SDH4, cybS, O14521, gene:639240literature, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MS+MIMOXPHOS > Complex II > CII subunits;
Metabolism > Carbohydrate metabolism > TCA cycle;
Metabolism > Metals and cofactors > Heme-containing proteins;
OXPHOS > OXPHOS subunits		all 14
MRPS35mitochondrial ­ribosomal ­protein ­S35HDCMD11P, MDS023, MRP-S28, MRPS28, P82673, gene:6048839literature, APEX_matrix, targetP signal, yeast mito homolog++, mito protein domain+, induction, coexpression++, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
UQCRQubiquinol-cytochrome ­c ­reductase ­complex ­III ­subunit ­VIIMC3DN4, QCR8, QP-C, QPC, UQCR7, O14949, gene:2708939literature, APEX_IMS, targetP signal, yeast mito homolog++, mito protein domain+, induction, coexpression++, MS/MS++MIMOXPHOS > Complex III > CIII subunits;
OXPHOS > OXPHOS subunits		all 14
MRPL53mitochondrial ­ribosomal ­protein ­L53L53MT, Q96EL3, gene:11654039literature, APEX_matrix, targetP signal, yeast mito homolog++, mito protein domain+, induction, coexpression++, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
DBTdihydrolipoamide ­branched ­chain ­transacylase ­E2BCATE2, BCKAD-E2, BCKADE2, BCKDH-E2, BCOADC-E2, E2, E2B, P11182, gene:162939literature, APEX_matrix, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain+, induction, coexpression+, MS/MS++MatrixMetabolism > Amino acid metabolism > BCAA metabolism;
Metabolism > Amino acid metabolism > BCAA dehydrogenase complex		all 14
PDK4pyruvate ­dehydrogenase ­kinase ­4Q16654, gene:516638literature, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain+, induction, coexpression, MS/MS++MIMMetabolism > Carbohydrate metabolism > Pyruvate metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, testis
MDH2malate ­dehydrogenase ­2EIEE51, M-MDH, MDH, MGC:3559, MOR1, P40926, gene:419138literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++MatrixMetabolism > Carbohydrate metabolism > Gluconeogenesis;
Metabolism > Carbohydrate metabolism > Malate-aspartate shuttle;
Metabolism > Carbohydrate metabolism > TCA cycle		all 14
MRPS27mitochondrial ­ribosomal ­protein ­S27MRP-S27, S27mt, Q92552, gene:2310738literature, APEX_matrix, targetP signal+, yeast mito homolog++, mito protein domain+, induction, coexpression+, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
CScitrate ­synthaseO75390, gene:143137literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++MatrixMetabolism > Carbohydrate metabolism > TCA cycleall 14
GRPEL1GrpE ­like ­1, ­mitochondrialGrpE, HMGE, mt-GrpE#1, Q9HAV7, gene:8027337literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MS++MatrixProtein import, sorting and homeostasis > Protein import and sorting > Import motor;
Metabolism > Metals and cofactors > Fe-S cluster biosynthesis		all 14
DLATdihydrolipoamide ­S-acetyltransferaseDLTA, E2, PBC, PDC-E2, PDCE2, P10515, gene:173737literature, APEX_matrix, yeast mito homolog+, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MS++MatrixMetabolism > Carbohydrate metabolism > Pyruvate metabolismall 14
LRPPRCleucine ­rich ­pentatricopeptide ­repeat ­containingCLONE-23970, GP130, LRP130, LSFC, P42704, gene:1012837literature, APEX_matrix, targetP signal+, yeast mito homolog++, mito protein domain, induction, coexpression++, MS/MS++MatrixMitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay;
Mitochondrial central dogma > Translation		all 14
DLSTdihydrolipoamide ­S-succinyltransferaseDLTS, KGD2, PGL7, P36957, gene:174337literature, APEX_IMS, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MS+MatrixMetabolism > Carbohydrate metabolism > TCA cycle;
Metabolism > Amino acid metabolism > Lysine metabolism		all 14
PDHXpyruvate ­dehydrogenase ­complex ­component ­XDLDBP, E3BP, OPDX, PDHXD, PDX1, proX, O00330, gene:805037literature, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MS+MatrixMetabolism > Carbohydrate metabolism > Pyruvate metabolismall 14
GFM1G ­elongation ­factor ­mitochondrial ­1COXPD1, EFG, EFG1, EFGM, EGF1, GFM, hEFG1, mtEF-G1, Q96RP9, gene:8547637literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++MatrixMitochondrial central dogma > Translation > Translation factorsall 14
MPC2mitochondrial ­pyruvate ­carrier ­2BRP44, SLC54A2, O95563, gene:2587437literature, yeast mito homolog++, mito protein domain+, induction, coexpression++, MS/MS++MIMMetabolism > Carbohydrate metabolism > Gluconeogenesis;
Metabolism > Carbohydrate metabolism > Pyruvate metabolism;
Metabolism > Carbohydrate metabolism > TCA-associated;
Small molecule transport		all 14
NDUFS1NADH:ubiquinone ­oxidoreductase ­core ­subunit ­S1CI-75Kd, CI-75k, MC1DN5, PRO1304, P28331, gene:471936literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
Metabolism > Metals and cofactors > Fe-S-containing proteins;
OXPHOS > OXPHOS subunits		all 14
MRPL46mitochondrial ­ribosomal ­protein ­L46C15orf4, LIECG2, P2ECSL, Q9H2W6, gene:2658936literature, APEX_matrix, targetP signal+, yeast mito homolog++, mito protein domain, induction, coexpression++, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, placenta, testis
ATP5F1EATP ­synthase ­F1 ­subunit ­epsilonATP5E, ATPE, MC5DN3, P56381, gene:51436literature, yeast mito homolog++, mito protein domain+, induction, coexpression++, MS/MS++MIMOXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits		all 14
SLC25A3solute ­carrier ­family ­25 ­member ­3OK/SW-cl.48, PHC, PTP, Q00325, gene:525036literature, yeast mito homolog++, mito protein domain+, induction, coexpression++, MS/MS++MIMMetabolism > Metals and cofactors > Copper metabolism;
Signaling > Calcium homeostasis > Calcium cycle;
Small molecule transport > SLC25A family		all 14
MRPS23mitochondrial ­ribosomal ­protein ­S23CGI-138, COXPD46, HSPC329, MRP-S23, Q9Y3D9, gene:5164936literature, APEX_matrix, yeast mito homolog++, mito protein domain+, induction, coexpression++, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
FHfumarate ­hydrataseFMRD, HLRCC, HsFH, LRCC, MCL, MCUL1, P07954, gene:227135literature, APEX_matrix, targetP signal+, yeast mito homolog, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++MatrixMetabolism > Carbohydrate metabolism > TCA cycleall 14
PMPCApeptidase, ­mitochondrial ­processing ­subunit ­alphaAlpha-MPP, CLA1, CPD3, INPP5E, P-55, SCAR2, Q10713, gene:2320335literature, APEX_matrix, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain+, coexpression, MS/MS++MatrixProtein import, sorting and homeostasis > Protein import and sorting > Preprotein cleavage;
Protein import, sorting and homeostasis > Protein homeostasis > Proteases		all 14
ATP5F1BATP ­synthase ­F1 ­subunit ­betaATP5B, ATPMB, ATPSB, HEL-S-271, P06576, gene:50635literature, APEX_matrix, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression++, MS/MS++MIMOXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits		all 14
SDHAF4succinate ­dehydrogenase ­complex ­assembly ­factor ­4C6orf57, Sdh8, Q5VUM1, gene:13515435yeast mito homolog++, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MSMatrixOXPHOS > Complex II > CII assembly factors;
OXPHOS > OXPHOS assembly factors		adipose, testis
UQCR10ubiquinol-cytochrome ­c ­reductase, ­complex ­III ­subunit ­XHSPC051, HSPC119, HSPC151, QCR9, UCCR7.2, UCRC, Q9UDW1, gene:2979635literature, yeast mito homolog++, mito protein domain+, induction, coexpression++, MS/MS++MIMOXPHOS > Complex III > CIII subunits;
OXPHOS > OXPHOS subunits		all 14
ISCA1iron-sulfur ­cluster ­assembly ­1HBLD2, ISA1, MMDS5, hIscA, Q9BUE6, gene:8168935APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain+, induction, MS/MS++MatrixMetabolism > Metals and cofactors > Fe-S cluster biosynthesis;
Metabolism > Metals and cofactors > Fe-S-containing proteins		cerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
SUCLA2succinate-CoA ­ligase ­ADP-forming ­subunit ­betaA-BETA, A-SCS, LINC00444, MTDPS5, SCS-betaA, Q9P2R7, gene:880335literature, APEX_matrix, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain, induction, coexpression+, MS/MS++MatrixMetabolism > Carbohydrate metabolism > TCA cycle;
Metabolism > Carbohydrate metabolism > Itaconate metabolism;
Metabolism > Nucleotide metabolism > Nucleotide synthesis and processing		all 14
COQ3coenzyme ­Q3, ­methyltransferaseDHHBMT, DHHBMTASE, UG0215E05, bA9819.1, Q9NZJ6, gene:5180534APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain+, induction, coexpression+, MS/MS+MIMMetabolism > Metals and cofactors > Coenzyme Q metabolismcerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
IARS2isoleucyl-tRNA ­synthetase ­2, ­mitochondrialCAGSSS, ILERS, Q9NSE4, gene:5569934APEX_matrix, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression++, MS/MS++MatrixMitochondrial central dogma > Translation > mt-tRNA synthetasesall 14
MRPS15mitochondrial ­ribosomal ­protein ­S15DC37, MPR-S15, RPMS15, S15mt, P82914, gene:6496034literature, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression++, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
IDH3Aisocitrate ­dehydrogenase ­(NAD(+)) ­3 ­catalytic ­subunit ­alphaP50213, gene:341934APEX_matrix, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++MatrixMetabolism > Carbohydrate metabolism > TCA cycleall 14
COX11cytochrome ­c ­oxidase ­copper ­chaperone ­COX11COX11P, Q9Y6N1, gene:135334literature, APEX_IMS, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain+, induction, coexpression, MS/MSMIMOXPHOS > Complex IV > CIV assembly factors;
Metabolism > Metals and cofactors > Copper metabolism;
OXPHOS > OXPHOS assembly factors		cerebrum, cerebellum, spinalcord, skeletalmuscle, largeintestine, placenta
ETFDHelectron ­transfer ­flavoprotein ­dehydrogenaseETFQO, MADD, Q16134, gene:211034literature, APEX_matrix, yeast mito homolog++, mito protein domain+, induction, coexpression++, MS/MS++MIMMetabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Amino acid metabolism > BCAA metabolism;
Metabolism > Amino acid metabolism > Lysine metabolism;
Metabolism > Amino acid metabolism > Glycine metabolism;
Metabolism > Metals and cofactors > Fe-S-containing proteins;
Metabolism > Vitamin metabolism > Choline and betaine metabolism;
Metabolism > Electron carriers > Q-linked reactions, other		all 14
TIMM10translocase ­of ­inner ­mitochondrial ­membrane ­10TIM10, TIM10A, TIMM10A, P62072, gene:2651934literature, APEX_IMS, yeast mito homolog++, mito protein domain+, induction, coexpression++, MS/MS++MIMProtein import, sorting and homeostasis > Protein homeostasis > Chaperones;
Protein import, sorting and homeostasis > Protein import and sorting		cerebrum, cerebellum, brainstem, spinalcord, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
MRPL34mitochondrial ­ribosomal ­protein ­L34L34mt, Q9BQ48, gene:6498134literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
MRPL2mitochondrial ­ribosomal ­protein ­L2CGI-22, MRP-L14, RPML14, Q5T653, gene:5106934literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomespinalcord, liver, heart, adipose, smallintestine, largeintestine, placenta, testis
BCKDHAbranched ­chain ­keto ­acid ­dehydrogenase ­E1 ­subunit ­alphaBCKDE1A, MSU, MSUD1, OVD1A, P12694, gene:59334literature, APEX_matrix, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain+, coexpression+, MS/MS++MIMMetabolism > Amino acid metabolism > BCAA metabolism;
Metabolism > Amino acid metabolism > BCAA dehydrogenase complex		cerebrum, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
UQCRHubiquinol-cytochrome ­c ­reductase ­hinge ­proteinQCR6, UQCR8, P07919, gene:738834literature, APEX_IMS, yeast mito homolog++, mito protein domain+, induction, coexpression++, MS/MS++MIMOXPHOS > Complex III > CIII subunits;
OXPHOS > OXPHOS subunits		all 14
HIGD2AHIG1 ­hypoxia ­inducible ­domain ­family ­member ­2ARCF1b, Q9BW72, gene:19228634APEX_IMS, yeast mito homolog++, mito protein domain+, induction, coexpression++, MS/MS++MIMOXPHOS > OXPHOS assembly factors;
OXPHOS > Respirasome assembly		all 14
ATP5POATP ­synthase ­peripheral ­stalk ­subunit ­OSCPATP5O, ATPO, HMC08D05, OSCP, P48047, gene:53934literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain+, induction, MS/MS++MIMOXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits		all 14
ECHS1enoyl-CoA ­hydratase, ­short ­chain ­1ECHS1D, SCEH, P30084, gene:189233literature, APEX_matrix, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain, induction, coexpression+, MS/MS++MatrixMetabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Amino acid metabolism > BCAA metabolism;
Metabolism > Amino acid metabolism > Lysine metabolism		all 14
LETMD1LETM1 ­domain ­containing ­11110019O13Rik, HCCR, HCCR-1, HCCR-2, HCCR1, HCCR2, HCRR-2, SLC55A3, Q6P1Q0, gene:2587533GFP, targetP signal+, yeast mito homolog+, mito protein domain+, induction, coexpression, MS/MS++MembraneSmall molecule transportcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, placenta, testis
COX6A1cytochrome ­c ­oxidase ­subunit ­6A1CMTRID, COX6A, COX6AL, P12074, gene:133733literature, targetP signal+, yeast mito homolog+, mito protein domain+, induction, coexpression, MS/MS++MIMOXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits		all 14
COX15cytochrome ­c ­oxidase ­assembly ­homolog ­COX15CEMCOX2, Q7KZN9, gene:135533literature, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain+, coexpression++, MS/MSMIMOXPHOS > Complex IV > CIV assembly factors;
Metabolism > Metals and cofactors > Heme synthesis and processing;
OXPHOS > OXPHOS assembly factors		cerebrum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
AFG3L2AFG3 ­like ­matrix ­AAA ­peptidase ­subunit ­2OPA12, SCA28, SPAX5, Q9Y4W6, gene:1093933literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MS++MIMProtein import, sorting and homeostasis > Protein homeostasis > Proteasesall 14
HADHAhydroxyacyl-CoA ­dehydrogenase ­trifunctional ­multienzyme ­complex ­subunit ­alphaECHA, GBP, HADH, LCEH, LCHAD, MTPA, TP-ALPHA, P40939, gene:303033literature, APEX_matrix, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpression++, MS/MS++MIMMetabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Lipid metabolism > Cardiolipin synthesis;
Metabolism > Amino acid metabolism > BCAA metabolism		all 14
ETFAelectron ­transfer ­flavoprotein ­subunit ­alphaEMA, GA2, MADD, P13804, gene:210833literature, APEX_matrix, targetP signal, yeast mito homolog++, mito protein domain, induction, coexpression++, MS/MS++MatrixMetabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Amino acid metabolism > BCAA metabolism;
Metabolism > Amino acid metabolism > Lysine metabolism;
Metabolism > Amino acid metabolism > Glycine metabolism;
Metabolism > Vitamin metabolism > Choline and betaine metabolism;
Metabolism > Electron carriers > Q-linked reactions, other		all 14
NDUFS7NADH:ubiquinone ­oxidoreductase ­core ­subunit ­S7CI-20, CI-20KD, MC1DN3, MY017, PSST, O75251, gene:37429133literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
Metabolism > Metals and cofactors > Fe-S-containing proteins;
OXPHOS > OXPHOS subunits		all 14
CPT2carnitine ­palmitoyltransferase ­2CPT1, CPTASE, IIAE4, P23786, gene:137632literature, APEX_matrix, targetP signal+, yeast mito homolog++, mito protein domain, induction, coexpression+, MS/MS++MIMMetabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Metals and cofactors > Carnitine synthesis and transport;
Metabolism > Metals and cofactors > Carnitine shuttle		all 14
BCKDHBbranched ­chain ­keto ­acid ­dehydrogenase ­E1 ­subunit ­betaBCKDE1B, BCKDH E1-beta, E1B, P21953, gene:59432literature, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain+, coexpression+, MS/MS++MIMMetabolism > Amino acid metabolism > BCAA metabolism;
Metabolism > Amino acid metabolism > BCAA dehydrogenase complex		cerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
IDH3Bisocitrate ­dehydrogenase ­(NAD(+)) ­3 ­non-catalytic ­subunit ­betaRP46, O43837, gene:342032literature, APEX_matrix, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS++MatrixMetabolism > Carbohydrate metabolism > TCA cycleall 14
LARS2leucyl-tRNA ­synthetase ­2, ­mitochondrialHLASA, LEURS, PRLTS4, mtLeuRS, Q15031, gene:2339532literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression++, MS/MS++MatrixMitochondrial central dogma > Translation > mt-tRNA synthetasesall 14
ACADSacyl-CoA ­dehydrogenase ­short ­chainACAD3, SCAD, P16219, gene:3532literature, APEX_matrix, targetP signal+, mito protein domain+, induction, coexpression++, MS/MS++MatrixMetabolism > Lipid metabolism > Fatty acid oxidationall 14
LETM1leucine ­zipper ­and ­EF-hand ­containing ­transmembrane ­protein ­1SLC55A1, O95202, gene:395432literature, APEX_IMS, APEX_matrix, targetP signal, yeast mito homolog+, mito protein domain+, coexpression++, MS/MS++MIMSignaling > Calcium homeostasis > Calcium cycleall 14
ATP5MEATP ­synthase ­membrane ­subunit ­eATP5I, ATP5K, P56385, gene:52132literature, APEX_IMS, yeast mito homolog++, mito protein domain+, coexpression++, MS/MS++MIMOXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits;
Mitochondrial dynamics and surveillance > Cristae formation		all 14
OPA1OPA1 ­mitochondrial ­dynamin ­like ­GTPaseBERHS, MGM1, MTDPS14, NPG, NTG, largeG, O60313, gene:497632literature, APEX_IMS, targetP signal, yeast mito homolog++, mito protein domain, induction, coexpression++, MS/MS++MIMMitochondrial dynamics and surveillance > Fusion;
Mitochondrial dynamics and surveillance > Cristae formation		all 14
AUHAU ­RNA ­binding ­methylglutaconyl-CoA ­hydrataseQ13825, gene:54932literature, APEX_matrix, yeast mito homolog+, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++MatrixMetabolism > Carbohydrate metabolism > Itaconate metabolism;
Metabolism > Amino acid metabolism > BCAA metabolism		all 14
SUCLG1succinate-CoA ­ligase ­GDP/ADP-forming ­subunit ­alphaGALPHA, MTDPS9, SUCLA1, P53597, gene:880232yeast mito homolog+, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++MatrixMetabolism > Carbohydrate metabolism > TCA cycle;
Metabolism > Carbohydrate metabolism > Itaconate metabolism;
Metabolism > Nucleotide metabolism > Nucleotide synthesis and processing		all 14
NDUFV2NADH:ubiquinone ­oxidoreductase ­core ­subunit ­V2CI-24k, MC1DN7, P19404, gene:472932literature, APEX_matrix, targetP signal+, Rickettsial homolog, induction, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
Metabolism > Metals and cofactors > Fe-S-containing proteins;
OXPHOS > OXPHOS subunits		all 14
COQ6coenzyme ­Q6, ­monooxygenaseCGI-10, CGI10, COQ10D6, Q9Y2Z9, gene:5100432literature, APEX_matrix, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression+, MS/MS+MIMMetabolism > Metals and cofactors > Coenzyme Q metabolismcerebrum, cerebellum, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
MRPL43mitochondrial ­ribosomal ­protein ­L43L43mt, MRP-L43, bMRP36a, Q8N983, gene:8454531literature, targetP signal+, yeast mito homolog++, mito protein domain, induction, coexpression, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
ABHD11abhydrolase ­domain ­containing ­11PP1226, WBSCR21, Q8NFV4, gene:8345131literature, APEX_matrix, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression+, MS/MS++MatrixMetabolism > Carbohydrate metabolism > TCA-associatedcerebrum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
ATP5PFATP ­synthase ­peripheral ­stalk ­subunit ­F6ATP5, ATP5A, ATP5J, ATPM, CF6, F6, P18859, gene:52231literature, APEX_IMS, targetP signal+, mito protein domain+, induction, coexpression++, MS/MS++MIMOXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits		all 14
NDUFB8NADH:ubiquinone ­oxidoreductase ­subunit ­B8ASHI, CI-ASHI, MC1DN32, O95169, gene:471431literature, APEX_IMS, APEX_matrix, targetP signal+, mito protein domain+, induction, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		all 14
LONP1lon ­peptidase ­1, ­mitochondrialCODASS, LON, LONP, LonHS, PIM1, PRSS15, hLON, P36776, gene:936131literature, APEX_matrix, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS++MatrixProtein import, sorting and homeostasis > Protein homeostasis > Proteasesall 14
DLDdihydrolipoamide ­dehydrogenaseDLDD, DLDH, E3, GCSL, LAD, OGDC-E3, PHE3, P09622, gene:173831literature, APEX_matrix, targetP signal, yeast mito homolog, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++MatrixMetabolism > Carbohydrate metabolism > Pyruvate metabolism;
Metabolism > Carbohydrate metabolism > TCA cycle;
Metabolism > Amino acid metabolism > BCAA metabolism;
Metabolism > Amino acid metabolism > BCAA dehydrogenase complex;
Metabolism > Amino acid metabolism > Lysine metabolism;
Metabolism > Amino acid metabolism > Glycine metabolism;
Metabolism > Amino acid metabolism > Glycine cleavage system		all 14
AIFM1apoptosis ­inducing ­factor ­mitochondria ­associated ­1AIF, AUNX1, CMT2D, CMTX4, COWCK, COXPD6, DFNX5, NADMR, NAMSD, PDCD8, SEMDHL, O95831, gene:913131literature, APEX_IMS, targetP signal, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++MIMProtein import, sorting and homeostasis > Protein import and sorting > MIA40;
OXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors;
Mitochondrial dynamics and surveillance > Apoptosis		all 14
ECHDC3enoyl-CoA ­hydratase ­domain ­containing ­3Q96DC8, gene:7974631targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS++Matrixnonespinalcord, kidney, liver, heart, adipose, largeintestine, stomach, placenta, testis
APOOLapolipoprotein ­O ­likeCXorf33, FAM121A, MICOS27, Mic27, UNQ8193, Q6UXV4, gene:13932231literature, yeast mito homolog, mito protein domain+, induction, coexpression++, MS/MS++MIMMitochondrial dynamics and surveillance > Cristae formation > MICOS complexall 14
MRPL10mitochondrial ­ribosomal ­protein ­L10L10MT, MRP-L10, MRP-L8, MRPL8, RPML8, Q7Z7H8, gene:12499531literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression+, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
MRRFmitochondrial ­ribosome ­recycling ­factorMRFF, MTRRF, RRF, Q96E11, gene:9239930literature, APEX_matrix, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMitochondrial central dogma > Translation > Translation factorsall 14
NDUFS8NADH:ubiquinone ­oxidoreductase ­core ­subunit ­S8CI-23k, CI23KD, MC1DN2, TYKY, O00217, gene:472830literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
Metabolism > Metals and cofactors > Fe-S-containing proteins;
OXPHOS > OXPHOS subunits		all 14
ACADMacyl-CoA ­dehydrogenase ­medium ­chainACAD1, MCAD, MCADH, P11310, gene:3430literature, APEX_matrix, targetP signal, mito protein domain+, induction, coexpression++, MS/MS++MatrixMetabolism > Lipid metabolism > Fatty acid oxidationall 14
IMMTinner ­membrane ­mitochondrial ­proteinHMP, MICOS60, MINOS2, Mic60, P87, P87/89, P89, PIG4, PIG52, Q16891, gene:1098930literature, targetP signal, yeast mito homolog, mito protein domain+, coexpression++, MS/MS++MIMMitochondrial dynamics and surveillance > Cristae formation > MICOS complexall 14
TIMM9translocase ­of ­inner ­mitochondrial ­membrane ­9TIM9, TIM9A, Q9Y5J7, gene:2652030literature, APEX_IMS, yeast mito homolog++, mito protein domain+, induction, coexpression+, MS/MS++MIMProtein import, sorting and homeostasis > Protein homeostasis > Chaperones;
Protein import, sorting and homeostasis > Protein import and sorting		all 14
SLC25A4solute ­carrier ­family ­25 ­member ­4AAC1, ANT, ANT 1, ANT1, MTDPS12, MTDPS12A, PEO2, PEO3, PEOA2, T1, P12235, gene:29130literature, yeast mito homolog++, mito protein domain+, induction, coexpression, MS/MS++MIMMetabolism > Nucleotide metabolism > Nucleotide import;
Signaling > Calcium homeostasis > Mitochondrial permeability transition pore;
Small molecule transport > SLC25A family		all 14
SAMM50SAMM50 ­sorting ­and ­assembly ­machinery ­componentCGI-51, OMP85, SAM50, TOB55, TRG-3, YNL026W, Q9Y512, gene:2581330literature, APEX_IMS, APEX_matrix, yeast mito homolog++, Rickettsial homolog, induction, coexpression++, MS/MS++MOMProtein import, sorting and homeostasis > Protein import and sorting > SAM;
Mitochondrial dynamics and surveillance > Intramitochondrial membrane interactions		all 14
NDUFS2NADH:ubiquinone ­oxidoreductase ­core ­subunit ­S2CI-49, MC1DN6, O75306, gene:472030literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
Metabolism > Metals and cofactors > Fe-S-containing proteins;
OXPHOS > OXPHOS subunits		all 14
NDUFV1NADH:ubiquinone ­oxidoreductase ­core ­subunit ­V1CI-51K, CI51KD, MC1DN4, UQOR1, P49821, gene:472330literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
Metabolism > Metals and cofactors > Fe-S-containing proteins;
OXPHOS > OXPHOS subunits		all 14
ACO2aconitase ­2ACONM, HEL-S-284, ICRD, OCA8, OPA9, Q99798, gene:5030literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++MatrixMetabolism > Carbohydrate metabolism > TCA cycle;
Metabolism > Metals and cofactors > Fe-S-containing proteins		all 14
SUPV3L1Suv3 ­like ­RNA ­helicaseSUV3, Q8IYB8, gene:683230literature, APEX_matrix, targetP signal+, yeast mito homolog++, mito protein domain, induction, coexpression+, MS/MS+MatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
FECHferrochelataseEPP, EPP1, FCE, P22830, gene:223530literature, APEX_matrix, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MIMMetabolism > Metals and cofactors > Heme synthesis and processing;
Metabolism > Metals and cofactors > Fe-S-containing proteins		all 14
MTIF2mitochondrial ­translational ­initiation ­factor ­2P46199, gene:452830literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MSMatrixMitochondrial central dogma > Translation > Translation factorsall 14
PHBprohibitinHEL-215, HEL-S-54e, PHB1, P35232, gene:524529literature, yeast mito homolog+, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++MIMProtein import, sorting and homeostasis > Protein homeostasis > Chaperonesall 14
HIBCH3-hydroxyisobutyryl-CoA ­hydrolaseHIBYLCOAH, Q6NVY1, gene:2627529literature, APEX_matrix, targetP signal+, yeast mito homolog+, mito protein domain, induction, coexpression, MS/MS++MatrixMetabolism > Amino acid metabolism > BCAA metabolismall 14
MRPS2mitochondrial ­ribosomal ­protein ­S2CGI-91, COXPD36, MRP-S2, S2mt, Q9Y399, gene:5111629literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression+, MS/MS+MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, brainstem, spinalcord, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
HSPA9heat ­shock ­protein ­family ­A ­(Hsp70) ­member ­9CRP40, CSA, EVPLS, GRP-75, GRP75, HEL-S-124m, HSPA9B, MOT, MOT2, MTHSP75, PBP74, SAAN, SIDBA4, P38646, gene:331329APEX_matrix, targetP signal+, yeast mito homolog, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS++MatrixMetabolism > Metals and cofactors > Fe-S cluster biosynthesisall 14
SURF1SURF1 ­cytochrome ­c ­oxidase ­assembly ­factorCMT4K, Q15526, gene:683429literature, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain+, coexpression, MS/MSMIMOXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
PRDX3peroxiredoxin ­3AOP-1, AOP1, HBC189, MER5, PRO1748, SP-22, prx-III, P30048, gene:1093529literature, APEX_IMS, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++MatrixMetabolism > Detoxification > ROS and glutathione metabolismall 14
GHITMgrowth ­hormone ­inducible ­transmembrane ­proteinDERP2, HSPC282, MICS1, My021, PTD010, TMBIM5, Q9H3K2, gene:2706929literature, APEX_IMS, targetP signal+, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MSMIMMitochondrial dynamics and surveillance > Apoptosisall 14
GUF1GUF1 ­homolog, ­GTPaseEF-4, EF4, EIEE40, Q8N442, gene:6055829APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain+, coexpression, MS/MS+MatrixMitochondrial central dogma > Translationcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
TIMM13translocase ­of ­inner ­mitochondrial ­membrane ­13TIM13, TIM13B, TIMM13A, TIMM13B, ppv1, Q9Y5L4, gene:2651729literature, APEX_IMS, yeast mito homolog++, mito protein domain+, induction, coexpression, MS/MS++MIMProtein import, sorting and homeostasis > Protein homeostasis > Chaperones;
Protein import, sorting and homeostasis > Protein import and sorting		all 14
LYRM4LYR ­motif ­containing ­4C6orf149, CGI-203, COXPD19, ISD11, Q9HD34, gene:5712829literature, APEX_matrix, targetP signal+, yeast mito homolog, mito protein domain+, induction, coexpression, MS/MS++MatrixMetabolism > Metals and cofactors > Fe-S cluster biosynthesisall 14
MRPL16mitochondrial ­ribosomal ­protein ­L16L16mt, MRP-L16, PNAS-111, Q9NX20, gene:5494828literature, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression++, MS/MS+MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, placenta, testis
MRPL40mitochondrial ­ribosomal ­protein ­L40L40mt, MRP-L22, MRP-L40, MRPL22, NLVCF, URIM, Q9NQ50, gene:6497628literature, APEX_matrix, targetP signal+, yeast mito homolog++, mito protein domain+, induction, coexpression, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
IDH3Gisocitrate ­dehydrogenase ­(NAD(+)) ­3 ­non-catalytic ­subunit ­gammaH-IDHG, P51553, gene:342128literature, APEX_matrix, yeast mito homolog+, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++MatrixMetabolism > Carbohydrate metabolism > TCA cycleall 14
SDHCsuccinate ­dehydrogenase ­complex ­subunit ­CCYB560, CYBL, PGL3, QPS1, SDH3, Q99643, gene:639128literature, targetP signal+, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS+MIMOXPHOS > Complex II > CII subunits;
Metabolism > Carbohydrate metabolism > TCA cycle;
Metabolism > Metals and cofactors > Heme-containing proteins;
OXPHOS > OXPHOS subunits		all 14
NDUFB5NADH:ubiquinone ­oxidoreductase ­subunit ­B5CISGDH, SGDH, O43674, gene:471128literature, APEX_IMS, APEX_matrix, targetP signal+, mito protein domain+, induction, coexpression++, MS/MS+MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		all 14
SDHAF2succinate ­dehydrogenase ­complex ­assembly ­factor ­2C11orf79, PGL2, SDH5, Q9NX18, gene:5494928GFP, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain+, induction, coexpression, MS/MSMatrixOXPHOS > Complex II > CII assembly factors;
OXPHOS > OXPHOS assembly factors		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
COQ10Acoenzyme ­Q10AQ96MF6, gene:9305828targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain+, induction, MS/MS++MIMMetabolism > Metals and cofactors > Coenzyme Q metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, largeintestine, stomach
GATD3Aglutamine ­amidotransferase ­like ­class ­1 ­domain ­containing ­3AC21orf33, ES1, GATD3, GT335, HES1, KNPH, KNPI, P30042, gene:820928APEX_matrix, targetP signal+, mito protein domain, induction, coexpression++, MS/MS++Matrixnoneall 14
TXN2thioredoxin ­2COXPD29, MT-TRX, MTRX, TRX2, TXN, Q99757, gene:2582828literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS+MatrixMetabolism > Vitamin metabolism > Vitamin C metabolism;
Metabolism > Detoxification > ROS and glutathione metabolism		all 14
MRPS18Amitochondrial ­ribosomal ­protein ­S18AHumanS18b, MRP-S18-3, MRP-S18-a, MRPS18-3, S18bmt, S18mt-a, Q9NVS2, gene:5516828literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, brainstem, spinalcord, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
COX6Ccytochrome ­c ­oxidase ­subunit ­6CP09669, gene:134528literature, APEX_IMS, APEX_matrix, targetP signal, mito protein domain+, induction, coexpression++, MS/MS++MIMOXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits		all 14
NDUFB9NADH:ubiquinone ­oxidoreductase ­subunit ­B9B22, CI-B22, LYRM3, MC1DN24, UQOR22, Q9Y6M9, gene:471528literature, APEX_IMS, APEX_matrix, targetP signal, mito protein domain+, induction, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		all 14
MTCH2mitochondrial ­carrier ­2HSPC032, MIMP, SLC25A50, Q9Y6C9, gene:2378828literature, APEX_IMS, yeast mito homolog+, mito protein domain+, induction, coexpression+, MS/MS++MOMSmall molecule transport > SLC25A family;
Mitochondrial dynamics and surveillance > Fusion		all 14
NDUFA6NADH:ubiquinone ­oxidoreductase ­subunit ­A6B14, CI-B14, LYRM6, MC1DN33, NADHB14, P56556, gene:470028literature, APEX_matrix, mito protein domain+, induction, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		all 14
SLC25A20solute ­carrier ­family ­25 ­member ­20CAC, CACT, O43772, gene:78828literature, yeast mito homolog+, mito protein domain+, induction, coexpression, MS/MS++MIMMetabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Metals and cofactors > Carnitine synthesis and transport;
Metabolism > Metals and cofactors > Carnitine shuttle;
Small molecule transport > SLC25A family		all 14
MRPL1mitochondrial ­ribosomal ­protein ­L1BM022, L1MT, MRP-L1, Q9BYD6, gene:6500828literature, APEX_matrix, targetP signal, yeast mito homolog++, mito protein domain, induction, coexpression, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, spinalcord, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
TIMM44translocase ­of ­inner ­mitochondrial ­membrane ­44TIM44, O43615, gene:1046928literature, APEX_matrix, targetP signal, yeast mito homolog, mito protein domain, induction, coexpression++, MS/MS++MIMProtein import, sorting and homeostasis > Protein import and sorting > Import motorall 14
COX17cytochrome ­c ­oxidase ­copper ­chaperone ­COX17Q14061, gene:1006328literature, APEX_IMS, yeast mito homolog, mito protein domain+, induction, coexpression++, MS/MS++IMSOXPHOS > Complex IV > CIV assembly factors;
Metabolism > Metals and cofactors > Copper metabolism;
OXPHOS > OXPHOS assembly factors		heart, adipose, smallintestine, placenta, testis
MICOS10mitochondrial ­contact ­site ­and ­cristae ­organizing ­system ­subunit ­10C1orf151, MINOS1, MIO10, Mic10, Q5TGZ0, gene:44057428literature, APEX_IMS, yeast mito homolog, mito protein domain+, induction, coexpression++, MS/MS++MIMMitochondrial dynamics and surveillance > Cristae formation > MICOS complexall 14
SOD2superoxide ­dismutase ­2GClnc1, IPO-B, IPOB, MNSOD, MVCD6, Mn-SOD, P04179, gene:664828literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, MS/MS++MatrixMetabolism > Detoxification > ROS and glutathione metabolismall 14
COX6B1cytochrome ­c ­oxidase ­subunit ­6B1COX6B, COXG, COXVIb1, P14854, gene:134028literature, APEX_IMS, yeast mito homolog+, mito protein domain+, coexpression++, MS/MS++MIMOXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits		all 14
VDAC1voltage ­dependent ­anion ­channel ­1PORIN, VDAC-1, P21796, gene:741628literature, APEX_IMS, yeast mito homolog+, mito protein domain+, coexpression++, MS/MS++MOMSignaling > Calcium homeostasis > Mitochondrial permeability transition pore;
Mitochondrial dynamics and surveillance > Organelle contact sites;
Small molecule transport		all 14
CLPPcaseinolytic ­mitochondrial ­matrix ­peptidase ­proteolytic ­subunitDFNB81, PRLTS3, Q16740, gene:819228literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, induction, coexpression+, MS/MS++MatrixProtein import, sorting and homeostasis > Protein homeostasis > Proteasesall 14
HADHhydroxyacyl-CoA ­dehydrogenaseHAD, HADH1, HADHSC, HCDH, HHF4, MSCHAD, SCHAD, Q16836, gene:303328literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++MatrixMetabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Amino acid metabolism > Lysine metabolism		all 14
ACADLacyl-CoA ­dehydrogenase ­long ­chainACAD4, LCAD, P28330, gene:3328literature, targetP signal+, mito protein domain+, induction, coexpression+, MS/MS++MIMMetabolism > Lipid metabolism > Fatty acid oxidationall 14
ACAT1acetyl-CoA ­acetyltransferase ­1ACAT, MAT, T2, THIL, P24752, gene:3828literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, induction, coexpression+, MS/MS++MatrixMetabolism > Carbohydrate metabolism > Ketone metabolism;
Metabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Amino acid metabolism > BCAA metabolism;
Metabolism > Amino acid metabolism > Lysine metabolism		all 14
MALSU1mitochondrial ­assembly ­of ­ribosomal ­large ­subunit ­1C7orf30, mtRsfA, Q96EH3, gene:11541628literature, APEX_matrix, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression+, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosome assemblycerebrum, cerebellum, liver, smallintestine, largeintestine, placenta
CLPXcaseinolytic ­mitochondrial ­matrix ­peptidase ­chaperone ­subunit ­XEPP2, O76031, gene:1084527literature, APEX_matrix, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS++MatrixProtein import, sorting and homeostasis > Protein homeostasis > Proteasesall 14
NDUFS4NADH:ubiquinone ­oxidoreductase ­subunit ­S4AQDQ, CI-18, CI-18 kDa, CI-AQDQ, MC1DN1, O43181, gene:472427literature, APEX_matrix, targetP signal+, mito protein domain+, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		all 14
MRPL4mitochondrial ­ribosomal ­protein ­L4CGI-28, L4mt, Q9BYD3, gene:5107327literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, placenta, testis
C1QBPcomplement ­C1q ­binding ­proteinCOXPD33, GC1QBP, HABP1, SF2AP32, SF2p32, gC1Q-R, gC1qR, p32, Q07021, gene:70827literature, APEX_matrix, targetP signal+, yeast mito homolog++, mito protein domain, induction, coexpression, MS/MS++MatrixSignaling > Immune responseall 14
PITRM1pitrilysin ­metallopeptidase ­1MP1, PreP, Q5JRX3, gene:1053127literature, APEX_matrix, targetP signal, yeast mito homolog++, mito protein domain+, coexpression, MS/MS++MatrixProtein import, sorting and homeostasis > Protein homeostasis > Proteasesall 14
UQCC1ubiquinol-cytochrome ­c ­reductase ­complex ­assembly ­factor ­1BFZB, C20orf44, CBP3, UQCC, Q9NVA1, gene:5524527literature, APEX_matrix, targetP signal, yeast mito homolog, mito protein domain+, coexpression++, MS/MS++MIMOXPHOS > Complex III > CIII assembly factors;
OXPHOS > OXPHOS assembly factors		cerebrum, cerebellum, brainstem, spinalcord, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
MRPL55mitochondrial ­ribosomal ­protein ­L55AAVG5835, L55nt, MRP-L55, PRO19675, Q7Z7F7, gene:12830827literature, APEX_matrix, targetP signal, mito protein domain+, induction, coexpression++, MS/MS+MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
MRM1mitochondrial ­rRNA ­methyltransferase ­1Q6IN84, gene:7992227APEX_matrix, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain+, induction, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications		spinalcord, placenta, testis
MECRmitochondrial ­trans-2-enoyl-CoA ­reductaseCGI-63, DYTOABG, ETR1, FASN2B, NRBF1, Q9BV79, gene:5110227literature, APEX_matrix, targetP signal+, yeast mito homolog++, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Lipid metabolism > Type II fatty acid synthesisall 14
MRPL44mitochondrial ­ribosomal ­protein ­L44COXPD16, L44MT, MRP-L44, Q9H9J2, gene:6508027literature, APEX_matrix, targetP signal, yeast mito homolog++, induction, coexpression+, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, spinalcord, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
HSDL2hydroxysteroid ­dehydrogenase ­like ­2C9orf99, SDR13C1, Q6YN16, gene:8426327APEX_matrix, GFP, Rickettsial homolog, mito protein domain+, induction, coexpression+, MS/MS++Matrixnoneall 14
MRPS14mitochondrial ­ribosomal ­protein ­S14COXPD38, DJ262D12.2, HSMRPS14, MRP-S14, S14mt, O60783, gene:6393127literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
ABCB8ATP ­binding ­cassette ­subfamily ­B ­member ­8EST328128, M-ABC1, MABC1, MITOSUR, Q9NUT2, gene:1119427literature, targetP signal+, Rickettsial homolog, mito protein domain, coexpression++, MS/MS++MIMSmall molecule transport > ABC transportersall 14
ATPAF2ATP ­synthase ­mitochondrial ­F1 ­complex ­assembly ­factor ­2ATP12, ATP12p, LP3663, MC5DN1, Q8N5M1, gene:9164727literature, targetP signal, yeast mito homolog++, mito protein domain+, induction, coexpression, MS/MSMatrixOXPHOS > Complex V > CV assembly factors;
OXPHOS > OXPHOS assembly factors		cerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
NDUFA9NADH:ubiquinone ­oxidoreductase ­subunit ­A9CC6, CI-39k, CI39k, COQ11, MC1DN26, NDUFS2L, SDR22E1, Q16795, gene:470427literature, APEX_matrix, targetP signal+, mito protein domain, induction, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		all 14
COA6cytochrome ­c ­oxidase ­assembly ­factor ­6C1orf31, CEMCOX4, Q5JTJ3, gene:38875327APEX_IMS, yeast mito homolog++, mito protein domain+, induction, coexpression+, MS/MSIMSOXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors		cerebrum, cerebellum, spinalcord, liver, adipose, smallintestine, largeintestine, placenta, testis
POLRMTRNA ­polymerase ­mitochondrialAPOLMT, MTRNAP, MTRPOL, h-mtRPOL, O00411, gene:544227literature, APEX_matrix, targetP signal, yeast mito homolog++, mito protein domain+, coexpression++, MS/MSMatrixMitochondrial central dogma > mtDNA maintenance > mtDNA replication;
Mitochondrial central dogma > mtDNA maintenance > mtDNA nucleoid;
Mitochondrial central dogma > mtRNA metabolism > Transcription		cerebrum, cerebellum, brainstem, spinalcord, kidney, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
ISCUiron-sulfur ­cluster ­assembly ­enzyme2310020H20Rik, HML, ISU2, NIFU, NIFUN, hnifU, Q9H1K1, gene:2347927literature, APEX_matrix, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain, MS/MS++MatrixMetabolism > Metals and cofactors > Fe-S cluster biosynthesis;
Metabolism > Metals and cofactors > Fe-S-containing proteins		all 14
RTN4IP1reticulon ­4 ­interacting ­protein ­1NIMP, OPA10, Q8WWV3, gene:8481627literature, APEX_matrix, targetP signal, yeast mito homolog, mito protein domain, induction, coexpression++, MS/MS++Matrixnoneall 14
OGDHLoxoglutarate ­dehydrogenase ­LQ9ULD0, gene:5575327APEX_matrix, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain+, MS/MS++MatrixMetabolism > Carbohydrate metabolism > TCA cycleall 14
ATP5F1CATP ­synthase ­F1 ­subunit ­gammaATP5C, ATP5C1, ATP5CL1, P36542, gene:50927literature, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, MS/MS++MIMOXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits		all 14
BCKDKbranched ­chain ­keto ­acid ­dehydrogenase ­kinaseBCKDKD, BDK, O14874, gene:1029527literature, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain+, MS/MS++MatrixMetabolism > Amino acid metabolism > BCAA metabolism;
Metabolism > Amino acid metabolism > BCAA dehydrogenase complex		cerebrum, cerebellum, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
GFM2GTP ­dependent ­ribosome ­recycling ­factor ­mitochondrial ­2EF-G2mt, EFG2, MRRF2, MST027, MSTP027, RRF, RRF2, RRF2mt, hEFG2, mEF-G 2, Q969S9, gene:8434027literature, APEX_matrix, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression++, MS/MS++MatrixMitochondrial central dogma > Translation > Translation factorsall 14
NDUFAF4NADH:ubiquinone ­oxidoreductase ­complex ­assembly ­factor ­4C6orf66, HRPAP20, HSPC125, MC1DN15, My013, bA22L21.1, Q9P032, gene:2907827APEX_matrix, GFP, mito protein domain+, induction, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors		all 14
SLC25A35solute ­carrier ­family ­25 ­member ­35Q3KQZ1, gene:39951227APEX_matrix, yeast mito homolog+, mito protein domain+, induction, coexpression, MS/MS++MIMSmall molecule transport > SLC25A familycerebrum, cerebellum, kidney, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
SLC25A11solute ­carrier ­family ­25 ­member ­11OGC, PGL6, SLC20A4, Q02978, gene:840227literature, yeast mito homolog+, mito protein domain+, induction, coexpression, MS/MS++MIMMetabolism > Carbohydrate metabolism > Gluconeogenesis;
Metabolism > Carbohydrate metabolism > Malate-aspartate shuttle;
Metabolism > Carbohydrate metabolism > TCA-associated;
Small molecule transport > SLC25A family		all 14
TIMM8Btranslocase ­of ­inner ­mitochondrial ­membrane ­8 ­homolog ­BDDP2, TIM8B, Q9Y5J9, gene:2652127literature, yeast mito homolog+, mito protein domain+, induction, coexpression, MS/MS++MIMProtein import, sorting and homeostasis > Protein homeostasis > Chaperones;
Protein import, sorting and homeostasis > Protein import and sorting		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
MCATmalonyl-CoA-acyl ­carrier ­protein ­transacylaseFASN2C, MCT, MCT1, MT, NET62, fabD, Q8IVS2, gene:2734926literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Lipid metabolism > Type II fatty acid synthesisspinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
IBA57iron-sulfur ­cluster ­assembly ­factor ­IBA57C1orf69, MMDS3, SPG74, Q5T440, gene:20020526APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Metals and cofactors > Fe-S cluster biosynthesiscerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
PHB2prohibitin ­2BAP, BCAP37, Bap37, PNAS-141, REA, hBAP, p22, Q99623, gene:1133126literature, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS++MIMProtein import, sorting and homeostasis > Protein homeostasis > Chaperonesall 14
DAP3death ­associated ­protein ­3DAP-3, MRP-S29, MRPS29, S29mt, bMRP-10, P51398, gene:781826literature, APEX_matrix, targetP signal, yeast mito homolog++, mito protein domain+, coexpression, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
CMC2C-X9-C ­motif ­containing ­22310061C15Rik, C16orf61, DC13, Q9NRP2, gene:5694226APEX_IMS, yeast mito homolog++, mito protein domain+, induction, coexpression, MS/MS+IMSOXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors		cerebrum, cerebellum, spinalcord, liver, heart, adipose, largeintestine, stomach, placenta, testis
SDHAF1succinate ­dehydrogenase ­complex ­assembly ­factor ­1LYRM8, A6NFY7, gene:64409626literature, targetP signal+, yeast mito homolog, mito protein domain+, induction, coexpression+, MS/MSMatrixOXPHOS > Complex II > CII assembly factors;
OXPHOS > OXPHOS assembly factors		cerebrum, cerebellum, brainstem, spinalcord, kidney, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
MRPS17mitochondrial ­ribosomal ­protein ­S17HSPC011, MRP-S17, RPMS17, S17mt, Q9Y2R5, gene:5137326literature, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS+MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
ADHFE1alcohol ­dehydrogenase ­iron ­containing ­1ADH8, HMFT2263, HOT, Q8IWW8, gene:13787226targetP signal+, yeast mito homolog++, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Amino acid metabolism > GABA metabolismkidney, liver, heart, adipose, stomach, placenta
NFS1NFS1 ­cysteine ­desulfuraseHUSSY-08, IscS, NIFS, Q9Y697, gene:905426literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain+, coexpression+, MS/MS++MatrixMetabolism > Metals and cofactors > Fe-S cluster biosynthesis;
Metabolism > Metals and cofactors > Fe-S-containing proteins		all 14
NDUFAF5NADH:ubiquinone ­oxidoreductase ­complex ­assembly ­factor ­5C20orf7, MC1DN16, bA526K24.2, dJ842G6.1, Q5TEU4, gene:7913326literature, APEX_matrix, GFP, targetP signal+, Rickettsial homolog, mito protein domain+, induction, coexpression, MS/MSMIMOXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors		cerebellum, brainstem, skeletalmuscle, largeintestine, stomach, placenta
MRPL17mitochondrial ­ribosomal ­protein ­L17L17mt, LIP2, MRP-L17, MRP-L26, RPL17L, RPML26, Q9NRX2, gene:6387526literature, APEX_matrix, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
TACO1translational ­activator ­of ­cytochrome ­c ­oxidase ­ICCDC44, Q9BSH4, gene:5120426literature, APEX_matrix, targetP signal+, yeast mito homolog, Rickettsial homolog, mito protein domain+, MS/MS++MatrixMitochondrial central dogma > Translation > Translation factors;
OXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors		all 14
MFN1mitofusin ­1hfzo1, hfzo2, Q8IWA4, gene:5566926literature, yeast mito homolog++, mito protein domain+, induction, coexpression, MS/MS+MOMMitochondrial dynamics and surveillance > Fusion;
Mitochondrial dynamics and surveillance > Organelle contact sites		cerebellum, brainstem, spinalcord, kidney, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
PPTC7protein ­phosphatase ­targeting ­COQ7TA-PP2C, TAPP2C, Q8NI37, gene:16076026APEX_matrix, yeast mito homolog, mito protein domain+, induction, coexpression, MS/MS++MatrixSignalingcerebrum, cerebellum, brainstem, spinalcord, heart, skeletalmuscle, largeintestine, placenta, testis
MRPL11mitochondrial ­ribosomal ­protein ­L11CGI-113, L11MT, MRP-L11, Q9Y3B7, gene:6500326literature, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression+, MS/MS+MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
COX4I1cytochrome ­c ­oxidase ­subunit ­4I1COX IV-1, COX4, COX4-1, COXIV, COXIV-1, P13073, gene:132726literature, APEX_IMS, APEX_matrix, targetP signal, mito protein domain+, coexpression++, MS/MS++MIMOXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits		all 14
NDUFS6NADH:ubiquinone ­oxidoreductase ­subunit ­S6CI-13kA, CI-13kD-A, CI13KDA, MC1DN9, O75380, gene:472626literature, APEX_matrix, targetP signal, mito protein domain+, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		all 14
MRPL13mitochondrial ­ribosomal ­protein ­L13L13, L13A, L13mt, RPL13, RPML13, Q9BYD1, gene:2899826literature, APEX_matrix, targetP signal, yeast mito homolog, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
OXCT13-oxoacid ­CoA-transferase ­1OXCT, SCOT, P55809, gene:501926literature, APEX_matrix, targetP signal, mito protein domain, induction, coexpression++, MS/MS++MatrixMetabolism > Carbohydrate metabolism > Ketone metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
COX5Bcytochrome ­c ­oxidase ­subunit ­5BCOXVB, P10606, gene:132926literature, APEX_IMS, APEX_matrix, targetP signal+, yeast mito homolog++, mito protein domain+, MS/MS++MIMOXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits		all 14
PDK2pyruvate ­dehydrogenase ­kinase ­2PDHK2, PDKII, Q15119, gene:516426literature, APEX_matrix, yeast mito homolog+, mito protein domain+, induction, coexpression, MS/MS++MatrixMetabolism > Carbohydrate metabolism > Pyruvate metabolismall 14
ALAS15'-aminolevulinate ­synthase ­1ALAS, ALAS-H, ALAS3, ALASH, MIG4, P13196, gene:21126literature, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain+, induction, coexpression, MS/MSMatrixMetabolism > Metals and cofactors > Heme synthesis and processingliver, heart, adipose, largeintestine, placenta
MRPL33mitochondrial ­ribosomal ­protein ­L33C2orf1, L33mt, MRP-L33, RPL33L, O75394, gene:955326literature, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, stomach, placenta, testis
MTO1mitochondrial ­tRNA ­translation ­optimization ­1CGI-02, COXPD10, Q9Y2Z2, gene:2582126literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression++MatrixMitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications;
Metabolism > Vitamin metabolism > Folate and 1-C metabolism		cerebellum, spinalcord, stomach, placenta
LIASlipoic ­acid ­synthetaseHGCLAS, HUSSY-01, LAS, LIP1, LS, PDHLD, O43766, gene:1101926literature, APEX_matrix, targetP signal, yeast mito homolog, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MSMatrixMetabolism > Lipid metabolism > Lipoate insertion;
Metabolism > Metals and cofactors > Fe-S-containing proteins		brainstem, skeletalmuscle, adipose, largeintestine, placenta
NDUFA5NADH:ubiquinone ­oxidoreductase ­subunit ­A5B13, CI-13KD-B, CI-13kB, NUFM, UQOR13, Q16718, gene:469826literature, APEX_matrix, mito protein domain+, induction, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		all 14
NDUFB6NADH:ubiquinone ­oxidoreductase ­subunit ­B6B17, CI, O95139, gene:471226literature, APEX_IMS, APEX_matrix, mito protein domain+, induction, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		all 14
MTX2metaxin ­2metaxin-2, O75431, gene:1065126literature, mito protein domain+, induction, coexpression++, MS/MS++MOMProtein import, sorting and homeostasis > Protein import and sorting > SAM;
Mitochondrial dynamics and surveillance > Intramitochondrial membrane interactions		all 14
SUCLG2succinate-CoA ­ligase ­GDP-forming ­subunit ­betaG-SCS, GBETA, GTPSCS, Q96I99, gene:880126literature, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Carbohydrate metabolism > TCA cycle;
Metabolism > Carbohydrate metabolism > Itaconate metabolism;
Metabolism > Nucleotide metabolism > Nucleotide synthesis and processing		all 14
FDX1ferredoxin ­1ADX, FDX, LOH11CR1D, P10109, gene:223026literature, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS+MatrixMetabolism > Lipid metabolism > Cholesterol, bile acid, steroid synthesis;
Metabolism > Metals and cofactors > Heme synthesis and processing;
Metabolism > Metals and cofactors > Fe-S cluster biosynthesis;
Metabolism > Metals and cofactors > Fe-S-containing proteins;
Metabolism > Vitamin metabolism > Vitamin D metabolism		all 14
SLC25A1solute ­carrier ­family ­25 ­member ­1CMS23, CTP, D2L2AD, SEA, SLC20A3, P53007, gene:657626literature, yeast mito homolog++, mito protein domain+, coexpression, MS/MS++MIMMetabolism > Carbohydrate metabolism > Gluconeogenesis;
Metabolism > Carbohydrate metabolism > TCA-associated;
Metabolism > Lipid metabolism;
Small molecule transport > SLC25A family		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
MRPS28mitochondrial ­ribosomal ­protein ­S28COXPD47, HSPC007, MRP-S28, MRP-S35, MRPS35, Q9Y2Q9, gene:2895726literature, targetP signal, mito protein domain+, coexpression++, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
TIMM50translocase ­of ­inner ­mitochondrial ­membrane ­50MGCA9, TIM50, TIM50L, Q3ZCQ8, gene:9260926literature, APEX_IMS, yeast mito homolog++, mito protein domain, coexpression++, MS/MS++MIMProtein import, sorting and homeostasis > Protein import and sorting > TIM23 presequence pathwayall 14
VDAC2voltage ­dependent ­anion ­channel ­2POR, P45880, gene:741726literature, APEX_IMS, yeast mito homolog+, mito protein domain+, induction, coexpression, MS/MS++MOMSmall molecule transportall 14
SLC25A42solute ­carrier ­family ­25 ­member ­42MECREN, Q86VD7, gene:28443926literature, yeast mito homolog+, mito protein domain+, induction, coexpression, MS/MS++MIMMetabolism > Metals and cofactors > Coenzyme A metabolism;
Small molecule transport > SLC25A family		all 14
TOMM7translocase ­of ­outer ­mitochondrial ­membrane ­7TOM7, Q9P0U1, gene:5454326literature, yeast mito homolog++, mito protein domain+, coexpression, MS/MS++MOMProtein import, sorting and homeostasis > Protein import and sorting > TOM;
Mitochondrial dynamics and surveillance > Mitophagy;
Mitochondrial dynamics and surveillance > Autophagy		cerebrum, cerebellum, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, stomach, placenta, testis
ECH1enoyl-CoA ­hydratase ­1HPXEL, Q13011, gene:189126APEX_matrix, targetP signal+, yeast mito homolog+, mito protein domain, coexpression+, MS/MS++MatrixMetabolism > Lipid metabolismall 14
GATBglutamyl-tRNA ­amidotransferase ­subunit ­BCOXPD41, HSPC199, PET112, PET112L, O75879, gene:518825literature, APEX_matrix, targetP signal, yeast mito homolog, Rickettsial homolog, mito protein domain+, coexpression+, MS/MSMatrixMitochondrial central dogma > Translation > mt-tRNA synthetasescerebrum, skeletalmuscle, adipose, stomach, testis
BCS1LBCS1 ­homolog, ­ubiquinol-cytochrome ­c ­reductase ­complex ­chaperoneBCS, BCS1, BJS, FLNMS, GRACILE, Hs.6719, MC3DN1, PTD, h-BCS, h-BCS1, Q9Y276, gene:61725literature, APEX_matrix, yeast mito homolog++, Rickettsial homolog, mito protein domain+, coexpression+, MS/MS+MIMOXPHOS > Complex III > CIII assembly factors;
OXPHOS > OXPHOS assembly factors		cerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
ERAL1Era ­like ­12S ­mitochondrial ­rRNA ­chaperone ­1CEGA, ERA, ERA-W, ERAL1A, ERAL1B, H-ERA, HERA-A, HERA-B, PRLTS6, O75616, gene:2628425APEX_matrix, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain, induction, coexpression++MatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly		none
CMC1C-X9-C ­motif ­containing ­1C3orf68, Q7Z7K0, gene:15210025APEX_IMS, GFP, yeast mito homolog++, mito protein domain+, induction, coexpression, MS/MS+IMSOXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
MRPL28mitochondrial ­ribosomal ­protein ­L28MAAT1, p15, Q13084, gene:1057325literature, APEX_matrix, yeast mito homolog++, Rickettsial homolog, coexpression, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, brainstem, spinalcord, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
TSFMTs ­translation ­elongation ­factor, ­mitochondrialEFTS, EFTSMT, P43897, gene:1010225literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS++MatrixMitochondrial central dogma > Translation > Translation factorscerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
FXNfrataxinCyaY, FA, FARR, FRDA, X25, Q16595, gene:239525literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MS+MatrixMetabolism > Metals and cofactors > Fe-S cluster biosynthesisall 14
NFU1NFU1 ­iron-sulfur ­cluster ­scaffoldCGI-33, HIRIP, HIRIP5, MMDS1, NIFUC, Nfu, NifU, Q9UMS0, gene:2724725APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MS+MatrixMetabolism > Metals and cofactors > Fe-S cluster biosynthesis;
Metabolism > Metals and cofactors > Fe-S-containing proteins		all 14
YARS2tyrosyl-tRNA ­synthetase ­2CGI-04, MLASA2, MT-TYRRS, TYRRS, Q9Y2Z4, gene:5106725literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MS+MatrixMitochondrial central dogma > Translation > mt-tRNA synthetasesbrainstem, spinalcord, kidney, adipose, smallintestine, largeintestine, stomach, placenta, testis
MRPL21mitochondrial ­ribosomal ­protein ­L21L21mt, MRP-L21, Q7Z2W9, gene:21992725literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MS+MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
ALDH4A1aldehyde ­dehydrogenase ­4 ­family ­member ­A1ALDH4, P5CD, P5CDh, P30038, gene:865925literature, APEX_matrix, targetP signal+, yeast mito homolog++, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Amino acid metabolism > Proline metabolism;
Metabolism > Amino acid metabolism > Glyoxylate metabolism		all 14
TOMM40Ltranslocase ­of ­outer ­mitochondrial ­membrane ­40 ­likeTOMM40B, Q969M1, gene:8413425targetP signal, yeast mito homolog+, mito protein domain+, induction, coexpression+, MS/MSMOMProtein import, sorting and homeostasis > Protein import and sorting > TOMcerebrum, cerebellum, brainstem, spinalcord, adipose, largeintestine, stomach, testis
NDUFAF1NADH:ubiquinone ­oxidoreductase ­complex ­assembly ­factor ­1CGI-65, CGI65, CIA30, MC1DN11, Q9Y375, gene:5110325literature, APEX_matrix, targetP signal, mito protein domain, induction, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors		cerebellum, brainstem, spinalcord, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
NDUFB10NADH:ubiquinone ­oxidoreductase ­subunit ­B10PDSW, O96000, gene:471625literature, APEX_matrix, mito protein domain+, induction, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		all 14
ACADSBacyl-CoA ­dehydrogenase ­short/branched ­chain2-MEBCAD, ACAD7, SBCAD, P45954, gene:3625literature, APEX_matrix, targetP signal, mito protein domain+, induction, coexpression, MS/MS++MatrixMetabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Amino acid metabolism > BCAA metabolism		all 14
MRPS9mitochondrial ­ribosomal ­protein ­S9MRP-S9, RPMS9, S9mt, P82933, gene:6496525literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MSMatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > Translation > Mitochondrial ribosome		all 14
COQ8Acoenzyme ­Q8AADCK3, ARCA2, CABC1, COQ10D4, COQ8, SCAR9, Q8NI60, gene:5699725literature, APEX_matrix, yeast mito homolog+, Rickettsial homolog, mito protein domain, induction, coexpression+, MS/MS++MIMMetabolism > Metals and cofactors > Coenzyme Q metabolismall 14
ACADVLacyl-CoA ­dehydrogenase ­very ­long ­chainACAD6, LCACD, VLCAD, P49748, gene:3725literature, APEX_matrix, targetP signal+, mito protein domain+, coexpression+, MS/MS++MIMMetabolism > Lipid metabolism > Fatty acid oxidationall 14
COQ4coenzyme ­Q4CGI-92, COQ10D7, Q9Y3A0, gene:5111725literature, targetP signal+, yeast mito homolog++, mito protein domain+, coexpression, MS/MSMIMMetabolism > Metals and cofactors > Coenzyme Q metabolismcerebrum, cerebellum, brainstem, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta
MRPL15mitochondrial ­ribosomal ­protein ­L15HSPC145, L15mt, MRP-L15, MRP-L7, RPML7, Q9P015, gene:2908825literature, APEX_matrix, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
NDUFA2NADH:ubiquinone ­oxidoreductase ­subunit ­A2B8, CD14, CIB8, MC1DN13, O43678, gene:469525literature, APEX_matrix, targetP signal, mito protein domain, induction, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		all 14
MRPL24mitochondrial ­ribosomal ­protein ­L24L24mt, MRP-L18, MRP-L24, Q96A35, gene:7959025literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
HSPD1heat ­shock ­protein ­family ­D ­(Hsp60) ­member ­1CPN60, GROEL, HLD4, HSP-60, HSP60, HSP65, HuCHA60, SPG13, P10809, gene:332925literature, APEX_matrix, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain, MS/MS++MatrixProtein import, sorting and homeostasis > Protein homeostasis > Chaperonesall 14
NDUFS5NADH:ubiquinone ­oxidoreductase ­subunit ­S5CI-15k, CI15K, O43920, gene:472525literature, APEX_IMS, mito protein domain+, induction, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		all 14
AK3adenylate ­kinase ­3AK3L1, AK6, AKL3L, AKL3L1, FIX, Q9UIJ7, gene:5080825literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS++MatrixMetabolism > Nucleotide metabolism > Nucleotide synthesis and processingall 14
CYCScytochrome ­c, ­somaticCYC, HCS, THC4, P99999, gene:5420524literature, APEX_IMS, yeast mito homolog++, Rickettsial homolog, mito protein domain+, MS/MS++IMSMetabolism > Metals and cofactors > Heme-containing proteins;
Metabolism > Electron carriers > Cytochromes;
OXPHOS > OXPHOS subunits;
OXPHOS > Cytochrome C;
Mitochondrial dynamics and surveillance > Apoptosis		all 14
MIPEPmitochondrial ­intermediate ­peptidaseCOXPD31, HMIP, MIP, Q99797, gene:428524literature, APEX_matrix, targetP signal+, yeast mito homolog++, mito protein domain, induction, MS/MS++MatrixProtein import, sorting and homeostasis > Protein import and sorting > Preprotein cleavage;
Protein import, sorting and homeostasis > Protein homeostasis > Proteases		cerebrum, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
LYRM7LYR ­motif ­containing ­7C5orf31, MC3DN8, MZM1L, Q5U5X0, gene:9062424literature, APEX_matrix, targetP signal, yeast mito homolog++, mito protein domain+, MS/MS++MatrixOXPHOS > Complex III > CIII assembly factors;
OXPHOS > OXPHOS assembly factors		cerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
CRATcarnitine ­O-acetyltransferaseCAT, CAT1, NBIA8, P43155, gene:138424literature, APEX_matrix, targetP signal, yeast mito homolog+, mito protein domain, induction, coexpression, MS/MS++MIMMetabolism > Lipid metabolism > Fatty acid oxidationall 14
PCCBpropionyl-CoA ­carboxylase ­subunit ­betaP05166, gene:509624literature, targetP signal+, Rickettsial homolog, mito protein domain, coexpression+, MS/MS++MatrixMetabolism > Carbohydrate metabolism > Propanoate metabolism;
Metabolism > Lipid metabolism > Fatty acid oxidation		all 14
MRPS7mitochondrial ­ribosomal ­protein ­S7COXPD34, MRP-S, MRP-S7, RP-S7, RPMS7, S7mt, bMRP27a, Q9Y2R9, gene:5108124literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression+, MS/MSMatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > Translation > Mitochondrial ribosome		all 14
MRPL3mitochondrial ­ribosomal ­protein ­L3COXPD9, MRL3, RPML3, P09001, gene:1122224literature, APEX_matrix, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpressionMatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, placenta, testis
PRODHproline ­dehydrogenase ­1HSPOX2, PIG6, POX, PRODH1, PRODH2, TP53I6, O43272, gene:562524literature, targetP signal, yeast mito homolog+, mito protein domain+, coexpression, MS/MS++MIMMetabolism > Amino acid metabolism > Proline metabolism;
Metabolism > Electron carriers > Q-linked reactions, other		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
PCCApropionyl-CoA ­carboxylase ­subunit ­alphaP05165, gene:509524literature, targetP signal, Rickettsial homolog, mito protein domain, induction, coexpression+, MS/MS++MatrixMetabolism > Carbohydrate metabolism > Propanoate metabolism;
Metabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Vitamin metabolism > Biotin utilizing proteins		all 14
MCCC1methylcrotonoyl-CoA ­carboxylase ­1MCC-B, MCCA, Q96RQ3, gene:5692224literature, targetP signal, Rickettsial homolog, mito protein domain, induction, coexpression+, MS/MS++MatrixMetabolism > Amino acid metabolism > BCAA metabolism;
Metabolism > Vitamin metabolism > Biotin utilizing proteins		all 14
MRPS12mitochondrial ­ribosomal ­protein ­S12MPR-S12, MT-RPS12, RPMS12, RPS12, RPSM12, O15235, gene:618324literature, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression+, MS/MS+MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, brainstem, spinalcord, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
CLPBcaseinolytic ­mitochondrial ­matrix ­peptidase ­chaperone ­subunit ­BANKCLB, HSP78, MEGCANN, MGCA7, SKD3, Q9H078, gene:8157024APEX_IMS, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain, MS/MS++IMSProtein import, sorting and homeostasis > Protein homeostasis > Proteasescerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
PDK1pyruvate ­dehydrogenase ­kinase ­1Q15118, gene:516324literature, APEX_matrix, targetP signal+, yeast mito homolog+, mito protein domain+, MS/MS++MatrixMetabolism > Carbohydrate metabolism > Pyruvate metabolismall 14
MRPL49mitochondrial ­ribosomal ­protein ­L49C11orf4, L49mt, MRP-L49, NOF, NOF1, Q13405, gene:74024literature, APEX_matrix, targetP signal, yeast mito homolog++, mito protein domain, coexpression, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
COX7A2cytochrome ­c ­oxidase ­subunit ­7A2COX7AL, COX7AL1, COXVIIAL, COXVIIa-L, VIIAL, P14406, gene:134724literature, mito protein domain+, induction, coexpression++, MS/MS++MIMOXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits		all 14
TMEM126Atransmembrane ­protein ­126AOPA7, Q9H061, gene:8423324literature, mito protein domain+, induction, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors		all 14
ECHDC2enoyl-CoA ­hydratase ­domain ­containing ­2Q86YB7, gene:5526824targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpression, MS/MS++Matrixnoneall 14
HCCSholocytochrome ­c ­synthaseCCHL, LSDMCA1, MCOPS7, MLS, P53701, gene:305224literature, APEX_IMS, yeast mito homolog++, mito protein domain+, induction, coexpression, MS/MSIMSMetabolism > Metals and cofactors > Heme synthesis and processing;
OXPHOS > OXPHOS subunits;
OXPHOS > Cytochrome C		cerebrum, spinalcord, heart, adipose, smallintestine, largeintestine, placenta, testis
HIBADH3-hydroxyisobutyrate ­dehydrogenaseNS5ATP1, P31937, gene:1111224literature, APEX_matrix, targetP signal+, mito protein domain, induction, coexpression+, MS/MS++MatrixMetabolism > Amino acid metabolism > BCAA metabolismall 14
MRPL19mitochondrial ­ribosomal ­protein ­L19L19mt, MRP-L15, MRP-L19, MRPL15, RLX1, RPML15, P49406, gene:980124literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MS+MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, brainstem, spinalcord, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
MRPL36mitochondrial ­ribosomal ­protein ­L36BRIP1, L36mt, MRP-L36, PRPL36, RPMJ, Q9P0J6, gene:6497924literature, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression+MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomenone
SLC25A30solute ­carrier ­family ­25 ­member ­30KMCP1, Q5SVS4, gene:25351224literature, yeast mito homolog+, mito protein domain+, coexpression, MS/MS++MIMMetabolism > Sulfur metabolism;
Small molecule transport > SLC25A family		cerebrum, cerebellum, liver, placenta, testis
BDH13-hydroxybutyrate ­dehydrogenase ­1BDH, SDR9C1, Q02338, gene:62224literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS++MIMMetabolism > Carbohydrate metabolism > Ketone metabolismall 14
FARS2phenylalanyl-tRNA ­synthetase ­2, ­mitochondrialCOXPD14, FARS1, HSPC320, PheRS, SPG77, mtPheRS, O95363, gene:1066723literature, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MSMatrixMitochondrial central dogma > Translation > mt-tRNA synthetasesadipose, largeintestine, stomach, placenta, testis
ABCB7ATP ­binding ­cassette ­subfamily ­B ­member ­7ABC7, ASAT, Atm1p, EST140535, O75027, gene:2223literature, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpression+, MS/MS+MIMMetabolism > Metals and cofactors > Heme synthesis and processing;
Metabolism > Metals and cofactors > Fe-S cluster biosynthesis;
Metabolism > Metals and cofactors > Molybdenum cofactor synthesis and proteins;
Small molecule transport > ABC transporters		all 14
MTX1metaxin ­1MTX, MTXN, Q13505, gene:458023literature, yeast mito homolog++, mito protein domain+, coexpression, MS/MS++MOMProtein import, sorting and homeostasis > Protein import and sorting > SAM;
Mitochondrial dynamics and surveillance > Intramitochondrial membrane interactions		all 14
NDUFA7NADH:ubiquinone ­oxidoreductase ­subunit ­A7B14.5a, CI-B14.5a, O95182, gene:470123literature, APEX_matrix, mito protein domain+, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		all 14
TIMM17Atranslocase ­of ­inner ­mitochondrial ­membrane ­17ATIM17, TIM17A, Q99595, gene:1044023literature, APEX_IMS, yeast mito homolog+, mito protein domain+, induction, coexpression, MS/MS+MIMProtein import, sorting and homeostasis > Protein import and sorting > TIM23 presequence pathwayall 14
ALDH9A1aldehyde ­dehydrogenase ­9 ­family ­member ­A1ALDH4, ALDH7, ALDH9, E3, TMABA-DH, TMABADH, TMABALDH, P49189, gene:22323APEX_matrix, targetP signal+, yeast mito homolog+, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Amino acid metabolism > GABA metabolism;
Metabolism > Detoxification > ROS and glutathione metabolism		brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
MRPS18Cmitochondrial ­ribosomal ­protein ­S18CCGI-134, MRP-S18-1, MRP-S18-c, MRPS18-1, S18mt-c, mrps18-c, Q9Y3D5, gene:5102323literature, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
MARS2methionyl-tRNA ­synthetase ­2, ­mitochondrialCOXPD25, MetRS, mtMetRS, Q96GW9, gene:9293523literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MS+MatrixMitochondrial central dogma > Translation > mt-tRNA synthetasescerebrum, cerebellum, brainstem, spinalcord, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
ALDH6A1aldehyde ­dehydrogenase ­6 ­family ­member ­A1MMSADHA, MMSDH, Q02252, gene:432923literature, APEX_matrix, targetP signal, yeast mito homolog+, mito protein domain, coexpression, MS/MS++MIMMetabolism > Amino acid metabolism > BCAA metabolismall 14
FDXRferredoxin ­reductaseADR, ADXR, ANOA, P22570, gene:223223literature, APEX_matrix, targetP signal+, yeast mito homolog++, coexpression, MS/MS++MatrixMetabolism > Lipid metabolism > Cholesterol, bile acid, steroid synthesis;
Metabolism > Metals and cofactors > Heme synthesis and processing;
Metabolism > Metals and cofactors > Fe-S cluster biosynthesis;
Metabolism > Vitamin metabolism > Vitamin D metabolism		spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
GATCglutamyl-tRNA ­amidotransferase ­subunit ­C15E1.2, COXPD42, O43716, gene:28345923literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain+, induction, coexpression, MS/MSMatrixMitochondrial central dogma > Translation > mt-tRNA synthetasescerebrum, cerebellum, spinalcord, kidney, largeintestine, testis
TRAP1TNF ­receptor ­associated ­protein ­1HSP 75, HSP75, HSP90L, TRAP-1, Q12931, gene:1013123APEX_matrix, GFP, targetP signal+, Rickettsial homolog, mito protein domain, coexpression++, MS/MS+MatrixProtein import, sorting and homeostasis > Protein homeostasis > Chaperonesall 14
ACAD8acyl-CoA ­dehydrogenase ­family ­member ­8ACAD-8, ARC42, IBDH, Q9UKU7, gene:2703422literature, APEX_matrix, targetP signal+, mito protein domain+, coexpression, MS/MS++MatrixMetabolism > Amino acid metabolism > BCAA metabolismall 14
ALDH2aldehyde ­dehydrogenase ­2 ­family ­memberALDH-E2, ALDHI, ALDM, P05091, gene:21722literature, APEX_matrix, targetP signal+, yeast mito homolog+, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Detoxification > Xenobiotic metabolismall 14
PPIFpeptidylprolyl ­isomerase ­FCYP3, CyP-M, Cyp-D, CypD, P30405, gene:1010522literature, APEX_matrix, targetP signal+, mito protein domain, induction, coexpression, MS/MS++MatrixSignaling > Calcium homeostasis > Mitochondrial permeability transition poreall 14
TIMM22translocase ­of ­inner ­mitochondrial ­membrane ­22COXPD43, TEX4, TIM22, Q9Y584, gene:2992822literature, yeast mito homolog++, mito protein domain+, induction, coexpression, MS/MSMIMProtein import, sorting and homeostasis > Protein import and sorting > TIM22 carrier pathwayall 14
IVDisovaleryl-CoA ­dehydrogenaseACAD2, P26440, gene:371222literature, APEX_matrix, targetP signal, mito protein domain+, coexpression+, MS/MS++MatrixMetabolism > Amino acid metabolism > BCAA metabolismall 14
L2HGDHL-2-hydroxyglutarate ­dehydrogenaseC14orf160, L2HGA, Q9H9P8, gene:7994422literature, APEX_matrix, targetP signal+, mito protein domain, induction, coexpression, MS/MS++MIMMetabolism > Carbohydrate metabolism > TCA-associatedcerebrum, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, testis
ETHE1ETHE1 ­persulfide ­dioxygenaseHSCO, YF13H12, O95571, gene:2347422literature, APEX_matrix, targetP signal, yeast mito homolog+, mito protein domain, induction, MS/MS++MatrixMetabolism > Sulfur metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta
MRPL20mitochondrial ­ribosomal ­protein ­L20L20mt, MRP-L20, Q9BYC9, gene:5505222literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
SLC25A5solute ­carrier ­family ­25 ­member ­52F1, AAC2, ANT2, T2, T3, P05141, gene:29222literature, yeast mito homolog+, mito protein domain+, induction, MS/MS++MIMMetabolism > Nucleotide metabolism > Nucleotide import;
Signaling > Calcium homeostasis > Mitochondrial permeability transition pore;
Small molecule transport > SLC25A family		all 14
SLC25A12solute ­carrier ­family ­25 ­member ­12AGC1, ARALAR, EIEE39, O75746, gene:860422literature, yeast mito homolog+, mito protein domain+, induction, MS/MS++MIMMetabolism > Carbohydrate metabolism > Malate-aspartate shuttle;
Metabolism > Amino acid metabolism > Glutamate metabolism;
Signaling > Calcium homeostasis > EF hand proteins;
Small molecule transport > SLC25A family		all 14
MRPS21mitochondrial ­ribosomal ­protein ­S21MDS016, MRP-S21, RPMS21, P82921, gene:5446022literature, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
TOMM22translocase ­of ­outer ­mitochondrial ­membrane ­221C9-2, MST065, MSTP065, TOM22, Q9NS69, gene:5699322literature, yeast mito homolog++, mito protein domain+, coexpression, MS/MS++MOMProtein import, sorting and homeostasis > Protein import and sorting > TOMall 14
ACAA2acetyl-CoA ­acyltransferase ­2DSAEC, P42765, gene:1044922literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, induction, coexpression+, MS/MS++MatrixMetabolism > Carbohydrate metabolism > Ketone metabolism;
Metabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Amino acid metabolism > Lysine metabolism		all 14
MRPL30mitochondrial ­ribosomal ­protein ­L30L28MT, L30MT, MRP-L28, MRP-L30, MRPL28, MRPL28M, RPML28, Q8TCC3, gene:5126322literature, APEX_matrix, yeast mito homolog++, mito protein domain, coexpression++, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
DNAJA3DnaJ ­heat ­shock ­protein ­family ­(Hsp40) ­member ­A3HCA57, TID1, hTID-1, Q96EY1, gene:909322literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS+MatrixProtein import, sorting and homeostasis > Protein homeostasis > Chaperonesall 14
NDUFB2NADH:ubiquinone ­oxidoreductase ­subunit ­B2AGGG, CI-AGGG, O95178, gene:470822literature, targetP signal, induction, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		all 14
MRPS34mitochondrial ­ribosomal ­protein ­S34COXPD32, MRP-S12, MRP-S34, MRPS12, P82930, gene:6599322literature, APEX_matrix, targetP signal, induction, coexpression++, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
ETFBelectron ­transfer ­flavoprotein ­subunit ­betaFP585, MADD, P38117, gene:210922literature, APEX_matrix, yeast mito homolog, mito protein domain, induction, coexpression+, MS/MS++MatrixMetabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Amino acid metabolism > BCAA metabolism;
Metabolism > Amino acid metabolism > Lysine metabolism;
Metabolism > Amino acid metabolism > Glycine metabolism;
Metabolism > Vitamin metabolism > Choline and betaine metabolism;
Metabolism > Electron carriers > Q-linked reactions, other		all 14
AFG1LAFG1 ­like ­ATPaseAFG1, LACE1, c222389, Q8WV93, gene:24626922APEX_matrix, yeast mito homolog++, Rickettsial homolog, coexpression, MS/MS+MIMProtein import, sorting and homeostasis > Protein homeostasis > Proteasescerebrum, cerebellum, spinalcord, heart, adipose, smallintestine, largeintestine, stomach, placenta
ATP5IF1ATP ­synthase ­inhibitory ­factor ­subunit ­1ATPI, ATPIF1, ATPIP, IP, Q9UII2, gene:9397422literature, APEX_matrix, targetP signal, yeast mito homolog++, mito protein domain+, MS/MS++MatrixOXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits		all 14
ATP5PBATP ­synthase ­peripheral ­stalk-membrane ­subunit ­bATP5F1, PIG47, P24539, gene:51522literature, APEX_matrix, yeast mito homolog++, mito protein domain+, MS/MS++MIMOXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits		all 14
COX7Ccytochrome ­c ­oxidase ­subunit ­7CP15954, gene:135022literature, APEX_IMS, yeast mito homolog++, mito protein domain+, MS/MS++MIMOXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits		all 14
CHCHD3coiled-coil-helix-coiled-coil-helix ­domain ­containing ­3MICOS19, MINOS3, Mic19, PPP1R22, Q9NX63, gene:5492722literature, APEX_IMS, APEX_matrix, GFP, yeast mito homolog, induction, coexpression++, MS/MS++MIMMitochondrial dynamics and surveillance > Cristae formation > MICOS complex;
Mitochondrial dynamics and surveillance > Intramitochondrial membrane interactions		all 14
COQ10Bcoenzyme ­Q10BQ9H8M1, gene:8021922GFP, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain+, MS/MS+MIMMetabolism > Metals and cofactors > Coenzyme Q metabolismcerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta
ACSF3acyl-CoA ­synthetase ­family ­member ­3Q4G176, gene:19732222APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, coexpression+, MS/MS++MatrixMetabolism > Lipid metabolism > Type II fatty acid synthesisall 14
POLDIP2DNA ­polymerase ­delta ­interacting ­protein ­2PDIP38, POLD4, p38, Q9Y2S7, gene:2607322literature, APEX_matrix, targetP signal+, induction, coexpression++, MS/MS++MatrixMitochondrial central dogma > mtDNA maintenance > mtDNA nucleoidall 14
SLC25A10solute ­carrier ­family ­25 ­member ­10DIC, MTDPS19, Q9UBX3, gene:146822literature, yeast mito homolog+, mito protein domain+, coexpression, MS/MS++MIMMetabolism > Carbohydrate metabolism > Gluconeogenesis;
Metabolism > Carbohydrate metabolism > Malate-aspartate shuttle;
Small molecule transport > SLC25A family		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
SLC25A13solute ­carrier ­family ­25 ­member ­13ARALAR2, CITRIN, CTLN2, NICCD, Q9UJS0, gene:1016522literature, yeast mito homolog+, mito protein domain+, coexpression, MS/MS++MIMMetabolism > Carbohydrate metabolism > Malate-aspartate shuttle;
Metabolism > Amino acid metabolism > Urea cycle;
Signaling > Calcium homeostasis > EF hand proteins;
Small molecule transport > SLC25A family		all 14
PDK3pyruvate ­dehydrogenase ­kinase ­3CMTX6, GS1-358P8.4, Q15120, gene:516521literature, APEX_matrix, targetP signal, yeast mito homolog+, mito protein domain+, MS/MS++MatrixMetabolism > Carbohydrate metabolism > Pyruvate metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
ME3malic ­enzyme ­3NADP-ME, Q16798, gene:1087321literature, yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Carbohydrate metabolism > TCA-associatedcerebrum, cerebellum, brainstem, spinalcord, kidney, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
MRPL22mitochondrial ­ribosomal ­protein ­L22HSPC158, L22mt, MRP-L22, MRP-L25, RPML25, Q9NWU5, gene:2909321literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
IDH2isocitrate ­dehydrogenase ­(NADP(+)) ­2D2HGA2, ICD-M, IDH, IDHM, IDP, IDPM, mNADP-IDH, P48735, gene:341821literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS++MatrixMetabolism > Carbohydrate metabolism > TCA cycleall 14
GCDHglutaryl-CoA ­dehydrogenaseACAD5, GCD, Q92947, gene:263921literature, APEX_matrix, targetP signal+, mito protein domain+, coexpression, MS/MS++MatrixMetabolism > Amino acid metabolism > Lysine metabolismall 14
MRPL47mitochondrial ­ribosomal ­protein ­L47CGI-204, L47mt, MRP-L47, NCM1, Q9HD33, gene:5712921literature, APEX_matrix, targetP signal+, yeast mito homolog++, mito protein domain, induction, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > Translation > Mitochondrial ribosome		all 14
PPA2inorganic ­pyrophosphatase ­2HSPC124, SCFAI, SCFI, SID6-306, Q9H2U2, gene:2706821literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMitochondrial central dogma > mtDNA maintenance;
Mitochondrial central dogma > mtRNA metabolism;
Mitochondrial central dogma > Translation;
Metabolism > Nucleotide metabolism		all 14
MRPL9mitochondrial ­ribosomal ­protein ­L9L9mt, Q9BYD2, gene:6500521literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, induction, coexpression+, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
CHCHD10coiled-coil-helix-coiled-coil-helix ­domain ­containing ­10C22orf16, FTDALS2, IMMD, MIX17A, N27C7-4, SMAJ, Q8WYQ3, gene:40091621literature, targetP signal, yeast mito homolog+, mito protein domain, induction, coexpression++IMSnonenone
WARS2tryptophanyl ­tRNA ­synthetase ­2, ­mitochondrialNEMMLAS, TrpRS, mtTrpRS, Q9UGM6, gene:1035221literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MSMatrixMitochondrial central dogma > Translation > mt-tRNA synthetasescerebrum, cerebellum, brainstem, kidney, skeletalmuscle, adipose, largeintestine, placenta, testis
SLC25A19solute ­carrier ­family ­25 ­member ­19DNC, MCPHA, MUP1, THMD3, THMD4, TPC, Q9HC21, gene:6038621literature, yeast mito homolog++, mito protein domain+, induction, coexpression, MS/MSMIMMetabolism > Vitamin metabolism > Vitamin B1 metabolism;
Small molecule transport > SLC25A family		cerebellum, spinalcord, adipose, testis
CBR4carbonyl ­reductase ­4SDR45C1, Q8N4T8, gene:8486921literature, APEX_matrix, Rickettsial homolog, mito protein domain, induction, coexpression+, MS/MS++MatrixMetabolism > Lipid metabolism > Type II fatty acid synthesisall 14
SMDT1single-pass ­membrane ­protein ­with ­aspartate ­rich ­tail ­1C22orf32, DDDD, EMRE, Q9H4I9, gene:9168921literature, targetP signal, mito protein domain+, induction, coexpression+, MS/MSMIMSignaling > Calcium homeostasis > Calcium cycle;
Small molecule transport > Calcium uniporter		cerebrum, cerebellum, brainstem, spinalcord, kidney, adipose, smallintestine, largeintestine, stomach, placenta, testis
HAGHhydroxyacylglutathione ­hydrolaseGLO2, GLX2, GLXII, HAGH1, Q16775, gene:302921literature, APEX_matrix, targetP signal+, mito protein domain, induction, coexpression, MS/MS++MatrixMetabolism > Detoxification > ROS and glutathione metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
COX7A1cytochrome ­c ­oxidase ­subunit ­7A1COX7A, COX7AH, COX7AM, P24310, gene:134621literature, targetP signal, mito protein domain+, induction, coexpression, MS/MS+MIMOXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits		cerebrum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
MTG1mitochondrial ­ribosome ­associated ­GTPase ­1GTP, GTPBP7, Q9BT17, gene:9217021literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression+, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosome assemblylargeintestine, testis
COX14cytochrome ­c ­oxidase ­assembly ­factor ­COX14C12orf62, PCAG1, Q96I36, gene:8498721literature, GFP, mito protein domain+, induction, coexpression++, MS/MS+MIMMitochondrial central dogma > Translation > Translation factors;
OXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors		cerebrum, cerebellum, brainstem, spinalcord, kidney, heart, smallintestine, testis
NDUFA12NADH:ubiquinone ­oxidoreductase ­subunit ­A12B17.2, DAP13, MC1DN23, Q9UI09, gene:5596721literature, APEX_matrix, Rickettsial homolog, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		all 14
MRPS16mitochondrial ­ribosomal ­protein ­S16CGI-132, COXPD2, MRP-S16, RPMS16, Q9Y3D3, gene:5102121literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, MS/MS+MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
MTERF2mitochondrial ­transcription ­termination ­factor ­2MTERFD3, mTERFL, Q49AM1, gene:8029821literature, APEX_matrix, targetP signal+, mito protein domain+, induction, coexpression, MS/MSMatrixMitochondrial central dogma > mtDNA maintenance > mtDNA nucleoidcerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, largeintestine, testis
ETFRF1electron ­transfer ­flavoprotein ­regulatory ­factor ­1LYRM5, Q6IPR1, gene:14436321mito protein domain+, induction, coexpression, MS/MS++MatrixMetabolism > Electron carriers > Q-linked reactions, othercerebrum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta
GPD2glycerol-3-phosphate ­dehydrogenase ­2GDH2, GPDM, mGDH, mGPDH, P43304, gene:282021literature, APEX_IMS, yeast mito homolog++, mito protein domain, coexpression, MS/MS++MIMMetabolism > Carbohydrate metabolism > Glycerol phosphate shuttle;
Metabolism > Electron carriers > Q-linked reactions, other		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
NDUFA10NADH:ubiquinone ­oxidoreductase ­subunit ­A10CI-42KD, CI-42k, MC1DN22, O95299, gene:470521literature, targetP signal, mito protein domain, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		all 14
NDUFC2NADH:ubiquinone ­oxidoreductase ­subunit ­C2B14.5b, CI-B14.5b, HLC-1, NADHDH2, O95298, gene:471821literature, APEX_IMS, mito protein domain+, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		all 14
SLIRPSRA ­stem-loop ­interacting ­RNA ­binding ­proteinC14orf156, DC50, PD04872, Q9GZT3, gene:8189221literature, APEX_matrix, GFP, targetP signal+, mito protein domain, induction, coexpression, MS/MS++MatrixMitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay;
Mitochondrial central dogma > Translation		all 14
MT-ATP6ATP ­synthase ­F0 ­subunit ­6ATPase6, MTATP6, P00846, gene:450821literature, yeast mito homolog++, Rickettsial homolog, mito protein domain+, MS/MS+MIMOXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits		all 14
MT-CO2cytochrome ­c ­oxidase ­subunit ­IICOII, MTCO2, P00403, gene:451321literature, APEX_IMS, APEX_matrix, yeast mito homolog++, Rickettsial homolog, mito protein domain+, MS/MS+MIMOXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits		all 14
TIMM21translocase ­of ­inner ­mitochondrial ­membrane ­21C18orf55, HSPC154, TIM21, Q9BVV7, gene:2909021APEX_IMS, GFP, yeast mito homolog++, mito protein domain+, coexpression, MS/MSMIMMitochondrial central dogma > Translation > Translation factors;
Protein import, sorting and homeostasis > Protein import and sorting > TIM23 presequence pathway;
OXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors		cerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
NDUFA8NADH:ubiquinone ­oxidoreductase ­subunit ­A8CI-19KD, CI-PGIV, PGIV, P51970, gene:470221literature, APEX_IMS, mito protein domain, induction, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		all 14
GLSglutaminaseAAD20, CASGID, EIEE71, GAC, GAM, GDPAG, GLS1, KGA, O94925, gene:274421literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain+, MS/MS++MIMMetabolism > Amino acid metabolism > Glutamate metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
NDUFB11NADH:ubiquinone ­oxidoreductase ­subunit ­B11CI-ESSS, ESSS, MC1DN30, NP17.3, Np15, P17.3, Q9NX14, gene:5453920literature, APEX_IMS, APEX_matrix, targetP signal+, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		all 14
DHTKD1dehydrogenase ­E1 ­and ­transketolase ­domain ­containing ­1AMOXAD, CMT2Q, Q96HY7, gene:5552620APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain+, MS/MSMatrixMetabolism > Amino acid metabolism > Lysine metabolismcerebrum, kidney, liver, adipose, placenta, testis
FAHD2Afumarylacetoacetate ­hydrolase ­domain ­containing ­2ACGI-105, Q96GK7, gene:5101120APEX_matrix, targetP signal, mito protein domain, induction, coexpression, MS/MS++Matrixnoneall 14
HSD17B8hydroxysteroid ­17-beta ­dehydrogenase ­8D6S2245E, FABG, FABGL, H2-KE6, HKE6, KE6, RING2, SDR30C1, dJ1033B10.9, Q92506, gene:792320APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Lipid metabolism > Type II fatty acid synthesis;
Metabolism > Lipid metabolism > Cholesterol, bile acid, steroid synthesis		all 14
HINT2histidine ­triad ­nucleotide ­binding ­protein ­2HIT-17, Q9BX68, gene:8468120APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Lipid metabolism > Cholesterol, bile acid, steroid synthesisall 14
MRPS5mitochondrial ­ribosomal ­protein ­S5MRP-S5, S5mt, P82675, gene:6496920literature, APEX_matrix, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
C6orf136chromosome ­6 ­open ­reading ­frame ­136Q5SQH8, gene:22154520targetP signal, induction, coexpression++, MS/MS+Matrixnoneall 14
SPRYD4SPRY ­domain ­containing ­4Q8WW59, gene:28337720APEX_matrix, GFP, targetP signal+, mito protein domain, coexpression+, MS/MS++Matrixnoneall 14
LIPT2lipoyl(octanoyl) ­transferase ­2A6NK58, gene:38778720targetP signal, yeast mito homolog, Rickettsial homolog, mito protein domain, coexpression, MS/MSMatrixMetabolism > Lipid metabolism > Lipoate insertioncerebrum, cerebellum, brainstem, spinalcord, skeletalmuscle, adipose, largeintestine, placenta, testis
DECR12,4-dienoyl-CoA ­reductase ­1DECR, NADPH, SDR18C1, Q16698, gene:166620literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, coexpression+, MS/MS++MatrixMetabolism > Lipid metabolism > Fatty acid oxidationall 14
MRPS26mitochondrial ­ribosomal ­protein ­S26C20orf193, GI008, MRP-S13, MRP-S26, MRPS13, NY-BR-87, RPMS13, dJ534B8.3, Q9BYN8, gene:6494920literature, APEX_matrix, targetP signal, mito protein domain+, induction, coexpression, MS/MS+MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
SLC25A15solute ­carrier ­family ­25 ­member ­15D13S327, HHH, LNC-HC, ORC1, ORNT1, Q9Y619, gene:1016620literature, yeast mito homolog+, mito protein domain+, coexpression, MS/MS++MIMMetabolism > Amino acid metabolism > Urea cycle;
Small molecule transport > SLC25A family		spinalcord, kidney, liver, adipose, smallintestine, largeintestine, placenta
NDUFV3NADH:ubiquinone ­oxidoreductase ­subunit ­V3CI-10k, CI-9KD, P56181, gene:473120literature, APEX_matrix, targetP signal, induction, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		all 14
BPHLbiphenyl ­hydrolase ­likeBPH-RP, MCNAA, VACVASE, Q86WA6, gene:67020targetP signal+, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Detoxification > Xenobiotic metabolismall 14
STOML2stomatin ­like ­2HSPC108, SLP-2, Q9UJZ1, gene:3096820literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MIMProtein import, sorting and homeostasis > Protein homeostasisall 14
MSRB2methionine ­sulfoxide ­reductase ­B2CBS-1, CBS1, CGI-131, MSRB, PILB, Q9Y3D2, gene:2292120APEX_matrix, targetP signal, yeast mito homolog+, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Detoxification > ROS and glutathione metabolism;
Metabolism > Sulfur metabolism		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
LACTBlactamase ­betaG24, MRPL56, P83111, gene:11429420literature, APEX_IMS, targetP signal+, Rickettsial homolog, mito protein domain, coexpression, MS/MS++IMSProtein import, sorting and homeostasis > Protein homeostasis > Proteases;
Metabolism > Lipid metabolism > Phospholipid metabolism		all 14
TOMM40translocase ­of ­outer ­mitochondrial ­membrane ­40C19orf1, D19S1177E, PER-EC1, PEREC1, TOM40, O96008, gene:1045220literature, yeast mito homolog+, mito protein domain+, coexpression, MS/MS++MOMProtein import, sorting and homeostasis > Protein import and sorting > TOMcerebrum, spinalcord, kidney, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
SLC25A25solute ­carrier ­family ­25 ­member ­25MCSC, PCSCL, SCAMC-2, Q6KCM7, gene:11478920literature, yeast mito homolog+, mito protein domain+, coexpression, MS/MS++MIMMetabolism > Nucleotide metabolism > Nucleotide import;
Signaling > Calcium homeostasis > EF hand proteins;
Small molecule transport > SLC25A family		cerebrum, brainstem, spinalcord, liver, adipose, smallintestine, largeintestine, placenta
ADCK5aarF ­domain ­containing ­kinase ­5Q3MIX3, gene:20305420targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MSunknownnonecerebrum, largeintestine, testis
ACAD10acyl-CoA ­dehydrogenase ­family ­member ­10Q6JQN1, gene:8072420APEX_matrix, targetP signal+, mito protein domain+, coexpression, MS/MS++MatrixMetabolism > Lipid metabolism > Fatty acid oxidationcerebrum, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
VWA8von ­Willebrand ­factor ­A ­domain ­containing ­8KIAA0564, P7BP2, A3KMH1, gene:2307820APEX_matrix, targetP signal, mito protein domain, coexpression+, MS/MS++Matrixnoneall 14
CCDC90Bcoiled-coil ­domain ­containing ­90BMDS011, MDS025, Q9GZT6, gene:6049220literature, APEX_matrix, targetP signal+, yeast mito homolog+, mito protein domain+, MS/MSMIMnoneall 14
DNAJC11DnaJ ­heat ­shock ­protein ­family ­(Hsp40) ­member ­C11dJ126A5.1, Q9NVH1, gene:5573520GFP, yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MOMProtein import, sorting and homeostasis > Protein import and sorting;
Mitochondrial dynamics and surveillance > Intramitochondrial membrane interactions		all 14
ALKBH7alkB ­homolog ­7ABH7, SPATA11, UNQ6002, Q9BT30, gene:8426620APEX_matrix, targetP signal, mito protein domain, induction, coexpression++, MS/MSMatrixnonecerebellum, brainstem, largeintestine, testis
GLDCglycine ­decarboxylaseGCE, GCSP, HYGN1, P23378, gene:273120literature, APEX_matrix, targetP signal, yeast mito homolog, mito protein domain+, coexpression, MS/MS++MatrixMetabolism > Amino acid metabolism > Glycine metabolism;
Metabolism > Amino acid metabolism > Glycine cleavage system		cerebrum, cerebellum, kidney, liver, adipose, placenta
MPC1Lmitochondrial ­pyruvate ­carrier ­1 ­likeSLC54A3, P0DKB6, gene:34741120targetP signal, yeast mito homolog+, mito protein domain+, MS/MS++MIMMetabolism > Carbohydrate metabolism > Gluconeogenesis;
Metabolism > Carbohydrate metabolism > Pyruvate metabolism;
Metabolism > Carbohydrate metabolism > TCA-associated;
Small molecule transport		testis
THEM4thioesterase ­superfamily ­member ­4CTMP, Q5T1C6, gene:11714520literature, APEX_matrix, targetP signal+, yeast mito homolog, mito protein domain, induction, MS/MS++MIMMetabolism > Lipid metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, placenta, testis
SSBP1single ­stranded ­DNA ­binding ­protein ­1Mt-SSB, SOSS-B1, SSBP, mtSSB, Q04837, gene:674219literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, MS/MSMatrixMitochondrial central dogma > mtDNA maintenance > mtDNA replication;
Mitochondrial central dogma > mtDNA maintenance > mtDNA nucleoid		all 14
MRPL27mitochondrial ­ribosomal ­protein ­L27L27mt, Q9P0M9, gene:5126419literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
HSCBHscB ­mitochondrial ­iron-sulfur ­cluster ­cochaperoneDNAJC20, HSC20, JAC1, Q8IWL3, gene:15027419APEX_matrix, GFP, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, MS/MSMatrixMetabolism > Metals and cofactors > Fe-S cluster biosynthesiscerebrum, cerebellum, brainstem, spinalcord, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
MRPS10mitochondrial ­ribosomal ­protein ­S10MRP-S10, PNAS-122, P82664, gene:5517319literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
AK4adenylate ­kinase ­4AK 4, AK3, AK3L1, AK3L2, P27144, gene:20519APEX_matrix, yeast mito homolog++, Rickettsial homolog, mito protein domain, MS/MS++MatrixMetabolism > Nucleotide metabolism > Nucleotide synthesis and processingall 14
ATAD3AATPase ­family ­AAA ­domain ­containing ­3AHAYOS, PHRINL, Q9NVI7, gene:5521019APEX_IMS, APEX_matrix, GFP, Rickettsial homolog, mito protein domain+, coexpression, MS/MS++MIMMitochondrial central dogma > mtDNA maintenance > mtDNA nucleoid;
Mitochondrial dynamics and surveillance > Organelle contact sites;
Mitochondrial dynamics and surveillance > Intramitochondrial membrane interactions		all 14
MRPL37mitochondrial ­ribosomal ­protein ­L37L2mt, L37mt, MRP-L2, MRP-L37, MRPL2, RPML2, Q9BZE1, gene:5125319literature, APEX_matrix, targetP signal, mito protein domain, induction, coexpression+, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomekidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
PTGES2prostaglandin ­E ­synthase ­2C9orf15, GBF-1, GBF1, PGES2, mPGES-2, Q9H7Z7, gene:8014219literature, APEX_IMS, APEX_matrix, targetP signal, mito protein domain, induction, coexpression+, MS/MS++MIMMetabolism > Lipid metabolism > Eicosanoid metabolismall 14
TXNRD2thioredoxin ­reductase ­2GCCD5, SELZ, TR, TR-BETA, TR3, TRXR2, Q9NNW7, gene:1058719literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Detoxification > ROS and glutathione metabolism;
Metabolism > Detoxification > Selenoproteins		cerebrum, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
ACSM5acyl-CoA ­synthetase ­medium ­chain ­family ­member ­5Q6NUN0, gene:5498819literature, targetP signal+, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Detoxification > Xenobiotic metabolism		kidney, liver, adipose, smallintestine, largeintestine
CISD1CDGSH ­iron ­sulfur ­domain ­1C10orf70, MDS029, ZCD1, mitoNEET, Q9NZ45, gene:5584719literature, mito protein domain, induction, coexpression++, MS/MS++MOMMetabolism > Metals and cofactors > Fe-S cluster biosynthesis;
Metabolism > Metals and cofactors > Fe-S-containing proteins		all 14
MRPL41mitochondrial ­ribosomal ­protein ­L41BMRP, MRP-L27, MRPL27, PIG3, RPML27, Q8IXM3, gene:6497519literature, APEX_matrix, yeast mito homolog++, mito protein domain, induction, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
MTARC2mitochondrial ­amidoxime ­reducing ­component ­2MARC2, MOSC2, Q969Z3, gene:5499619literature, targetP signal, mito protein domain+, induction, coexpression, MS/MS++MOMMetabolism > Metals and cofactors > Molybdenum cofactor synthesis and proteins;
Metabolism > Detoxification > Xenobiotic metabolism;
Metabolism > Detoxification > Amidoxime reducing complex		all 14
MRPS6mitochondrial ­ribosomal ­protein ­S6C21orf101, MRP-S6, RPMS6, S6mt, P82932, gene:6496819literature, APEX_matrix, yeast mito homolog++, Rickettsial homolog, mito protein domain, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
MRPL23mitochondrial ­ribosomal ­protein ­L23L23MRP, RPL23, RPL23L, Q16540, gene:615019literature, APEX_matrix, targetP signal, yeast mito homolog++, mito protein domain, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
SQORsulfide ­quinone ­oxidoreductaseCGI-44, PRO1975, SQR, SQRDL, Q9Y6N5, gene:5847219literature, targetP signal, yeast mito homolog++, mito protein domain, MS/MS++MIMMetabolism > Electron carriers > Q-linked reactions, other;
Metabolism > Sulfur metabolism		spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
SCO1synthesis ­of ­cytochrome ­C ­oxidase ­1SCOD1, O75880, gene:634119literature, APEX_IMS, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, MS/MS++MIMOXPHOS > Complex IV > CIV assembly factors;
Metabolism > Metals and cofactors > Copper metabolism;
OXPHOS > OXPHOS assembly factors		cerebrum, cerebellum, brainstem, spinalcord, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
GCATglycine ­C-acetyltransferaseKBL, O75600, gene:2346419literature, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, MS/MS++unknownMetabolism > Amino acid metabolism > Glycine metabolismbrainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
MTHFD1Lmethylenetetrahydrofolate ­dehydrogenase ­(NADP+ ­dependent) ­1 ­likeFTHFSDC1, MTC1THFS, dJ292B18.2, Q6UB35, gene:2590219literature, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, MS/MS++MatrixMetabolism > Vitamin metabolism > Folate and 1-C metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
ECI2enoyl-CoA ­delta ­isomerase ­2ACBD2, DRS-1, DRS1, HCA88, PECI, dJ1013A10.3, O75521, gene:1045519literature, APEX_matrix, targetP signal+, mito protein domain, coexpression+, MS/MS++MatrixMetabolism > Lipid metabolism > Fatty acid oxidationall 14
UQCRBubiquinol-cytochrome ­c ­reductase ­binding ­proteinMC3DN3, QCR7, QP-C, QPC, UQBC, UQBP, UQCR6, UQPC, P14927, gene:738119literature, APEX_matrix, yeast mito homolog++, mito protein domain+, MS/MS++MIMOXPHOS > Complex III > CIII subunits;
OXPHOS > OXPHOS subunits		all 14
ATP5PDATP ­synthase ­peripheral ­stalk ­subunit ­dAPT5H, ATP5H, ATPQ, O75947, gene:1047619literature, APEX_matrix, yeast mito homolog++, mito protein domain+, MS/MS++MIMOXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits		all 14
ATP5MGATP ­synthase ­membrane ­subunit ­gATP5JG, ATP5L, O75964, gene:1063219literature, APEX_matrix, yeast mito homolog++, mito protein domain+, MS/MS++MIMOXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits;
Mitochondrial dynamics and surveillance > Cristae formation		all 14
CCDC58coiled-coil ­domain ­containing ­58Q4VC31, gene:13107619APEX_IMS, yeast mito homolog++, mito protein domain+, MS/MS++IMSnoneall 14
MCCC2methylcrotonoyl-CoA ­carboxylase ­2MCCB, Q9HCC0, gene:6408719literature, targetP signal, Rickettsial homolog, mito protein domain, coexpression+, MS/MS++MatrixMetabolism > Amino acid metabolism > BCAA metabolismall 14
MCEEmethylmalonyl-CoA ­epimeraseGLOD2, MCE, Q96PE7, gene:8469319targetP signal, induction, coexpression++, MS/MS++MatrixMetabolism > Carbohydrate metabolism > Propanoate metabolism;
Metabolism > Lipid metabolism > Fatty acid oxidation		all 14
MOCS1molybdenum ­cofactor ­synthesis ­1MIG11, MOCOD, MOCS1A, MOCS1B, Q9NZB8, gene:433719APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain+, MS/MS+MatrixMetabolism > Metals and cofactors > Molybdenum cofactor synthesis and proteinscerebrum, liver, adipose, smallintestine, largeintestine, stomach, placenta
MMUTmethylmalonyl-CoA ­mutaseMCM, MUT, P22033, gene:459419literature, APEX_matrix, targetP signal, mito protein domain+, coexpression, MS/MS++MatrixMetabolism > Carbohydrate metabolism > Propanoate metabolism;
Metabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Vitamin metabolism > Vitamin B12 metabolism		all 14
SLC25A51solute ­carrier ­family ­25 ­member ­51CG7943, MCART1, Q9H1U9, gene:9201419GFP, yeast mito homolog+, mito protein domain+, coexpression, MS/MS++MIMMetabolism > Metals and cofactors > NAD biosynthesis and metabolism;
Small molecule transport > SLC25A family		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
SPG7SPG7 ­matrix ­AAA ­peptidase ­subunit, ­parapleginCAR, CMAR, PGN, SPG5C, Q9UQ90, gene:668719literature, targetP signal, Rickettsial homolog, mito protein domain+, coexpression+, MS/MSMIMProtein import, sorting and homeostasis > Protein homeostasis > Proteasescerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
ATP5MC3ATP ­synthase ­membrane ­subunit ­c ­locus ­3ATP5G3, P3, P48201, gene:51819literature, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpression++MIMOXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits		none
ATAD1ATPase ­family ­AAA ­domain ­containing ­1AFDC1, FNP001, HKPX4, Msp1, THORASE, Q8NBU5, gene:8489619yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MOMProtein import, sorting and homeostasis > Protein homeostasis > Proteasescerebrum, cerebellum, brainstem, spinalcord, kidney, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
OXNAD1oxidoreductase ­NAD ­binding ­domain ­containing ­1Q96HP4, gene:9210619APEX_matrix, GFP, mito protein domain, coexpression++, MS/MS++Matrixnonecerebrum, spinalcord, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
MTG2mitochondrial ­ribosome ­associated ­GTPase ­2GTPBP5, ObgH1, dJ1005F21.2, Q9H4K7, gene:2616419literature, APEX_matrix, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpressionMatrixMitochondrial central dogma > Translation > Mitochondrial ribosome assemblylargeintestine, placenta
DARS2aspartyl-tRNA ­synthetase ­2, ­mitochondrialASPRS, LBSL, MT-ASPRS, mtAspRS, Q6PI48, gene:5515719APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MSMatrixMitochondrial central dogma > Translation > mt-tRNA synthetasescerebrum, cerebellum, brainstem, spinalcord, kidney, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
MRPL51mitochondrial ­ribosomal ­protein ­L51CDA09, HSPC241, MRP64, bMRP64, Q4U2R6, gene:5125819literature, APEX_matrix, targetP signal, mito protein domain+, coexpression, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
PCpyruvate ­carboxylasePCB, P11498, gene:509119literature, targetP signal, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Carbohydrate metabolism > Gluconeogenesis;
Metabolism > Carbohydrate metabolism > TCA-associated;
Metabolism > Vitamin metabolism > Biotin utilizing proteins		all 14
CLYBLcitramalyl-CoA ­lyaseCLB, Q8N0X4, gene:17142519APEX_matrix, targetP signal+, induction, coexpression+, MS/MS++MatrixMetabolism > Carbohydrate metabolism > Itaconate metabolismall 14
PNPLA8patatin ­like ­phospholipase ­domain ­containing ­8IPLA2-2, IPLA2G, MMLA, PNPLA-gamma, iPLA2gamma, Q9NP80, gene:5064019APEX_matrix, Rickettsial homolog, induction, coexpression++, MS/MS+MIMMetabolism > Lipid metabolism > Cardiolipin synthesis;
Metabolism > Lipid metabolism > Phospholipid metabolism		cerebrum, cerebellum, brainstem, spinalcord, liver, adipose, largeintestine, testis
MLYCDmalonyl-CoA ­decarboxylaseMCD, O95822, gene:2341719literature, APEX_matrix, targetP signal+, mito protein domain, coexpression++, MS/MSMatrixMetabolism > Lipid metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta
PYURFPIGY ­upstream ­reading ­framePREY, Q96I23, gene:10099693919targetP signal+, induction, coexpression++, MS/MSunknownnonecerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta
TIMM17Btranslocase ­of ­inner ­mitochondrial ­membrane ­17BDXS9822, JM3, TIM17B, O60830, gene:1024519literature, APEX_IMS, yeast mito homolog+, mito protein domain+, coexpression, MS/MS+MIMProtein import, sorting and homeostasis > Protein import and sorting > TIM23 presequence pathwayall 14
MRPL32mitochondrial ­ribosomal ­protein ­L32HSPC283, L32mt, MRP-L32, bMRP-59b, Q9BYC8, gene:6498319literature, APEX_matrix, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
SLC25A26solute ­carrier ­family ­25 ­member ­26COXPD28, SAMC, Q70HW3, gene:11528619literature, yeast mito homolog++, mito protein domain+, coexpression, MS/MSMIMSmall molecule transport > SLC25A familycerebrum, cerebellum, adipose, largeintestine, placenta, testis
PRODH2proline ­dehydrogenase ­2HSPOX1, HYPDH, Q9UF12, gene:5851019targetP signal, yeast mito homolog+, mito protein domain+, MS/MS+MIMMetabolism > Amino acid metabolism > Glyoxylate metabolism;
Metabolism > Electron carriers > Q-linked reactions, other		kidney, liver
NIT2nitrilase ­family ­member ­2HEL-S-8a, Q9NQR4, gene:5695419Rickettsial homolog, mito protein domain, coexpression, MS/MS++unknownMetabolism > Detoxificationcerebellum, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
FDX2ferredoxin ­2FDX1L, MEOAL, Q6P4F2, gene:11281218APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpression, MS/MSMatrixMetabolism > Metals and cofactors > Heme synthesis and processing;
Metabolism > Metals and cofactors > Fe-S cluster biosynthesis;
Metabolism > Metals and cofactors > Fe-S-containing proteins		cerebrum, cerebellum, brainstem, spinalcord, liver, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
ME2malic ­enzyme ­2ODS1, P23368, gene:420018literature, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, MS/MS++MatrixMetabolism > Carbohydrate metabolism > TCA-associatedcerebrum, cerebellum, brainstem, spinalcord, kidney, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
TUFMTu ­translation ­elongation ­factor, ­mitochondrialCOXPD4, EF-TuMT, EFTU, P43, P49411, gene:728418literature, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, MS/MS++MatrixMitochondrial central dogma > Translation > Translation factorsall 14
COX6A2cytochrome ­c ­oxidase ­subunit ­6A2COX6AH, COXVIAH, Q02221, gene:133918literature, targetP signal, yeast mito homolog+, mito protein domain+, induction, coexpressionMIMOXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits		heart, skeletalmuscle
SLC30A9solute ­carrier ­family ­30 ­member ­9BILAPES, C4orf1, GAC63, HUEL, ZNT9, Q6PML9, gene:1046318APEX_matrix, targetP signal+, yeast mito homolog++, mito protein domain, induction, MS/MSMIMSmall molecule transportcerebrum, cerebellum, brainstem, spinalcord, adipose, largeintestine, stomach, placenta, testis
TAMM41TAM41 ­mitochondrial ­translocator ­assembly ­and ­maintenance ­homologC3orf31, RAM41, TAM41, Q96BW9, gene:13200118GFP, yeast mito homolog++, mito protein domain+, MS/MS++MIMMetabolism > Lipid metabolism > Cardiolipin synthesis;
Metabolism > Lipid metabolism > Phospholipid metabolism		cerebrum, cerebellum, brainstem, spinalcord, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
TIMM23translocase ­of ­inner ­mitochondrial ­membrane ­23TIM23, O14925, gene:10028793218literature, APEX_IMS, yeast mito homolog++, mito protein domain+, MS/MS++MIMProtein import, sorting and homeostasis > Protein import and sorting > TIM23 presequence pathwayall 14
RMND1required ­for ­meiotic ­nuclear ­division ­1 ­homologC6orf96, COXPD11, RMD1, bA351K16, bA351K16.3, Q9NWS8, gene:5500518literature, GFP, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, MS/MSMIMMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > Translation		cerebrum, cerebellum, brainstem, spinalcord, adipose, smallintestine, largeintestine, stomach, placenta, testis
MFN2mitofusin ­2CMT2A, CMT2A2, CMT2A2A, CMT2A2B, CPRP1, HMSN6A, HSG, MARF, O95140, gene:992718literature, mito protein domain+, induction, coexpression, MS/MS+MOMMitochondrial dynamics and surveillance > Fusion;
Mitochondrial dynamics and surveillance > Organelle contact sites		cerebrum, cerebellum, brainstem, spinalcord, liver, adipose, largeintestine, stomach, placenta, testis
DNLZDNL-type ­zinc ­fingerC9orf151, HEP, HEP1, TIMM15, ZIM17, bA413M3.2, Q5SXM8, gene:72848918APEX_matrix, targetP signal, yeast mito homolog++, mito protein domain+, MS/MSMatrixProtein import, sorting and homeostasis > Protein homeostasis > Chaperonescerebrum, cerebellum, brainstem, heart, adipose, smallintestine, largeintestine, stomach, placenta
CHCHD4coiled-coil-helix-coiled-coil-helix ­domain ­containing ­4MIA40, TIMM40, Q8N4Q1, gene:13147418literature, APEX_IMS, yeast mito homolog++, mito protein domain, induction, coexpression, MS/MS+IMSProtein import, sorting and homeostasis > Protein import and sorting > MIA40cerebrum, cerebellum, brainstem, spinalcord, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
NDUFAB1NADH:ubiquinone ­oxidoreductase ­subunit ­AB1ACP, ACP1, FASN2A, SDAP, O14561, gene:470618literature, APEX_matrix, targetP signal+, yeast mito homolog, Rickettsial homolog, mito protein domain, MS/MS+MIMOXPHOS > Complex I > CI subunits;
Metabolism > Lipid metabolism > Type II fatty acid synthesis;
Metabolism > Metals and cofactors > Fe-S cluster biosynthesis;
OXPHOS > OXPHOS subunits		all 14
ACSM1acyl-CoA ­synthetase ­medium ­chain ­family ­member ­1BUCS1, MACS1, Q08AH1, gene:11628518literature, targetP signal, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Lipid metabolism > Fatty acid oxidationspinalcord, kidney, liver, smallintestine
COA5cytochrome ­c ­oxidase ­assembly ­factor ­56330578E17Rik, C2orf64, CEMCOX3, Pet191, Q86WW8, gene:49375318literature, APEX_IMS, yeast mito homolog++, mito protein domain+, induction, MS/MSIMSOXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors		adipose, placenta, testis
COQ2coenzyme ­Q2, ­polyprenyltransferaseCL640, COQ10D1, MSA1, PHB:PPT, Q96H96, gene:2723518literature, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression+MIMMetabolism > Metals and cofactors > Coenzyme Q metabolismnone
PDSS2decaprenyl ­diphosphate ­synthase ­subunit ­2C6orf210, COQ10D3, COQ1B, DLP1, bA59I9.3, hDLP1, Q86YH6, gene:5710718literature, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpression+MIMMetabolism > Metals and cofactors > Coenzyme Q metabolismsmallintestine, largeintestine
EHHADHenoyl-CoA ­hydratase ­and ­3-hydroxyacyl ­CoA ­dehydrogenaseECHD, FRTS3, L-PBE, LBFP, LBP, MFE1, PBFE, Q08426, gene:196218yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpression, MS/MS+unknownMetabolism > Lipid metabolismspinalcord, kidney, liver, adipose, smallintestine, largeintestine, placenta
NDUFS3NADH:ubiquinone ­oxidoreductase ­core ­subunit ­S3CI-30, MC1DN8, O75489, gene:472218literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		all 14
ABCB10ATP ­binding ­cassette ­subfamily ­B ­member ­10EST20237, M-ABC2, MTABC2, Q9NRK6, gene:2345618literature, APEX_matrix, yeast mito homolog+, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS+MIMMetabolism > Metals and cofactors > Heme synthesis and processing;
Small molecule transport > ABC transporters		cerebellum, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
CKMT2creatine ­kinase, ­mitochondrial ­2SMTCK, P17540, gene:116018literature, targetP signal, mito protein domain, induction, coexpression, MS/MS++MIMMetabolism > Nucleotide metabolism > Creatine metabolismcerebrum, cerebellum, brainstem, spinalcord, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta
MRPS25mitochondrial ­ribosomal ­protein ­S25MRP-S25, RPMS25, P82663, gene:6443218literature, APEX_matrix, targetP signal, mito protein domain, induction, coexpression, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
ACAD9acyl-CoA ­dehydrogenase ­family ­member ­9MC1DN20, NPD002, Q9H845, gene:2897618literature, APEX_matrix, targetP signal+, mito protein domain+, MS/MS++MIMOXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors		all 14
COX16cytochrome ­c ­oxidase ­assembly ­factor ­COX16C14orf112, HSPC203, hCOX16, Q9P0S2, gene:5124118APEX_IMS, GFP, yeast mito homolog++, mito protein domain+, MS/MS+MIMOXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors		kidney, liver, adipose, largeintestine, placenta, testis
FAM210Afamily ­with ­sequence ­similarity ­210 ­member ­AC18orf19, HsT2329, Q96ND0, gene:12522818GFP, targetP signal, induction, coexpression++, MS/MSMIMnoneall 14
ACOT13acyl-CoA ­thioesterase ­13HT012, PNAS-27, THEM2, Q9NPJ3, gene:5585618mito protein domain, induction, coexpression, MS/MS++MatrixMetabolism > Lipid metabolismall 14
CPT1Acarnitine ­palmitoyltransferase ­1ACPT1, CPT1-L, L-CPT1, P50416, gene:137418literature, yeast mito homolog+, mito protein domain, induction, coexpression, MS/MS++MOMMetabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Metals and cofactors > Carnitine synthesis and transport;
Metabolism > Metals and cofactors > Carnitine shuttle		all 14
DHRS4dehydrogenase/reductase ­4CR, NRDR, PHCR, PSCD, SCAD-SRL, SDR-SRL, SDR25C1, SDR25C2, Q9BTZ2, gene:1090118targetP signal, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Vitamin metabolism > Vitamin A metabolismall 14
PRELID2PRELI ­domain ­containing ­2Q8N945, gene:15376818yeast mito homolog+, mito protein domain+, induction, coexpression, MS/MSIMSnonelargeintestine, stomach
CARS2cysteinyl-tRNA ­synthetase ­2, ­mitochondrialCOXPD27, cysRS, Q9HA77, gene:7958718APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, coexpression++, MS/MSMatrixMitochondrial central dogma > Translation > mt-tRNA synthetasescerebrum, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
NIPSNAP2nipsnap ­homolog ­2GBAS, O75323, gene:263118literature, APEX_matrix, targetP signal+, induction, coexpression, MS/MS++MatrixMitochondrial dynamics and surveillance > Mitophagy;
Mitochondrial dynamics and surveillance > Autophagy		all 14
NDUFB7NADH:ubiquinone ­oxidoreductase ­subunit ­B7B18, CI-B18, P17568, gene:471318literature, APEX_IMS, APEX_matrix, induction, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		all 14
MICOS13mitochondrial ­contact ­site ­and ­cristae ­organizing ­system ­subunit ­13C19orf70, MIC13, P117, QIL1, Q5XKP0, gene:12598818induction, coexpression++, MS/MS++MIMMitochondrial dynamics and surveillance > Cristae formation > MICOS complexall 14
GLRX2glutaredoxin ­2CGI-133, GRX2, Q9NS18, gene:5102217literature, targetP signal+, yeast mito homolog, Rickettsial homolog, mito protein domain, coexpression, MS/MSMatrixMetabolism > Metals and cofactors > Fe-S-containing proteins;
Metabolism > Vitamin metabolism > Vitamin C metabolism;
Metabolism > Detoxification > ROS and glutathione metabolism		cerebrum, cerebellum, brainstem, spinalcord, skeletalmuscle, largeintestine, placenta, testis
MRPS30mitochondrial ­ribosomal ­protein ­S30MRP-S30, PAP, PDCD9, S30mt, Q9NP92, gene:1088417literature, APEX_matrix, targetP signal, mito protein domain, induction, coexpression, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, spinalcord, kidney, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
SCO2synthesis ­of ­cytochrome ­C ­oxidase ­2CEMCOX1, ECGF1, Gliostatin, MYP6, PD-ECGF, SCO1L, TP, TYMP, TdRPase, O43819, gene:999717literature, APEX_IMS, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain, MS/MSMIMOXPHOS > Complex IV > CIV assembly factors;
Metabolism > Metals and cofactors > Copper metabolism;
OXPHOS > OXPHOS assembly factors		all 14
HSD17B10hydroxysteroid ­17-beta ­dehydrogenase ­1017b-HSD10, ABAD, CAMR, DUPXp11.22, ERAB, HADH2, HCD2, HSD10MD, MHBD, MRPP2, MRX17, MRX31, MRXS10, SCHAD, SDR5C1, Q99714, gene:302817literature, APEX_matrix, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS++MatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > Polycistronic mtRNA processing;
Mitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications;
Metabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Lipid metabolism > Cholesterol, bile acid, steroid synthesis;
Metabolism > Amino acid metabolism > BCAA metabolism		all 14
NARS2asparaginyl-tRNA ­synthetase ­2, ­mitochondrialDFNB94, SLM5, asnRS, Q96I59, gene:7973117GFP, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpressionMatrixMitochondrial central dogma > Translation > mt-tRNA synthetasesspinalcord, adipose, largeintestine, stomach, placenta
RPUSD3RNA ­pseudouridine ­synthase ­D3Q6P087, gene:28536717APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications		largeintestine
SUOXsulfite ­oxidaseP51687, gene:682117APEX_IMS, targetP signal, mito protein domain+, coexpression, MS/MS++IMSMetabolism > Metals and cofactors > Molybdenum cofactor synthesis and proteins;
Metabolism > Sulfur metabolism		cerebrum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
SARDHsarcosine ­dehydrogenaseBPR-2, DMGDHL1, SAR, SARD, SDH, Q9UL12, gene:175717literature, targetP signal+, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Amino acid metabolism > Glycine metabolism;
Metabolism > Vitamin metabolism > Choline and betaine metabolism;
Metabolism > Vitamin metabolism > Folate and 1-C metabolism		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
RIDAreactive ­intermediate ­imine ­deaminase ­A ­homologHRSP12, P14.5, PSP, UK114, hp14.5, P52758, gene:1024717APEX_matrix, yeast mito homolog++, mito protein domain, coexpression, MS/MS+Matrixnonecerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
COX8Acytochrome ­c ­oxidase ­subunit ­8ACOX, COX8, COX8-2, COX8L, VIII, VIII-L, P10176, gene:135117literature, targetP signal, mito protein domain+, coexpression+, MS/MSMIMOXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits		stomach, placenta, testis
SPRsepiapterin ­reductaseSDR38C1, P35270, gene:669717Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS++unknownMetabolism > Metals and cofactors > Tetrahydrobiopterin synthesiscerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, largeintestine, stomach
MPV17mitochondrial ­inner ­membrane ­protein ­MPV17CMT2EE, MTDPS6, SYM1, P39210, gene:435817literature, yeast mito homolog+, mito protein domain, coexpression, MS/MS++MIMSmall molecule transportall 14
ZADH2zinc ­binding ­alcohol ­dehydrogenase ­domain ­containing ­2PRG-3, Q8N4Q0, gene:28427317mito protein domain, induction, coexpression+, MS/MS++unknownMetabolism > Lipid metabolism > Eicosanoid metabolismall 14
MRPL18mitochondrial ­ribosomal ­protein ­L18HSPC071, L18mt, MRP-L18, Q9H0U6, gene:2907417literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, coexpression, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
ALDH1B1aldehyde ­dehydrogenase ­1 ­family ­member ­B1ALDH5, ALDHX, P30837, gene:21917literature, APEX_matrix, targetP signal, yeast mito homolog+, mito protein domain, MS/MS++MatrixMetabolism > Detoxification > Xenobiotic metabolismall 14
CHCHD1coiled-coil-helix-coiled-coil-helix ­domain ­containing ­1C10orf34, C2360, MRP-S37, Q96BP2, gene:11848717GFP, targetP signal, yeast mito homolog+, mito protein domain, coexpression+, MS/MSMIMMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
TIMM8Atranslocase ­of ­inner ­mitochondrial ­membrane ­8ADDP, DDP1, DFN1, MTS, TIM8, O60220, gene:167817literature, yeast mito homolog+, mito protein domain+, MS/MS++MIMProtein import, sorting and homeostasis > Protein homeostasis > Chaperones;
Protein import, sorting and homeostasis > Protein import and sorting		all 14
VDAC3voltage ­dependent ­anion ­channel ­3HD-VDAC3, VDAC-3, Q9Y277, gene:741917literature, yeast mito homolog+, mito protein domain+, MS/MS++MOMSignaling > Calcium homeostasis > Mitochondrial permeability transition pore;
Small molecule transport		all 14
SLC25A24solute ­carrier ­family ­25 ­member ­24APC1, SCAMC-1, SCAMC1, Q6NUK1, gene:2995717literature, APEX_IMS, yeast mito homolog+, mito protein domain+, MS/MS++MIMMetabolism > Nucleotide metabolism > Nucleotide import;
Signaling > Calcium homeostasis > EF hand proteins;
Small molecule transport > SLC25A family		adipose, smallintestine, largeintestine, stomach, placenta, testis
MPC1mitochondrial ­pyruvate ­carrier ­1BRP44L, CGI-129, MPYCD, SLC54A1, Q9Y5U8, gene:5166017GFP, yeast mito homolog+, mito protein domain+, MS/MS++MIMMetabolism > Carbohydrate metabolism > Gluconeogenesis;
Metabolism > Carbohydrate metabolism > Pyruvate metabolism;
Metabolism > Carbohydrate metabolism > TCA-associated;
Small molecule transport		all 14
SLC25A22solute ­carrier ­family ­25 ­member ­22EIEE3, GC-1, GC1, NET44, Q9H936, gene:7975117literature, yeast mito homolog+, mito protein domain+, MS/MS++MIMSmall molecule transport > SLC25A familycerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
SLC25A31solute ­carrier ­family ­25 ­member ­31AAC4, ANT 4, ANT4, SFEC35kDa, Q9H0C2, gene:8344717literature, yeast mito homolog+, mito protein domain+, MS/MS++MIMMetabolism > Nucleotide metabolism > Nucleotide import;
Signaling > Calcium homeostasis > Mitochondrial permeability transition pore;
Small molecule transport > SLC25A family		all 14
SLC25A18solute ­carrier ­family ­25 ­member ­18GC2, Q9H1K4, gene:8373317literature, yeast mito homolog+, mito protein domain+, MS/MS++MIMSmall molecule transport > SLC25A familycerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
TRNT1tRNA ­nucleotidyl ­transferase ­1CCA1, CGI-47, MtCCA, RPEM, SIFD, Q96Q11, gene:5109517literature, targetP signal, yeast mito homolog, Rickettsial homolog, mito protein domain, induction, MS/MSMatrixMitochondrial central dogma > mtRNA metabolism > mt-tRNA modificationsspinalcord, adipose, placenta, testis
IDEinsulin ­degrading ­enzymeINSULYSIN, P14735, gene:341617literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain+, coexpression, MS/MS+MatrixProtein import, sorting and homeostasis > Protein homeostasis > Proteasescerebrum, cerebellum, brainstem, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
GSTK1glutathione ­S-transferase ­kappa ­1GST, GST 13-13, GST13, GST13-13, GSTK1-1, hGSTK1, Q9Y2Q3, gene:37315617literature, mito protein domain+, coexpression+, MS/MS++MatrixMetabolism > Detoxification > Xenobiotic metabolismall 14
VARS2valyl-tRNA ­synthetase ­2, ­mitochondrialCOXPD20, VALRS, VARS2L, VARSL, Q5ST30, gene:5717617APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMitochondrial central dogma > Translation > mt-tRNA synthetasescerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
HSPE1heat ­shock ­protein ­family ­E ­(Hsp10) ­member ­1CPN10, EPF, GROES, HSP10, P61604, gene:333617literature, APEX_IMS, APEX_matrix, yeast mito homolog++, Rickettsial homolog, mito protein domain, MS/MS++MatrixProtein import, sorting and homeostasis > Protein homeostasis > Chaperonesall 14
MRPL54mitochondrial ­ribosomal ­protein ­L54L54mt, MRP-L54, Q6P161, gene:11654117literature, APEX_matrix, yeast mito homolog++, mito protein domain, coexpression, MS/MS+MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
MRPL38mitochondrial ­ribosomal ­protein ­L38HSPC262, L38MT, MRP-L3, MRP-L38, RPML3, Q96DV4, gene:6497817APEX_matrix, targetP signal, mito protein domain, coexpression++, MS/MS+MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, spinalcord, heart, smallintestine, largeintestine, stomach, placenta, testis
MRPL58mitochondrial ­ribosomal ­protein ­L58DS-1, DS1, ICT1, MRP-L58, Q14197, gene:339617APEX_matrix, GFP, targetP signal+, mito protein domain, induction, coexpression, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosome;
Mitochondrial central dogma > Translation > Translation factors		all 14
MTCH1mitochondrial ­carrier ­1CGI-64, PIG60, PSAP, SLC25A49, Q9NZJ7, gene:2378717literature, yeast mito homolog+, mito protein domain+, MS/MS++MOMSmall molecule transport > SLC25A familycerebrum, cerebellum, brainstem, spinalcord, kidney, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
TRIAP1TP53 ­regulated ­inhibitor ­of ­apoptosis ­1HSPC132, MDM35, P53CSV, WF-1, O43715, gene:5149917literature, yeast mito homolog++, mito protein domain, induction, MS/MS++IMSMetabolism > Lipid metabolism > Cardiolipin synthesis;
Metabolism > Lipid metabolism > Phospholipid metabolism		adipose, stomach, placenta
PTCD3pentatricopeptide ­repeat ­domain ­3MRP-S39, Q96EY7, gene:5503717literature, APEX_matrix, targetP signal+, mito protein domain+, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosome;
Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly		all 14
COX10cytochrome ­c ­oxidase ­assembly ­factor ­heme ­A:farnesyltransferase ­COX10Q12887, gene:135217literature, yeast mito homolog, Rickettsial homolog, mito protein domain, induction, coexpression++MIMOXPHOS > Complex IV > CIV assembly factors;
Metabolism > Metals and cofactors > Heme synthesis and processing;
OXPHOS > OXPHOS assembly factors		none
AKAP1A-kinase ­anchoring ­protein ­1AKAP, AKAP121, AKAP149, AKAP84, D-AKAP1, PPP1R43, PRKA1, SAKAP84, TDRD17, Q92667, gene:816517literature, mito protein domain, induction, coexpression++, MS/MS+MOMSignaling > cAMP-PKA signalingcerebellum, skeletalmuscle, testis
MRM3mitochondrial ­rRNA ­methyltransferase ­3RMTL1, RNMTL1, Q9HC36, gene:5517817APEX_matrix, GFP, targetP signal+, mito protein domain+, induction, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications;
Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly		none
AK2adenylate ­kinase ­2ADK2, P54819, gene:20417literature, APEX_IMS, Rickettsial homolog, mito protein domain, coexpression+, MS/MS++IMSMetabolism > Nucleotide metabolism > Nucleotide synthesis and processingall 14
TMEM70transmembrane ­protein ­70MC5DN2, Q9BUB7, gene:5496817literature, APEX_matrix, mito protein domain+, coexpression++, MS/MSMIMOXPHOS > Complex I > CI assembly factors;
OXPHOS > Complex V > CV assembly factors;
OXPHOS > OXPHOS assembly factors		cerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
NOA1nitric ­oxide ­associated ­1C4orf14, MTG3, hAtNOS1, hNOA1, mAtNOS1, Q8NC60, gene:8427317literature, APEX_matrix, targetP signal+, yeast mito homolog++, mito protein domain, coexpressionMIMMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly		adipose, largeintestine
MCUR1mitochondrial ­calcium ­uniporter ­regulator ­1C6orf79, CCDC90A, FMP32, Q96AQ8, gene:6393317APEX_matrix, yeast mito homolog+, mito protein domain+, induction, MS/MSMIMnonecerebrum, cerebellum, brainstem, spinalcord, kidney, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta
TMEM14Ctransmembrane ­protein ­14CC6orf53, HSPC194, MSTP073, NET26, bA421M1.6, Q9P0S9, gene:5152217literature, mito protein domain+, induction, coexpression, MS/MS+MIMnoneall 14
NDUFC1NADH:ubiquinone ­oxidoreductase ­subunit ­C1KFYI, O43677, gene:471717literature, targetP signal+, mito protein domain+, coexpression++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		none
MRPL35mitochondrial ­ribosomal ­protein ­L35L35mt, MRP-L35, Q9NZE8, gene:5131817literature, APEX_matrix, targetP signal, mito protein domain, induction, coexpression++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomelargeintestine, testis
NDUFA3NADH:ubiquinone ­oxidoreductase ­subunit ­A3B9, CI-B9, O95167, gene:469617literature, induction, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		all 14
PDSS1decaprenyl ­diphosphate ­synthase ­subunit ­1COQ1, COQ10D2, COQ1A, DPS, SPS, TPRT, TPT, TPT 1, hDPS1, Q5T2R2, gene:2359017APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpressionMIMMetabolism > Metals and cofactors > Coenzyme Q metabolismskeletalmuscle, largeintestine, placenta, testis
SLC25A16solute ­carrier ­family ­25 ­member ­16D10S105E, GDA, GDC, HGT.1, ML7, hML7, P16260, gene:803417literature, yeast mito homolog+, mito protein domain+, coexpression, MS/MSMIMSmall molecule transport > SLC25A familycerebrum, brainstem, spinalcord, liver, adipose, largeintestine, stomach, placenta, testis
FMC1formation ­of ­mitochondrial ­complex ­V ­assembly ­factor ­1 ­homologC7orf55, HSPC268, 0, gene:15479117literature, APEX_matrix, targetP signal, induction, coexpression++, MS/MSMatrixOXPHOS > Complex V > CV assembly factors;
OXPHOS > OXPHOS assembly factors		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
ACSM3acyl-CoA ­synthetase ­medium ­chain ­family ­member ­3SA, SAH, Q53FZ2, gene:629617literature, targetP signal+, Rickettsial homolog, mito protein domain, MS/MS++MatrixMetabolism > Lipid metabolism > Fatty acid oxidationkidney, liver, adipose, smallintestine, largeintestine, stomach
NDUFAF6NADH:ubiquinone ­oxidoreductase ­complex ­assembly ­factor ­6C8orf38, FRTS5, MC1DN17, Q330K2, gene:13768216literature, targetP signal, mito protein domain, induction, coexpression++, MS/MSMIMOXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors		adipose, smallintestine, largeintestine, placenta, testis
UCP1uncoupling ­protein ­1SLC25A7, UCP, P25874, gene:735016literature, yeast mito homolog+, mito protein domain+, coexpression, MS/MSMIMSmall molecule transport > SLC25A familyadipose
SFXN1sideroflexin ­1SLC56A1, TCC, Q9H9B4, gene:9408116literature, mito protein domain+, coexpression, MS/MS++MIMMetabolism > Amino acid metabolism > Serine metabolism;
Metabolism > Vitamin metabolism > Folate and 1-C metabolism;
Small molecule transport > Sideroflexins		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
GLRX5glutaredoxin ­5C14orf87, FLB4739, GRX5, PR01238, PRO1238, PRSA, SIDBA3, SPAHGC, Q86SX6, gene:5121816literature, APEX_matrix, yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpression, MS/MS+MatrixMetabolism > Metals and cofactors > Fe-S cluster biosynthesis;
Metabolism > Metals and cofactors > Fe-S-containing proteins		all 14
PTRH1peptidyl-tRNA ­hydrolase ­1 ­homologC9orf115, PTH1, Q86Y79, gene:13842816APEX_matrix, GFP, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, MS/MSMatrixnonecerebrum, cerebellum, brainstem, largeintestine, placenta
MRPS11mitochondrial ­ribosomal ­protein ­S11HCC-2, MRP-S11, S11mt, P82912, gene:6496316literature, targetP signal, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
XPNPEP3X-prolyl ­aminopeptidase ­3APP3, ICP55, NPHPL1, Q9NQH7, gene:6392916literature, APEX_matrix, yeast mito homolog, Rickettsial homolog, mito protein domain, coexpression, MS/MS+MatrixProtein import, sorting and homeostasis > Protein import and sorting > Preprotein cleavage;
Protein import, sorting and homeostasis > Protein homeostasis > Proteases		cerebrum, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
PDP1pyruvate ­dehyrogenase ­phosphatase ­catalytic ­subunit ­1PDH, PDP, PDPC, PPM2A, PPM2C, Q9P0J1, gene:5470416literature, APEX_matrix, targetP signal, yeast mito homolog+, mito protein domain, MS/MS++MIMMetabolism > Carbohydrate metabolism > Pyruvate metabolismcerebrum, cerebellum, spinalcord, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
ECI1enoyl-CoA ­delta ­isomerase ­1DCI, P42126, gene:163216literature, APEX_matrix, targetP signal, mito protein domain, coexpression+, MS/MS++MatrixMetabolism > Lipid metabolism > Fatty acid oxidationall 14
SLC25A21solute ­carrier ­family ­25 ­member ­21MTDPS18, ODC, ODC1, Q9BQT8, gene:8987416literature, yeast mito homolog+, mito protein domain+, MS/MS++MIMMetabolism > Amino acid metabolism;
Small molecule transport > SLC25A family		spinalcord, kidney, liver, testis
SLC25A29solute ­carrier ­family ­25 ­member ­29C14orf69, CACL, ORNT3, Q8N8R3, gene:12309616literature, yeast mito homolog+, mito protein domain+, MS/MS++MIMMetabolism > Amino acid metabolism > Lysine metabolism;
Small molecule transport > SLC25A family		placenta, testis
SLC25A45solute ­carrier ­family ­25 ­member ­45Q8N413, gene:28313016yeast mito homolog+, mito protein domain+, MS/MS++MIMSmall molecule transport > SLC25A familyliver, adipose, smallintestine, stomach, placenta, testis
SDHAF3succinate ­dehydrogenase ­complex ­assembly ­factor ­3ACN9, DC11, LYRM10, Sdh7, Q9NRP4, gene:5700116targetP signal, yeast mito homolog++, coexpression+, MS/MSMatrixOXPHOS > Complex II > CII assembly factors;
OXPHOS > OXPHOS assembly factors		adipose, testis
PRELID3BPRELI ­domain ­containing ­3BC20orf45, SLMO2, dJ543J19.5, Q9Y3B1, gene:5101216GFP, yeast mito homolog+, mito protein domain+, induction, coexpressionIMSMetabolism > Lipid metabolism > Phospholipid metabolismnone
SLC25A39solute ­carrier ­family ­25 ­member ­39CGI-69, CGI69, Q9BZJ4, gene:5162916literature, yeast mito homolog+, mito protein domain+, induction, coexpressionMIMSmall molecule transport > SLC25A familynone
NDUFB3NADH:ubiquinone ­oxidoreductase ­subunit ­B3B12, CI-B12, MC1DN25, O43676, gene:470916literature, APEX_IMS, APEX_matrix, mito protein domain+, coexpression+, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		all 14
REXO2RNA ­exonuclease ­2CGI-114, REX2, RFN, SFN, Q9Y3B8, gene:2599616APEX_matrix, targetP signal, yeast mito homolog++, mito protein domain, MS/MS+MatrixMitochondrial central dogma > mtRNA metabolism > mtRNA stability and decayall 14
GATMglycine ­amidinotransferaseAGAT, AT, CCDS3, FRTS1, P50440, gene:262816literature, targetP signal, mito protein domain+, MS/MS++MatrixMetabolism > Nucleotide metabolism > Creatine metabolismspinalcord, kidney, liver, stomach, placenta, testis
TIMM10Btranslocase ­of ­inner ­mitochondrial ­membrane ­10BFXC1, TIM10B, Tim9b, Q9Y5J6, gene:2651516literature, APEX_IMS, yeast mito homolog+, mito protein domain+, MS/MS+MIMProtein import, sorting and homeostasis > Protein homeostasis > Chaperones;
Protein import, sorting and homeostasis > Protein import and sorting		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, largeintestine, stomach, placenta, testis
SARS2seryl-tRNA ­synthetase ­2, ­mitochondrialSARS, SARSM, SERS, SYS, SerRS, SerRSmt, mtSerRS, Q9NP81, gene:5493816literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, MS/MSMatrixMitochondrial central dogma > Translation > mt-tRNA synthetasesskeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
CHDHcholine ­dehydrogenaseQ8NE62, gene:5534916APEX_matrix, targetP signal+, mito protein domain, coexpression, MS/MS++MIMMetabolism > Amino acid metabolism > Glycine metabolism;
Metabolism > Vitamin metabolism > Choline and betaine metabolism;
Metabolism > Vitamin metabolism > Folate and 1-C metabolism;
Metabolism > Electron carriers > Q-linked reactions, other		spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
MRPL57mitochondrial ­ribosomal ­protein ­L57MRP63, bMRP63, Q9BQC6, gene:7898816literature, APEX_matrix, targetP signal, mito protein domain+, coexpression, MS/MS+MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, placenta, testis
C8orf82chromosome ­8 ­open ­reading ­frame ­82Q6P1X6, gene:41491916APEX_matrix, targetP signal, coexpression+, MS/MS++Matrixnoneall 14
EARS2glutamyl-tRNA ­synthetase ­2, ­mitochondrialCOXPD12, MSE1, gluRS, mtGlnRS, Q5JPH6, gene:12445416literature, APEX_matrix, GFP, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpressionMatrixMitochondrial central dogma > Translation > mt-tRNA synthetasesadipose, largeintestine, placenta
GRHPRglyoxylate ­and ­hydroxypyruvate ­reductaseGLXR, GLYD, PH2, Q9UBQ7, gene:938016APEX_matrix, targetP signal, yeast mito homolog, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Amino acid metabolism > Glyoxylate metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
MTFMTmitochondrial ­methionyl-tRNA ­formyltransferaseCOXPD15, FMT1, MC1DN27, Q96DP5, gene:12326316literature, APEX_matrix, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpressionMatrixMitochondrial central dogma > Translation > fMet processing;
Metabolism > Vitamin metabolism > Folate and 1-C metabolism		none
PTCD2pentatricopeptide ­repeat ­domain ­2Q8WV60, gene:7981016literature, mito protein domain+, induction, coexpression, MS/MSMatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > Polycistronic mtRNA processing		smallintestine, largeintestine, placenta
ECHDC1ethylmalonyl-CoA ­decarboxylase ­1HEL-S-76, MMCD, dJ351K20.2, Q9NTX5, gene:5586216yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpression, MS/MS+unknownMetabolism > Lipid metabolismbrainstem, spinalcord, liver, adipose, largeintestine, placenta, testis
HARS2histidyl-tRNA ­synthetase ­2, ­mitochondrialHARSL, HARSR, HO3, HisRS, PRLTS2, P49590, gene:2343816literature, APEX_matrix, GFP, targetP signal, Rickettsial homolog, mito protein domain, coexpression, MS/MS+MatrixMitochondrial central dogma > Translation > mt-tRNA synthetasescerebellum, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
NDUFA4NDUFA4 ­mitochondrial ­complex ­associatedCI-9k, CI-MLRQ, COXFA4, MLRQ, O00483, gene:469716literature, mito protein domain, coexpression++, MS/MS++MIMOXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits		all 14
NDUFA13NADH:ubiquinone ­oxidoreductase ­subunit ­A13B16.6, CDA016, CGI-39, GRIM-19, GRIM19, MC1DN28, Q9P0J0, gene:5107916literature, APEX_IMS, mito protein domain, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		all 14
NAXENAD(P)HX ­epimeraseAIBP, APOA1BP, PEBEL, YJEFN1, Q8NCW5, gene:12824016APEX_matrix, targetP signal, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Metals and cofactors > NAD biosynthesis and metabolismall 14
TMEM11transmembrane ­protein ­11C17orf35, PM1, PMI, P17152, gene:883416GFP, targetP signal, mito protein domain+, induction, coexpression, MS/MSMIMMitochondrial dynamics and surveillanceall 14
SELENOOselenoprotein ­OSELO, Q9BVL4, gene:8364215targetP signal+, yeast mito homolog, mito protein domain+, coexpressionMatrixMetabolism > Detoxification > Selenoproteinsspinalcord, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
NAGSN-acetylglutamate ­synthaseAGAS, ARGA, Q8N159, gene:16241715literature, targetP signal, yeast mito homolog++, mito protein domain+, MS/MSMatrixMetabolism > Amino acid metabolism > Urea cycleliver
HSD17B4hydroxysteroid ­17-beta ­dehydrogenase ­4DBP, MFE-2, MFP-2, MPF-2, PRLTS1, SDR8C1, P51659, gene:329515Rickettsial homolog, mito protein domain+, induction, coexpressionunknownMetabolism > Lipid metabolismkidney, liver, adipose, largeintestine, stomach, testis
NDUFAF7NADH:ubiquinone ­oxidoreductase ­complex ­assembly ­factor ­7C2orf56, MidA, PRO1853, Q7L592, gene:5547115APEX_matrix, GFP, targetP signal, Rickettsial homolog, mito protein domain+, coexpression, MS/MSMIMOXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors		cerebrum, brainstem, spinalcord, kidney, adipose, largeintestine, stomach, placenta, testis
OATornithine ­aminotransferaseGACR, HOGA, OATASE, OKT, P04181, gene:494215literature, APEX_matrix, targetP signal+, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Amino acid metabolism > Proline metabolismall 14
MRPL50mitochondrial ­ribosomal ­protein ­L50MRP-L50, Q8N5N7, gene:5453415literature, APEX_matrix, targetP signal, mito protein domain+, coexpression+, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
COX7Bcytochrome ­c ­oxidase ­subunit ­7BAPLCC, LSDMCA2, P24311, gene:134915literature, targetP signal, mito protein domain+, induction, MS/MS+MIMOXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta
RARS2arginyl-tRNA ­synthetase ­2, ­mitochondrialArgRS, DALRD2, PCH6, PRO1992, RARSL, Q5T160, gene:5703815literature, APEX_matrix, GFP, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMitochondrial central dogma > Translation > mt-tRNA synthetasesall 14
OXLD1oxidoreductase ­like ­domain ­containing ­1C17orf90, Q5BKU9, gene:33922915targetP signal, yeast mito homolog, mito protein domain+, coexpression+unknownnoneadipose, largeintestine, stomach
LACTB2lactamase ­beta ­2CGI-83, Q53H82, gene:5111015targetP signal, yeast mito homolog+, mito protein domain, induction, MS/MS+MatrixMitochondrial central dogma > mtRNA metabolism > mtRNA stability and decaykidney, liver, largeintestine, stomach
NDUFA11NADH:ubiquinone ­oxidoreductase ­subunit ­A11B14.7, CI-B14.7, MC1DN14, Q86Y39, gene:12632815literature, APEX_IMS, mito protein domain+, induction, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		all 14
AURKAIP1aurora ­kinase ­A ­interacting ­protein ­1AIP, AKIP, MRP-S38, Q9NWT8, gene:5499815GFP, targetP signal, yeast mito homolog++, mito protein domain, coexpression+MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomenone
CHCHD7coiled-coil-helix-coiled-coil-helix ­domain ­containing ­7COX23, Q9BUK0, gene:7914515yeast mito homolog, induction, coexpression, MS/MS+IMSMetabolism > Metals and cofactors > Copper metabolismcerebrum, smallintestine, placenta, testis
SHMT2serine ­hydroxymethyltransferase ­2GLYA, HEL-S-51e, SHMT, P34897, gene:647215literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Amino acid metabolism > Serine metabolism;
Metabolism > Amino acid metabolism > Glycine metabolism;
Metabolism > Vitamin metabolism > Folate and 1-C metabolism		cerebrum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
MTHFD2methylenetetrahydrofolate ­dehydrogenase ­(NADP+ ­dependent) ­2, ­methenyltetrahydrofolate ­cyclohydrolaseNMDMC, P13995, gene:1079715literature, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, MS/MSMatrixMetabolism > Vitamin metabolism > Folate and 1-C metabolismspinalcord, placenta, testis
RMDN1regulator ­of ­microtubule ­dynamics ­1CGI-90, FAM82B, RMD-1, RMD1, Q96DB5, gene:5111515APEX_IMS, targetP signal+, induction, coexpression, MS/MSMOMnoneall 14
CPS1carbamoyl-phosphate ­synthase ­1CPSASE1, PHN, P31327, gene:137315literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, MS/MS++MatrixMetabolism > Amino acid metabolism > Urea cyclecerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta
MRPL14mitochondrial ­ribosomal ­protein ­L14L14mt, L32mt, MRP-L14, MRP-L32, MRPL32, RMPL32, RPML32, Q6P1L8, gene:6492815literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, coexpression+, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
MTRES1mitochondrial ­transcription ­rescue ­factor ­1C6orf203, HSPC230, PRED31, Q9P0P8, gene:5125015GFP, induction, coexpression++, MS/MS+MatrixMitochondrial central dogma > mtRNA metabolism > Transcription;
Mitochondrial central dogma > Translation		all 14
RDH13retinol ­dehydrogenase ­13SDR7C3, Q8NBN7, gene:11272415yeast mito homolog+, Rickettsial homolog, mito protein domain, induction, MS/MSMIMMetabolism > Vitamin metabolism > Vitamin A metabolismcerebrum, cerebellum, brainstem, spinalcord, adipose, largeintestine, stomach, placenta, testis
TRMT2BtRNA ­methyltransferase ­2 ­homolog ­BCXorf34, dJ341D10.3, Q96GJ1, gene:7997915GFP, targetP signal, Rickettsial homolog, mito protein domain+, induction, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications;
Mitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications		none
MRPS24mitochondrial ­ribosomal ­protein ­S24HSPC335, MRP-S24, S24mt, bMRP-47, bMRP47, Q96EL2, gene:6495115literature, APEX_matrix, induction, coexpression++, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
COA3cytochrome ­c ­oxidase ­assembly ­factor ­3CCDC56, COX25, HSPC009, MITRAC12, Q9Y2R0, gene:2895815APEX_IMS, APEX_matrix, mito protein domain+, coexpression, MS/MS++MIMMitochondrial central dogma > Translation > Translation factors;
OXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors		all 14
GSRglutathione-disulfide ­reductaseGR, GSRD, HEL-75, HEL-S-122m, P00390, gene:293615literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, MS/MS++MatrixMetabolism > Detoxification > ROS and glutathione metabolismspinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
ENDOGendonuclease ­GQ14249, gene:202115literature, APEX_IMS, mito protein domain, coexpression++, MS/MSIMSMitochondrial central dogma > mtDNA maintenance > mtDNA stability and decay;
Mitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay;
Mitochondrial dynamics and surveillance > Apoptosis		all 14
BCAT2branched ­chain ­amino ­acid ­transaminase ­2BCAM, BCATM, BCT2, HVLI, PP18, O15382, gene:58715literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Amino acid metabolism > BCAA metabolismcerebrum, cerebellum, brainstem, kidney, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
TEFMtranscription ­elongation ­factor, ­mitochondrialC17orf42, Q96QE5, gene:7973615APEX_matrix, targetP signal, induction, coexpression++, MS/MSMatrixMitochondrial central dogma > mtRNA metabolism > Transcriptionliver, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
ACSF2acyl-CoA ­synthetase ­family ­member ­2ACSMW, AVYV493, Q96CM8, gene:8022115APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, MS/MS++MatrixMetabolism > Lipid metabolism > Fatty acid oxidationcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
TRMUtRNA ­mitochondrial ­2-thiouridylaseLCAL3, MTO2, MTU1, TRMT, TRMT1, O75648, gene:5568715literature, APEX_matrix, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mt-tRNA modificationsadipose, largeintestine, placenta
MRPS22mitochondrial ­ribosomal ­protein ­S22C3orf5, COXPD5, GIBT, GK002, MRP-S22, ODG7, RPMS22, P82650, gene:5694515literature, APEX_matrix, mito protein domain+, induction, coexpression, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
LAP3leucine ­aminopeptidase ­3HEL-S-106, LAP, LAPEP, PEPS, P28838, gene:5105615APEX_matrix, targetP signal+, Rickettsial homolog, coexpression, MS/MS++MatrixProtein import, sorting and homeostasis > Protein homeostasis > Proteasesall 14
GLUD1glutamate ­dehydrogenase ­1GDH, GDH1, GLUD, P00367, gene:274615literature, APEX_matrix, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Amino acid metabolism > Glutamate metabolism;
Metabolism > Amino acid metabolism > GABA metabolism		all 14
HMGCL3-hydroxy-3-methylglutaryl-CoA ­lyaseHL, P35914, gene:315515literature, APEX_matrix, targetP signal+, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Carbohydrate metabolism > Ketone metabolism;
Metabolism > Amino acid metabolism > BCAA metabolism		all 14
FHITfragile ­histidine ­triad ­diadenosine ­triphosphataseAP3Aase, FRA3B, P49789, gene:227215yeast mito homolog, Rickettsial homolog, mito protein domain, MS/MS++MatrixMetabolism > Nucleotide metabolism > Nucleotide synthesis and processingcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
MRPS33mitochondrial ­ribosomal ­protein ­S33CGI-139, MRP-S33, PTD003, S33mt, Q9Y291, gene:5165015literature, targetP signal, mito protein domain+, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
NUBPLnucleotide ­binding ­protein ­likeC14orf127, IND1, MC1DN21, huInd1, Q8TB37, gene:8022414literature, targetP signal, Rickettsial homolog, mito protein domain, coexpression+, MS/MS+MatrixOXPHOS > Complex I > CI assembly factors;
Metabolism > Metals and cofactors > Fe-S cluster biosynthesis;
Metabolism > Metals and cofactors > Fe-S-containing proteins;
OXPHOS > OXPHOS assembly factors		spinalcord, adipose, largeintestine, stomach, placenta, testis
LYRM2LYR ­motif ­containing ­2DJ122O8.2, Q9NU23, gene:5722614APEX_matrix, GFP, targetP signal+, mito protein domain+, coexpression, MS/MSMatrixOXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors		heart, adipose, largeintestine, placenta
MPV17LMPV17 ­mitochondrial ­inner ­membrane ­protein ­likeM-LPH, MLPH1, MLPH2, MPV17L1, Q2QL34, gene:25502714targetP signal, yeast mito homolog+, mito protein domain, MS/MS++MIMSmall molecule transportcerebrum, cerebellum, brainstem, spinalcord, kidney, liver
GADD45GIP1GADD45G ­interacting ­protein ­1CKBBP2, CKbetaBP2, CRIF1, MRP-L59, PLINP, PLINP-1, PRG6, Plinp1, Q8TAE8, gene:9048014APEX_matrix, GFP, targetP signal, coexpression+, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
TFAMtranscription ­factor ­A, ­mitochondrialMTDPS15, MTTF1, MTTFA, TCF6, TCF6L1, TCF6L2, TCF6L3, Q00059, gene:701914literature, APEX_matrix, targetP signal, mito protein domain, induction, MS/MS++MatrixMitochondrial central dogma > mtDNA maintenance > mtDNA replication;
Mitochondrial central dogma > mtDNA maintenance > mtDNA nucleoid;
Mitochondrial central dogma > mtRNA metabolism > Transcription		all 14
ALDH18A1aldehyde ­dehydrogenase ­18 ­family ­member ­A1ADCL3, ARCL3A, GSAS, P5CS, PYCS, SPG9, SPG9A, SPG9B, P54886, gene:583214literature, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Amino acid metabolism > Proline metabolismcerebrum, cerebellum, brainstem, spinalcord, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
OXSM3-oxoacyl-ACP ­synthase, ­mitochondrialCEM1, FASN2D, KASI, KS, Q9NWU1, gene:5499514literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, MS/MS++MatrixMetabolism > Lipid metabolism > Type II fatty acid synthesisliver, heart, adipose, smallintestine, largeintestine, placenta, testis
KYAT3kynurenine ­aminotransferase ­3CCBL2, KAT3, KATIII, Q6YP21, gene:5626714APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, MS/MS++MatrixMetabolism > Amino acid metabolism > Kynurenine metabolism;
Metabolism > Metals and cofactors > NAD biosynthesis and metabolism		spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
TARS2threonyl-tRNA ­synthetase ­2, ­mitochondrialCOXPD21, TARSL1, thrRS, Q9BW92, gene:8022214APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, MS/MS++MatrixMitochondrial central dogma > Translation > mt-tRNA synthetasescerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
TBRG4transforming ­growth ­factor ­beta ­regulator ­4CPR2, FASTKD4, Q969Z0, gene:923814literature, APEX_matrix, targetP signal+, coexpression, MS/MS++MatrixMitochondrial central dogma > mtRNA metabolism > Polycistronic mtRNA processing;
Mitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay		all 14
TMEM65transmembrane ­protein ­65Q6PI78, gene:15737814literature, APEX_matrix, targetP signal+, coexpression, MS/MS++MIMnonecerebrum, cerebellum, brainstem, spinalcord, kidney, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
ROMO1reactive ­oxygen ­species ­modulator ­1C20orf52, MTGM, MTGMP, bA353C18.2, P60602, gene:14082314literature, APEX_IMS, yeast mito homolog++, mito protein domain+, MS/MSMIMProtein import, sorting and homeostasis > Protein import and sorting > TIM23 presequence pathway;
Small molecule transport		cerebrum, cerebellum, brainstem, spinalcord, liver, skeletalmuscle, adipose, smallintestine, stomach, placenta, testis
PRDX5peroxiredoxin ­5ACR1, AOEB166, B166, HEL-S-55, PLP, PMP20, PRDX6, PRXV, SBBI10, prx-V, P30044, gene:2582414literature, APEX_matrix, targetP signal, mito protein domain, induction, MS/MS++MatrixMetabolism > Detoxification > ROS and glutathione metabolismall 14
DNAJC15DnaJ ­heat ­shock ­protein ­family ­(Hsp40) ­member ­C15DNAJD1, HSD18, MCJ, Q9Y5T4, gene:2910314GFP, mito protein domain, induction, coexpression, MS/MS+MIMProtein import, sorting and homeostasis > Protein import and sorting > Import motorcerebrum, spinalcord, liver, adipose, smallintestine, largeintestine, placenta, testis
MAIP1matrix ­AAA ­peptidase ­interacting ­protein ­1C2orf47, Q8WWC4, gene:7956814GFP, targetP signal, induction, coexpression, MS/MS+MatrixProtein import, sorting and homeostasis > Protein homeostasis > Chaperonesall 14
HIGD1AHIG1 ­hypoxia ­inducible ­domain ­family ­member ­1AHIG1, RCF1a, Q9Y241, gene:2599414literature, APEX_IMS, mito protein domain+, induction, MS/MS++MIMOXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors;
OXPHOS > Respirasome assembly		all 14
C16orf91chromosome ­16 ­open ­reading ­frame ­91CCSMST1, URLC5, gs103, Q4G0I0, gene:28395114APEX_IMS, targetP signal+, induction, coexpression, MS/MSIMSnonebrainstem, spinalcord, adipose, largeintestine, placenta, testis
RHOT2ras ­homolog ­family ­member ­T2ARHT2, C16orf39, MIRO-2, MIRO2, RASL, Q8IXI1, gene:8994114literature, mito protein domain+, induction, coexpressionMOMSignaling > Calcium homeostasis > EF hand proteins;
Mitochondrial dynamics and surveillance > Trafficking		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
SLC25A44solute ­carrier ­family ­25 ­member ­44Q96H78, gene:967314yeast mito homolog+, mito protein domain+, induction, MS/MSMIMMetabolism > Amino acid metabolism > BCAA metabolism;
Small molecule transport > SLC25A family		placenta
HAO2hydroxyacid ­oxidase ­2GIG16, HAOX2, Q9NYQ3, gene:5117914yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpression, MS/MSunknownMetabolism > Lipid metabolismkidney, largeintestine, testis
OXA1LOXA1L ­mitochondrial ­inner ­membrane ­proteinOXA1, Q15070, gene:501814literature, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpressionMIMProtein import, sorting and homeostasis > Protein import and sorting > OXA;
Mitochondrial central dogma > Translation		cerebrum, brainstem, spinalcord, kidney, heart, adipose, smallintestine, largeintestine, placenta, testis
SLC25A46solute ­carrier ­family ­25 ­member ­46HMSN6B, Q96AG3, gene:9113714yeast mito homolog++, mito protein domain+, inductionMOMSmall molecule transport > SLC25A family;
Mitochondrial dynamics and surveillance > Fission;
Mitochondrial dynamics and surveillance > Intramitochondrial membrane interactions		cerebrum, spinalcord, adipose, placenta
RDH14retinol ­dehydrogenase ­14PAN2, SDR7C4, Q9HBH5, gene:5766514yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpression, MS/MSMembraneMetabolism > Vitamin metabolism > Vitamin A metabolismcerebrum, cerebellum, brainstem, spinalcord, smallintestine, largeintestine, stomach, placenta, testis
ATP5MPLATP ­synthase ­membrane ­subunit ­6.8PL6.8PL, C14orf2, MLQ, MP68, PLPM, P56378, gene:955614induction, coexpression++, MS/MS+MIMOXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta
CA5Bcarbonic ­anhydrase ­5BCA-VB, CAVB, Q9Y2D0, gene:1123814literature, targetP signal, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Amino acid metabolism > Urea cyclecerebellum, spinalcord, kidney, adipose, stomach, placenta, testis
COASYCoenzyme ­A ­synthaseDPCK, NBIA6, NBP, PCH12, PPAT, UKR1, pOV-2, Q13057, gene:8034714APEX_matrix, targetP signal, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Metals and cofactors > Coenzyme A metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
CISD3CDGSH ­iron ­sulfur ­domain ­3MiNT, Miner2, P0C7P0, gene:28410614APEX_matrix, targetP signal, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Metals and cofactors > Fe-S cluster biosynthesis;
Metabolism > Metals and cofactors > Fe-S-containing proteins		all 14
GSTZ1glutathione ­S-transferase ­zeta ­1GSTZ1-1, MAAI, MAAID, MAI, O43708, gene:295414APEX_matrix, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Detoxification > Xenobiotic metabolismall 14
COX6B2cytochrome ­c ­oxidase ­subunit ­6B2COXVIB2, CT59, Q6YFQ2, gene:12596514literature, yeast mito homolog+, mito protein domain+, MS/MS+MIMOXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits		testis
GLOD4glyoxalase ­domain ­containing ­4C17orf25, CGI-150, HC71, Q9HC38, gene:5103114APEX_matrix, targetP signal+, coexpression, MS/MS++Matrixnonecerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
ACAA1acetyl-CoA ­acyltransferase ­1ACAA, PTHIO, THIO, P09110, gene:3014yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpressionunknownMetabolism > Lipid metabolismkidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
CROTcarnitine ­O-octanoyltransferaseCOT, Q9UKG9, gene:5467714yeast mito homolog+, mito protein domain, induction, coexpression, MS/MSunknownMetabolism > Lipid metabolism > Fatty acid oxidationkidney, liver
PDFpeptide ­deformylase, ­mitochondrialQ9HBH1, gene:6414614APEX_matrix, Rickettsial homolog, mito protein domain+, coexpression, MS/MSMatrixMitochondrial central dogma > Translation > fMet processing;
Protein import, sorting and homeostasis > Protein homeostasis		cerebrum, heart, skeletalmuscle, adipose, largeintestine, placenta, testis
COX19cytochrome ­c ­oxidase ­assembly ­factor ­COX19Q49B96, gene:9063914yeast mito homolog, mito protein domain, induction, coexpression+, MS/MSIMSOXPHOS > Complex IV > CIV assembly factors;
Metabolism > Metals and cofactors > Copper metabolism;
OXPHOS > OXPHOS assembly factors		liver, placenta
AMTaminomethyltransferaseGCE, GCST, GCVT, NKH, P48728, gene:27514targetP signal+, mito protein domain, MS/MS++MatrixMetabolism > Amino acid metabolism > Glycine metabolism;
Metabolism > Amino acid metabolism > Glycine cleavage system;
Metabolism > Vitamin metabolism > Folate and 1-C metabolism		cerebrum, brainstem, spinalcord, kidney, liver, largeintestine, stomach, placenta
PDPRpyruvate ­dehydrogenase ­phosphatase ­regulatory ­subunitPDP3, Q8NCN5, gene:5506614APEX_matrix, targetP signal+, mito protein domain, MS/MS++MatrixMetabolism > Carbohydrate metabolism > Pyruvate metabolismcerebrum, cerebellum, brainstem, spinalcord, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
NLNneurolysinAGTBP, EP24.16, MEP, MOP, Q9BYT8, gene:5748614literature, APEX_matrix, targetP signal+, mito protein domain, MS/MS++MatrixProtein import, sorting and homeostasis > Protein homeostasis > Proteasescerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
BOLA1bolA ­family ­member ­1CGI-143, Q9Y3E2, gene:5102713GFP, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Metals and cofactors > Fe-S cluster biosynthesis;
Metabolism > Metals and cofactors > Fe-S-containing proteins		cerebrum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
ALAS25'-aminolevulinate ­synthase ­2ALAS-E, ALASE, ANH1, ASB, SIDBA1, XLDPP, XLEPP, XLSA, P22557, gene:21213literature, yeast mito homolog+, Rickettsial homolog, mito protein domain+, coexpressionMatrixMetabolism > Metals and cofactors > Heme synthesis and processingnone
GRPEL2GrpE ­like ­2, ­mitochondrialMt-GrpE#2, Q8TAA5, gene:13426613literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain+MatrixProtein import, sorting and homeostasis > Protein import and sorting > Import motor;
Metabolism > Metals and cofactors > Fe-S cluster biosynthesis		placenta
ALDH7A1aldehyde ­dehydrogenase ­7 ­family ­member ­A1ATQ1, EPD, PDE, P49419, gene:50113literature, APEX_matrix, targetP signal, yeast mito homolog, mito protein domain, MS/MS++MatrixMetabolism > Amino acid metabolism > Lysine metabolism;
Metabolism > Amino acid metabolism > Glycine metabolism;
Metabolism > Vitamin metabolism > Choline and betaine metabolism;
Metabolism > Vitamin metabolism > Folate and 1-C metabolism;
Metabolism > Detoxification > ROS and glutathione metabolism		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
HDHD5haloacid ­dehalogenase ­like ­hydrolase ­domain ­containing ­5CECR5, Q9BXW7, gene:2744013APEX_matrix, targetP signal, yeast mito homolog, mito protein domain, MS/MS++Matrixnonecerebellum, kidney, liver, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
GFERgrowth ­factor, ­augmenter ­of ­liver ­regenerationALR, ERV1, HERV1, HPO, HPO1, HPO2, HSS, MMCHD, MPMCD, P55789, gene:267113literature, GFP, yeast mito homolog++, mito protein domain, coexpression, MS/MSIMSProtein import, sorting and homeostasis > Protein import and sorting > MIA40cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
IMMP2Linner ­mitochondrial ­membrane ­peptidase ­subunit ­2IMMP2L-IT1, IMP2, IMP2-LIKE, Q96T52, gene:8394313literature, yeast mito homolog++, mito protein domain, coexpression, MS/MSMIMProtein import, sorting and homeostasis > Protein import and sorting > Preprotein cleavage;
Protein import, sorting and homeostasis > Protein homeostasis > Proteases		cerebellum, brainstem, liver, skeletalmuscle, adipose, largeintestine, testis
COX4I2cytochrome ­c ­oxidase ­subunit ­4I2COX4, COX4-2, COX4B, COX4L2, COXIV-2, dJ857M17.2, Q96KJ9, gene:8470113literature, targetP signal, yeast mito homolog++, mito protein domain+MIMOXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits		cerebrum, spinalcord, heart, adipose, largeintestine, stomach, placenta
MRPL39mitochondrial ­ribosomal ­protein ­L39C21orf92, L39mt, L5mt, MRP-L5, MRPL5, MSTP003, PRED22, PRED66, RPML5, Q9NYK5, gene:5414813literature, APEX_matrix, targetP signal, Rickettsial homolog, induction, coexpression, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, brainstem, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
AADATaminoadipate ­aminotransferaseKAT2, KATII, KYAT2, Q8N5Z0, gene:5116613targetP signal, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Amino acid metabolism > Lysine metabolismkidney, liver
FUNDC2FUN14 ­domain ­containing ­2DC44, HCBP6, HCC3, PD03104, Q9BWH2, gene:6599113mito protein domain+, MS/MS++MOMMitochondrial dynamics and surveillance > Organelle contact sites;
Signaling		cerebrum, cerebellum, brainstem, spinalcord, kidney, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
LYPLA1lysophospholipase ­1APT-1, APT1, LPL-I, LPL1, hAPT1, O75608, gene:1043413Rickettsial homolog, mito protein domain, coexpression, MS/MS++unknownMetabolism > Lipid metabolism;
Signaling		all 14
PDHA2pyruvate ­dehydrogenase ­E1 ­subunit ­alpha ­2PDHAL, P29803, gene:516113targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain+MatrixMetabolism > Carbohydrate metabolism > Pyruvate metabolismnone
MTRF1Lmitochondrial ­translational ­release ­factor ­1 ­likeHMRF1L, MRF1L, mtRF1a, Q9UGC7, gene:5451613literature, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domainMatrixMitochondrial central dogma > Translation > Translation factorstestis
GTPBP3GTP ­binding ­protein ­3, ­mitochondrialCOXPD23, GTPBG3, MSS1, MTGP1, THDF1, Q969Y2, gene:8470513literature, APEX_matrix, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domainMatrixMitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications;
Metabolism > Vitamin metabolism > Folate and 1-C metabolism		largeintestine
MTHFD2Lmethylenetetrahydrofolate ­dehydrogenase ­(NADP+ ­dependent) ­2 ­likeQ9H903, gene:44102413literature, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domainMatrixMetabolism > Vitamin metabolism > Folate and 1-C metabolismspinalcord, placenta
YME1L1YME1 ­like ­1 ­ATPaseFTSH, MEG4, OPA11, PAMP, YME1L, Q96TA2, gene:1073013literature, APEX_IMS, Rickettsial homolog, mito protein domain+, MS/MS+MIMProtein import, sorting and homeostasis > Protein homeostasis > Proteasescerebrum, cerebellum, brainstem, spinalcord, kidney, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
ACOT2acyl-CoA ­thioesterase ­2CTE-IA, CTE1A, MTE1, PTE2, PTE2A, ZAP128, P49753, gene:1096513literature, mito protein domain, induction, coexpression, MS/MS++MatrixMetabolism > Lipid metabolismall 14
COA8cytochrome ­c ­oxidase ­assembly ­factor ­8APOP, APOP1, APOPT1, C14orf153, Q96IL0, gene:8433413literature, targetP signal, mito protein domain+, coexpression+MIMOXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors		largeintestine, placenta
MMABmetabolism ­of ­cobalamin ­associated ­BATR, CFAP23, cblB, cob, Q96EY8, gene:32662513APEX_matrix, GFP, targetP signal, coexpression, MS/MS++MatrixMetabolism > Vitamin metabolism > Vitamin B12 metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
PPOXprotoporphyrinogen ­oxidasePPO, V290M, VP, P50336, gene:549813literature, APEX_matrix, yeast mito homolog++, mito protein domain, coexpression, MS/MSMIMMetabolism > Metals and cofactors > Heme synthesis and processingcerebrum, spinalcord, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
ABCD3ATP ­binding ­cassette ­subfamily ­D ­member ­3ABC43, CBAS5, PMP70, PXMP1, ZWS2, P28288, gene:582513GFP, targetP signal, Rickettsial homolog, mito protein domain, induction, coexpressionMembraneSmall molecule transport > ABC transporterskidney, liver, adipose, placenta, testis
SIRT3sirtuin ­3SIR2L3, Q9NTG7, gene:2341013literature, APEX_matrix, mito protein domain, induction, coexpression, MS/MS+MatrixMetabolism > Metals and cofactors > NAD biosynthesis and metabolism;
Signaling		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
LDHDlactate ­dehydrogenase ­DDLACD, DLD, Q86WU2, gene:19725713literature, yeast mito homolog+, mito protein domain, MS/MS++MIMMetabolism > Carbohydrate metabolismkidney, liver, heart, adipose, stomach, placenta, testis
MRPL45mitochondrial ­ribosomal ­protein ­L45L45mt, MRP-L45, B4DEF8, gene:8431113literature, APEX_matrix, mito protein domain, induction, coexpression, MS/MS+MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, brainstem, spinalcord, adipose, smallintestine, largeintestine, stomach, placenta, testis
C12orf65chromosome ­12 ­open ­reading ­frame ­65COXPD7, SPG55, Q9H3J6, gene:9157413literature, yeast mito homolog++, Rickettsial homolog, mito protein domain, MS/MSMatrixMitochondrial central dogma > Translation > Translation factorscerebrum, brainstem, spinalcord, adipose, largeintestine, stomach, placenta
IMMP1Linner ­mitochondrial ­membrane ­peptidase ­subunit ­1IMMP1, IMP1, IMP1-LIKE, Q96LU5, gene:19629413literature, yeast mito homolog++, Rickettsial homolog, mito protein domain, MS/MSMIMProtein import, sorting and homeostasis > Protein import and sorting > Preprotein cleavage;
Protein import, sorting and homeostasis > Protein homeostasis > Proteases		testis
POLGDNA ­polymerase ­gamma, ­catalytic ­subunitMDP1, MIRAS, MTDPS4A, MTDPS4B, PEO, POLG1, POLGA, SANDO, SCAE, P54098, gene:542813literature, APEX_matrix, targetP signal, yeast mito homolog++, mito protein domain, MS/MSMatrixMitochondrial central dogma > mtDNA maintenance > mtDNA replication;
Mitochondrial central dogma > mtDNA maintenance > mtDNA nucleoid;
Mitochondrial central dogma > mtDNA maintenance > mtDNA repair		cerebrum, cerebellum, spinalcord, liver, adipose, largeintestine, placenta, testis
ATPAF1ATP ­synthase ­mitochondrial ­F1 ­complex ­assembly ­factor ­1ATP11, ATP11p, Q5TC12, gene:6475613literature, APEX_matrix, yeast mito homolog++, mito protein domain+, MS/MSMatrixOXPHOS > Complex V > CV assembly factors;
OXPHOS > OXPHOS assembly factors		cerebrum, brainstem, spinalcord, adipose, smallintestine, largeintestine, placenta, testis
BLOC1S1biogenesis ­of ­lysosomal ­organelles ­complex ­1 ­subunit ­1BLOS1, BORCS1, GCN5L1, MICoA, RT14, P78537, gene:264713mito protein domain, coexpression++, MS/MSunknownSignalingplacenta, testis
ALDH1L2aldehyde ­dehydrogenase ­1 ­family ­member ­L2mtFDH, Q3SY69, gene:16042813literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, coexpression, MS/MSMatrixMetabolism > Vitamin metabolism > Folate and 1-C metabolismbrainstem, spinalcord, liver, skeletalmuscle, smallintestine, largeintestine, stomach, placenta
COX18cytochrome ­c ­oxidase ­assembly ­factor ­COX18COX18HS, Q8N8Q8, gene:28552113literature, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpressionMIMOXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors		none
CPOXcoproporphyrinogen ­oxidaseCOX, CPO, CPX, HARPO, HCP, P36551, gene:137113literature, APEX_IMS, APEX_matrix, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MIMMetabolism > Metals and cofactors > Heme synthesis and processingcerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
NNTnicotinamide ­nucleotide ­transhydrogenaseGCCD4, Q13423, gene:2353013literature, APEX_matrix, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS+MIMMetabolism > Metals and cofactors > NAD biosynthesis and metabolismcerebrum, spinalcord, skeletalmuscle, adipose, placenta
NDUFAF8NADH:ubiquinone ­oxidoreductase ­complex ­assembly ­factor ­8C17orf89, MC1DN34, A1L188, gene:28418413targetP signal, induction, coexpression+, MS/MSIMSOXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors		adipose, stomach
ATP23ATP23 ­metallopeptidase ­and ­ATP ­synthase ­assembly ­factor ­homologKUB3, XRCC6BP1, Q9Y6H3, gene:9141913yeast mito homolog++, mito protein domain+, coexpressionIMSProtein import, sorting and homeostasis > Protein homeostasis > Proteasescerebellum
DCAKDdephospho-CoA ­kinase ­domain ­containingQ8WVC6, gene:7987713GFP, yeast mito homolog, Rickettsial homolog, mito protein domain, coexpression, MS/MSMembranenoneall 14
ATP5MFATP ­synthase ­membrane ­subunit ­fATP5J2, ATP5JL, P56134, gene:955113literature, coexpression++, MS/MS++MIMOXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits		all 14
SFXN3sideroflexin ­3BA108L7.2, SFX3, SLC56A3, Q9BWM7, gene:8185513mito protein domain+, MS/MS++MIMMetabolism > Amino acid metabolism > Serine metabolism;
Metabolism > Vitamin metabolism > Folate and 1-C metabolism;
Small molecule transport > Sideroflexins		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
AASSaminoadipate-semialdehyde ­synthaseLKR/SDH, LKRSDH, LORSDH, Q9UDR5, gene:1015713APEX_matrix, targetP signal, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Amino acid metabolism > Lysine metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, largeintestine, stomach, placenta, testis
DIABLOdiablo ­IAP-binding ­mitochondrial ­proteinDFNA64, SMAC, Q9NR28, gene:5661613literature, APEX_IMS, targetP signal+, mito protein domain+, MS/MSIMSMitochondrial dynamics and surveillance > Apoptosiscerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
COX8Ccytochrome ­c ­oxidase ­subunit ­8CCOX8-3, Q7Z4L0, gene:34194713literature, targetP signal+, mito protein domain+, MS/MSMIMOXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits		testis
PXMP2peroxisomal ­membrane ­protein ­2MPV17L3, PMP22, Q9NR77, gene:582713GFP, targetP signal, yeast mito homolog+, mito protein domain, inductionMIMnonecerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, placenta, testis
AGXT2alanine--glyoxylate ­aminotransferase ­2AGT2, BAIBA, DAIBAT, Q9BYV1, gene:6490213literature, yeast mito homolog++, mito protein domain, MS/MS+MatrixMetabolism > Amino acid metabolism > Glyoxylate metabolismkidney, liver
GLS2glutaminase ­2GA, GLS, LGA, hLGA, Q9UI32, gene:2716513targetP signal, mito protein domain+, MS/MS++MatrixMetabolism > Amino acid metabolism > Glutamate metabolismcerebrum, cerebellum, brainstem, spinalcord, liver, skeletalmuscle, smallintestine, largeintestine
APOOapolipoprotein ­OFAM121B, MIC26, MICOS26, Mic23, My025, Q9BUR5, gene:7913513literature, APEX_IMS, mito protein domain+, MS/MS++MIMMitochondrial dynamics and surveillance > Cristae formation > MICOS complexall 14
ACAD11acyl-CoA ­dehydrogenase ­family ­member ­11ACAD-11, Q709F0, gene:8412913mito protein domain+, induction, coexpressionMIMMetabolism > Lipid metabolism > Fatty acid oxidationspinalcord, kidney, liver, adipose
C5orf63chromosome ­5 ­open ­reading ­frame ­63YDR286C, A6NC05, gene:40120713targetP signal+, coexpression+, MS/MSMatrixnonecerebrum, cerebellum, spinalcord, liver, heart, skeletalmuscle, adipose, placenta, testis
HOGA14-hydroxy-2-oxoglutarate ­aldolase ­1C10orf65, DHDPS2, DHDPSL, HP3, NPL2, Q86XE5, gene:11281713GFP, targetP signal, Rickettsial homolog, mito protein domain, coexpression, MS/MSMatrixMetabolism > Amino acid metabolism > Glyoxylate metabolismkidney, liver, adipose, largeintestine, placenta, testis
TTC19tetratricopeptide ­repeat ­domain ­192010204O13Rik, MC3DN2, Q6DKK2, gene:5490213literature, APEX_IMS, targetP signal+, mito protein domain, induction, MS/MS+MIMOXPHOS > Complex III > CIII assembly factors;
OXPHOS > OXPHOS assembly factors		cerebrum, cerebellum, spinalcord, adipose, largeintestine, stomach, testis
NGRNneugrin, ­neurite ­outgrowth ­associatedDSC92, Q9NPE2, gene:5133512APEX_matrix, yeast mito homolog++, mito protein domain+, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications;
Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly		none
TCAIMT ­cell ­activation ­inhibitor, ­mitochondrialC3orf23, TOAG-1, TOAG1, Q8N3R3, gene:28534312literature, APEX_matrix, targetP signal, mito protein domain+, coexpression++Matrixnonenone
GCSHglycine ­cleavage ­system ­protein ­HGCE, NKH, P23434, gene:265312literature, APEX_matrix, targetP signal, yeast mito homolog, mito protein domain+, MS/MSMatrixMetabolism > Lipid metabolism > Lipoate insertion;
Metabolism > Amino acid metabolism > Glycine metabolism;
Metabolism > Amino acid metabolism > Glycine cleavage system		spinalcord, liver, adipose, smallintestine, largeintestine, placenta, testis
SIRT5sirtuin ­5SIR2L5, Q9NXA8, gene:2340812literature, APEX_matrix, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MSMatrixMetabolism > Metals and cofactors > NAD biosynthesis and metabolismcerebrum, brainstem, spinalcord, liver, heart, adipose, largeintestine, stomach
GPT2glutamic--pyruvic ­transaminase ­2ALT2, GPT 2, MRT49, Q8TD30, gene:8470612APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, MS/MS++MatrixMetabolism > Amino acid metabolism > Glutamate metabolismcerebrum, brainstem, liver, skeletalmuscle, adipose, stomach, placenta, testis
TOMM6translocase ­of ­outer ­mitochondrial ­membrane ­6OBTP, TOM6, Q96B49, gene:10018889312GFP, mito protein domain+, coexpression, MS/MS+MOMProtein import, sorting and homeostasis > Protein import and sorting > TOMcerebrum, cerebellum, brainstem, spinalcord, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
FAM162Afamily ­with ­sequence ­similarity ­162 ­member ­AC3orf28, E2IG5, HGTD-P, Q96A26, gene:2635512APEX_IMS, APEX_matrix, targetP signal, induction, coexpression, MS/MS++MIMnoneall 14
ABCB6ATP ­binding ­cassette ­subfamily ­B ­member ­6 ­(Langereis ­blood ­group)ABC, LAN, MTABC3, PRP, umat, Q9NP58, gene:1005812literature, yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpression, MS/MSMOMMetabolism > Metals and cofactors > Heme synthesis and processing;
Small molecule transport > ABC transporters		cerebrum, brainstem, liver, largeintestine, stomach, placenta, testis
MACROD1mono-ADP ­ribosylhydrolase ­1LRP16, Q9BQ69, gene:2899212APEX_matrix, targetP signal+, coexpression, MS/MS++MatrixSignalingcerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
TSTthiosulfate ­sulfurtransferaseRDS, Q16762, gene:726312literature, APEX_matrix, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Detoxification > Xenobiotic metabolism;
Metabolism > Sulfur metabolism		all 14
MT-CO1cytochrome ­c ­oxidase ­subunit ­ICOI, MTCO1, P00395, gene:451212literature, APEX_IMS, yeast mito homolog++, Rickettsial homolog, mito protein domain, MS/MSMIMOXPHOS > Complex IV > CIV subunits;
Metabolism > Metals and cofactors > Heme-containing proteins;
OXPHOS > OXPHOS subunits		all 14
MTFR1Lmitochondrial ­fission ­regulator ­1 ­likeFAM54B, HYST1888, MST116, MSTP116, Q9H019, gene:5618112mito protein domain+, induction, coexpression, MS/MSunknownnonecerebrum, cerebellum, brainstem, spinalcord, placenta, testis
SDR39U1short ­chain ­dehydrogenase/reductase ­family ­39U ­member ­1C14orf124, HCDI, Q9NRG7, gene:5694812mito protein domain, coexpression, MS/MS++unknownnonecerebrum, cerebellum, brainstem, spinalcord, kidney, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
DMGDHdimethylglycine ­dehydrogenaseDMGDHD, ME2GLYDH, Q9UI17, gene:2995812literature, targetP signal, mito protein domain, MS/MS++MatrixMetabolism > Amino acid metabolism > Glycine metabolism;
Metabolism > Vitamin metabolism > Choline and betaine metabolism;
Metabolism > Vitamin metabolism > Folate and 1-C metabolism		spinalcord, kidney, liver, smallintestine, stomach, placenta
SLC25A14solute ­carrier ­family ­25 ­member ­14BMCP1, UCP5, O95258, gene:901612literature, yeast mito homolog+, mito protein domain+, MS/MSMIMMetabolism > Sulfur metabolism;
Small molecule transport > SLC25A family		cerebrum, cerebellum, brainstem, placenta, testis
PRELID3APRELI ­domain ­containing ­3AC18orf43, HFL-EDDG1, SLMO1, Q96N28, gene:1065012yeast mito homolog+, mito protein domain+, MS/MSIMSMetabolism > Lipid metabolism > Cardiolipin synthesis;
Metabolism > Lipid metabolism > Phospholipid metabolism		cerebrum, cerebellum, spinalcord
CHCHD5coiled-coil-helix-coiled-coil-helix ­domain ­containing ­5C2orf9, CHTM1, MIC14, MIX14, Q9BSY4, gene:8426912APEX_IMS, yeast mito homolog++, mito protein domain, coexpression, MS/MSIMSnoneadipose, placenta, testis
TOMM70translocase ­of ­outer ­mitochondrial ­membrane ­70TOMM70A, Tom70, O94826, gene:986812literature, yeast mito homolog+, mito protein domain, induction, coexpressionMOMProtein import, sorting and homeostasis > Protein import and sorting > TOMcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
NIT1nitrilase ­1Q86X76, gene:481712APEX_matrix, targetP signal, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Detoxification > ROS and glutathione metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
FAHD1fumarylacetoacetate ­hydrolase ­domain ­containing ­1C16orf36, YISKL, Q6P587, gene:8188912literature, APEX_matrix, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Carbohydrate metabolism > TCA-associatedall 14
MCUmitochondrial ­calcium ­uniporterC10orf42, CCDC109A, HsMCU, Q8NE86, gene:9055012literature, GFP, mito protein domain+, MS/MS++MIMSignaling > Calcium homeostasis > Calcium cycle;
Small molecule transport > Calcium uniporter		cerebrum, cerebellum, brainstem, spinalcord, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta
CYP27A1cytochrome ­P450 ­family ­27 ­subfamily ­A ­member ­1CP27, CTX, CYP27, Q02318, gene:159312literature, targetP signal+, mito protein domain, MS/MS++MIMMetabolism > Lipid metabolism > Cholesterol, bile acid, steroid synthesis;
Metabolism > Metals and cofactors > Heme-containing proteins		kidney, liver, skeletalmuscle, adipose, smallintestine, placenta, testis
MT-ND2MTND2MTND2, P03891, gene:453612literature, Rickettsial homolog, mito protein domain+, MS/MS+MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		cerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
MT-ND4NADH ­dehydrogenase, ­subunit ­4 ­(complex ­I)MTND4, P03905, gene:453812literature, APEX_IMS, APEX_matrix, Rickettsial homolog, mito protein domain+, MS/MS+MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		all 14
MT-ND5NADH ­dehydrogenase, ­subunit ­5 ­(complex ­I)MTND5, P03915, gene:454012literature, APEX_IMS, APEX_matrix, Rickettsial homolog, mito protein domain+, MS/MS+MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		all 14
DELE1DAP3 ­binding ­cell ­death ­enhancer ­1DELE, DELE1(L), KIAA0141, Q14154, gene:981212targetP signal+, mito protein domain, induction, coexpressionMIMSignalingnone
FASTKD1FAST ­kinase ­domains ­1Q53R41, gene:7967512APEX_matrix, induction, coexpression++MatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > Polycistronic mtRNA processing;
Mitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay		liver, largeintestine
MRPS31mitochondrial ­ribosomal ­protein ­S31IMOGN38, MRP-S31, S31mt, Q92665, gene:1024012literature, APEX_matrix, targetP signal, induction, coexpression, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
ALDH1L1aldehyde ­dehydrogenase ­1 ­family ­member ­L110-FTHFDH, 10-fTHF, FDH, FTHFD, O75891, gene:1084012APEX_matrix, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MSMatrixMetabolism > Vitamin metabolism > Folate and 1-C metabolismcerebrum, liver, smallintestine, largeintestine, stomach, placenta, testis
PTCD1pentatricopeptide ­repeat ­domain ­1O75127, gene:2602411targetP signal+, mito protein domain+, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granulescerebellum, largeintestine, placenta
HADHBhydroxyacyl-CoA ­dehydrogenase ­trifunctional ­multienzyme ­complex ­subunit ­betaECHB, MSTP029, MTPB, TP-BETA, P55084, gene:303211literature, targetP signal, Rickettsial homolog, mito protein domain, MS/MS++MIMMetabolism > Carbohydrate metabolism > Ketone metabolism;
Metabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Amino acid metabolism > Lysine metabolism		all 14
TRIT1tRNA ­isopentenyltransferase ­1COXPD35, GRO1, IPPT, IPT, IPTase, MOD5, hGRO1, Q9H3H1, gene:5480211literature, APEX_matrix, targetP signal, yeast mito homolog, Rickettsial homolog, mito protein domain, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications;
Mitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications		cerebellum
CRLS1cardiolipin ­synthase ­1C20orf155, CLS, CLS1, GCD10, dJ967N21.6, Q9UJA2, gene:5467511literature, targetP signal, yeast mito homolog, Rickettsial homolog, mito protein domain, coexpressionMIMMetabolism > Lipid metabolism > Cardiolipin synthesisnone
GLYCTKglycerate ­kinaseHBEBP2, HBEBP4, HBeAgBP4A, Q8IVS8, gene:13215811targetP signal+, coexpression, MS/MS+MatrixMetabolism > Carbohydrate metabolismkidney, liver, placenta
SLC25A32solute ­carrier ­family ­25 ­member ­32MFT, MFTC, RREI, Q9H2D1, gene:8103411literature, targetP signal, yeast mito homolog+, mito protein domain+MIMMetabolism > Vitamin metabolism > Vitamin B2 metabolism;
Metabolism > Vitamin metabolism > Folate and 1-C metabolism;
Small molecule transport > SLC25A family		adipose, placenta
AARS2alanyl-tRNA ­synthetase ­2, ­mitochondrialAARSL, COXPD8, LKENP, MT-ALARS, MTALARS, Q5JTZ9, gene:5750511literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, MS/MSMatrixMitochondrial central dogma > Translation > mt-tRNA synthetasescerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
EXOGexo/endonuclease ­GENDOGL1, ENDOGL2, ENGL, ENGL-a, ENGL-b, ENGLA, ENGLB, Q9Y2C4, gene:994111APEX_IMS, targetP signal, mito protein domain, MS/MS++MIMMitochondrial central dogma > mtDNA maintenance > mtDNA replication;
Mitochondrial central dogma > mtDNA maintenance > mtDNA repair;
Mitochondrial central dogma > mtDNA maintenance > mtDNA stability and decay		cerebrum, cerebellum, brainstem, spinalcord, adipose, largeintestine, placenta
CHCHD2coiled-coil-helix-coiled-coil-helix ­domain ­containing ­2C7orf17, MIX17B, MNRR1, NS2TP, PARK22, Q9Y6H1, gene:5114211APEX_IMS, targetP signal, yeast mito homolog+, mito protein domain, coexpressionIMSMitochondrial dynamics and surveillance > Apoptosisnone
KMOkynurenine ­3-monooxygenasedJ317G22.1, O15229, gene:856411yeast mito homolog, Rickettsial homolog, mito protein domain, MS/MS++MOMMetabolism > Amino acid metabolism > Kynurenine metabolism;
Metabolism > Metals and cofactors > NAD biosynthesis and metabolism		kidney, liver
SFXN5sideroflexin ­5BBG-TCC, SLC56A5, Q8TD22, gene:9409711literature, mito protein domain+, MS/MS++MIMMetabolism > Carbohydrate metabolism > TCA-associated;
Small molecule transport > Sideroflexins		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, largeintestine
ELAC2elaC ­ribonuclease ­Z ­2COXPD17, ELC2, HPC2, Q9BQ52, gene:6052811APEX_matrix, GFP, targetP signal, Rickettsial homolog, mito protein domain, MS/MSMatrixMitochondrial central dogma > mtRNA metabolism > Polycistronic mtRNA processingcerebellum, skeletalmuscle, adipose, largeintestine, stomach
GPX1glutathione ­peroxidase ­1GPXD, GSHPX1, P07203, gene:287611literature, APEX_matrix, targetP signal, mito protein domain, coexpression, MS/MS+MatrixMetabolism > Detoxification > ROS and glutathione metabolism;
Metabolism > Detoxification > Selenoproteins		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
CPT1Bcarnitine ­palmitoyltransferase ­1BCPT1-M, CPT1M, CPTI, CPTI-M, M-CPT1, MCCPT1, MCPT1, Q92523, gene:137511literature, yeast mito homolog+, mito protein domain, MS/MS++MOMMetabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Metals and cofactors > Carnitine synthesis and transport;
Metabolism > Metals and cofactors > Carnitine shuttle		cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
FIS1fission, ­mitochondrial ­1CGI-135, TTC11, Q9Y3D6, gene:5102411literature, yeast mito homolog++, mito protein domain+MOMMitochondrial dynamics and surveillance > Fissionall 14
PRELID1PRELI ­domain ­containing ­1CGI-106, PRELI, PX19, SBBI12, Q9Y255, gene:2716611literature, APEX_IMS, GFP, targetP signal, yeast mito homolog++, mito protein domain+IMSMetabolism > Lipid metabolism > Cardiolipin synthesis;
Metabolism > Lipid metabolism > Phospholipid metabolism		none
UCP3uncoupling ­protein ­3SLC25A9, P55916, gene:735211literature, yeast mito homolog+, mito protein domain+, MS/MSMIMSmall molecule transport > SLC25A familyspinalcord, heart, skeletalmuscle, adipose
SLC25A27solute ­carrier ­family ­25 ­member ­27UCP4, O95847, gene:948111literature, yeast mito homolog+, mito protein domain+, MS/MSMIMSmall molecule transport > SLC25A familycerebrum, cerebellum, brainstem, spinalcord
SLC25A40solute ­carrier ­family ­25 ­member ­40MCFP, Q8TBP6, gene:5597211literature, yeast mito homolog+, mito protein domain+, MS/MSMIMSmall molecule transport > SLC25A familycerebrum, cerebellum, brainstem, spinalcord, adipose, largeintestine, stomach, placenta, testis
SLC25A23solute ­carrier ­family ­25 ­member ­23APC2, MCSC2, SCaMC-3, Q9BV35, gene:7908511literature, yeast mito homolog+, mito protein domain+, MS/MSMIMMetabolism > Nucleotide metabolism > Nucleotide import;
Signaling > Calcium homeostasis > EF hand proteins;
Small molecule transport > SLC25A family		cerebrum, brainstem, spinalcord, liver, adipose, smallintestine, largeintestine, stomach, placenta
HEMK1HemK ­methyltransferase ­family ­member ­1HEMK, MPRMC, MTQ1, Q9Y5R4, gene:5140911GFP, targetP signal, yeast mito homolog, Rickettsial homolog, mito protein domain+MatrixMitochondrial central dogma > Translation > Translation factorsnone
SLC25A36solute ­carrier ­family ­25 ­member ­36PNC2, Q96CQ1, gene:5518611GFP, yeast mito homolog+, mito protein domain+, coexpressionMIMMetabolism > Nucleotide metabolism > Nucleotide import;
Small molecule transport > SLC25A family		brainstem, spinalcord, placenta, testis
OMA1OMA1 ­zinc ­metallopeptidase2010001O09Rik, DAB1, MPRP-1, MPRP1, YKR087C, ZMPOMA1, peptidase, Q96E52, gene:11520911APEX_IMS, GFP, targetP signal+, yeast mito homolog++, coexpressionMIMProtein import, sorting and homeostasis > Protein homeostasis > Proteases;
Mitochondrial dynamics and surveillance > Fusion;
Mitochondrial dynamics and surveillance > Fission		skeletalmuscle, adipose, largeintestine
LETM2leucine ­zipper ­and ­EF-hand ­containing ­transmembrane ­protein ­2SLC55A2, Q2VYF4, gene:13799411targetP signal, yeast mito homolog+, mito protein domain+MIMnonenone
DHRS1dehydrogenase/reductase ­1SDR19C1, Q96LJ7, gene:11581711Rickettsial homolog, mito protein domain, MS/MS++MIMMetabolism > Lipid metabolism > Cholesterol, bile acid, steroid synthesiscerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
STOMstomatinBND7, EPB7, EPB72, P27105, gene:204011yeast mito homolog+, Rickettsial homolog, mito protein domain, MS/MSMembranenoneadipose, placenta, testis
METTL5methyltransferase ­like ­5HSPC133, MRT72, Q9NRN9, gene:2908110Rickettsial homolog, mito protein domain+, induction, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mt-rRNA modificationsnone
MSRAmethionine ­sulfoxide ­reductase ­APMSR, Q9UJ68, gene:448210mito protein domain, coexpression, MS/MS++MatrixMetabolism > Detoxification > ROS and glutathione metabolism;
Metabolism > Sulfur metabolism		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta
NUDT8nudix ­hydrolase ­8Q8WV74, gene:25455210APEX_matrix, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Metals and cofactors > Coenzyme A metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
NMNAT3nicotinamide ­nucleotide ­adenylyltransferase ­3FKSG76, PNAT-3, PNAT3, Q96T66, gene:34956510literature, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Metals and cofactors > NAD biosynthesis and metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, placenta, testis
SLC25A38solute ­carrier ­family ­25 ­member ­38SIDBA2, Q96DW6, gene:5497710yeast mito homolog++, mito protein domain+, coexpressionMIMMetabolism > Amino acid metabolism > Glycine metabolism;
Metabolism > Metals and cofactors > Heme synthesis and processing;
Metabolism > Vitamin metabolism > Folate and 1-C metabolism;
Small molecule transport > SLC25A family		none
MPV17L2MPV17 ­mitochondrial ­inner ­membrane ­protein ­like ­2FKSG24, Q567V2, gene:8476910GFP, targetP signal, yeast mito homolog+, mito protein domain, coexpressionMIMMitochondrial central dogma > Translation > Mitochondrial ribosome assemblylargeintestine, placenta
ACLYATP ­citrate ­lyaseACL, ATPCL, CLATP, P53396, gene:4710APEX_matrix, yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpressionMatrixMetabolism > Carbohydrate metabolism > TCA-associatedcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, largeintestine, stomach, placenta, testis
ABCD2ATP ­binding ­cassette ­subfamily ­D ­member ­2ABC39, ALDL1, ALDR, ALDRP, hALDR, Q9UBJ2, gene:22510targetP signal, Rickettsial homolog, mito protein domain, coexpression, MS/MS+MembraneSmall molecule transport > ABC transportersadipose
FLAD1flavin ­adenine ­dinucleotide ­synthetase ­1FAD1, FADS, LSMFLAD, PP591, Q8NFF5, gene:8030810APEX_matrix, targetP signal, mito protein domain, induction, coexpressionMatrixMetabolism > Vitamin metabolism > Vitamin B2 metabolismspinalcord, adipose, stomach
LIPT1lipoyltransferase ­1LIPT1D, Q9Y234, gene:5160110GFP, yeast mito homolog++, mito protein domain, coexpressionMatrixMetabolism > Lipid metabolism > Lipoate insertionadipose, placenta
DGLUCYD-glutamate ­cyclaseC14orf159, Q7Z3D6, gene:8001710literature, APEX_matrix, targetP signal, coexpression, MS/MS++MatrixMetabolism > Amino acid metabolismall 14
PCK2phosphoenolpyruvate ­carboxykinase ­2, ­mitochondrialPEPCK, PEPCK-M, PEPCK2, Q16822, gene:510610literature, targetP signal, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Carbohydrate metabolism > Gluconeogenesis;
Metabolism > Carbohydrate metabolism > TCA-associated		cerebrum, cerebellum, brainstem, spinalcord, adipose, smallintestine, largeintestine, stomach, placenta, testis
NADK2NAD ­kinase ­2, ­mitochondrialC5orf33, DECRD, MNADK, NADKD1, Q4G0N4, gene:13368610APEX_matrix, targetP signal, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Metals and cofactors > NAD biosynthesis and metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
MPSTmercaptopyruvate ­sulfurtransferaseMST, TST2, TUM1, P25325, gene:435710APEX_matrix, mito protein domain, coexpression, MS/MS++MIMMetabolism > Detoxification > Xenobiotic metabolism;
Metabolism > Sulfur metabolism		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
NUDT13nudix ­hydrolase ­13Q86X67, gene:2596110GFP, targetP signal, mito protein domain, induction, coexpression, MS/MSMatrixMetabolism > Metals and cofactors > NAD biosynthesis and metabolismcerebellum, brainstem, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta
ACACAacetyl-CoA ­carboxylase ­alphaACAC, ACACAD, ACC, ACC1, ACCA, Q13085, gene:3110targetP signal, Rickettsial homolog, mito protein domain, coexpressionMatrixMetabolism > Lipid metabolism > Type II fatty acid synthesis;
Metabolism > Vitamin metabolism > Biotin utilizing proteins		brainstem, spinalcord, liver, adipose, largeintestine, stomach, placenta, testis
ABAT4-aminobutyrate ­aminotransferaseGABA-AT, GABAT, NPD009, P80404, gene:1810literature, APEX_matrix, targetP signal, mito protein domain, MS/MS++MatrixMetabolism > Amino acid metabolism > GABA metabolism;
Metabolism > Metals and cofactors > Fe-S-containing proteins		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
HMGCS23-hydroxy-3-methylglutaryl-CoA ­synthase ­2P54868, gene:315810literature, targetP signal, mito protein domain, MS/MS++MatrixMetabolism > Carbohydrate metabolism > Ketone metabolismcerebellum, spinalcord, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
ALDH5A1aldehyde ­dehydrogenase ­5 ­family ­member ­A1SSADH, SSDH, P51649, gene:791510APEX_matrix, targetP signal, mito protein domain, MS/MS++MatrixMetabolism > Amino acid metabolism > GABA metabolismall 14
CKMT1Acreatine ­kinase, ­mitochondrial ­1ACKMT1, U-MtCK, mia-CK, 0, gene:54859610literature, targetP signal, mito protein domain, MS/MS++MIMMetabolism > Nucleotide metabolism > Creatine metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta
PISDphosphatidylserine ­decarboxylaseDJ858B16, LIBF, PSD, PSDC, PSSC, dJ858B16.2, Q9UG56, gene:2376110yeast mito homolog++, mito protein domain, MS/MSMIMMetabolism > Lipid metabolism > Phospholipid metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
NDUFB4NADH:ubiquinone ­oxidoreductase ­subunit ­B4B15, CI-B15, O95168, gene:471010literature, mito protein domain+, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		all 14
UQCR11ubiquinol-cytochrome ­c ­reductase, ­complex ­III ­subunit ­XI0710008D09Rik, QCR10, UQCR, O14957, gene:1097510literature, mito protein domain+, MS/MS++MIMOXPHOS > Complex III > CIII subunits;
OXPHOS > OXPHOS subunits		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, stomach, placenta, testis
FAM136Afamily ­with ­sequence ­similarity ­136 ­member ­AQ96C01, gene:8490810APEX_IMS, mito protein domain+, MS/MS++IMSnoneall 14
TOMM5translocase ­of ­outer ­mitochondrial ­membrane ­5C9orf105, Tom5, bA613M10.3, Q8N4H5, gene:40150510literature, mito protein domain+, MS/MS++MOMProtein import, sorting and homeostasis > Protein import and sorting > TOMcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
HTRA2HtrA ­serine ­peptidase ­2MGCA8, OMI, PARK13, PRSS25, O43464, gene:2742910literature, APEX_IMS, targetP signal, Rickettsial homolog, mito protein domain, MS/MS+MIMProtein import, sorting and homeostasis > Protein homeostasis > Proteases;
Mitochondrial dynamics and surveillance > Apoptosis		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
NDUFA1NADH:ubiquinone ­oxidoreductase ­subunit ­A1CI-MWFE, MC1DN12, MWFE, ZNF183, O15239, gene:469410literature, coexpression+, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		all 14
MT-CYBcytochrome ­bMTCYB, P00156, gene:451910literature, yeast mito homolog++, Rickettsial homolog, mito protein domainMIMOXPHOS > Complex III > CIII subunits;
Metabolism > Metals and cofactors > Heme-containing proteins;
Metabolism > Electron carriers > Cytochromes;
OXPHOS > OXPHOS subunits		all 14
GUK1guanylate ­kinase ­1GMK, Q16774, gene:298710APEX_matrix, targetP signal, Rickettsial homolog, induction, coexpression, MS/MSMatrixMetabolism > Nucleotide metabolism > Nucleotide synthesis and processingcerebrum, cerebellum, brainstem, spinalcord, adipose, largeintestine, stomach
D2HGDHD-2-hydroxyglutarate ­dehydrogenaseD2HGD, Q8N465, gene:72829410literature, APEX_matrix, targetP signal, yeast mito homolog++, mito protein domainMatrixMetabolism > Carbohydrate metabolism > TCA-associatedadipose, largeintestine
OPA3outer ­mitochondrial ­membrane ­lipid ­metabolism ­regulator ­OPA3MGA3, Q9H6K4, gene:8020710literature, targetP signal, coexpression, MS/MS++MOMnoneall 14
AKR1B10aldo-keto ­reductase ­family ­1 ­member ­B10AKR1B11, AKR1B12, ALDRLn, ARL-1, ARL1, HIS, HSI, O60218, gene:5701610induction, coexpression, MS/MS++unknownMetabolism > Vitamin metabolism > Vitamin A metabolism;
Metabolism > Detoxification > Xenobiotic metabolism		cerebrum, brainstem, spinalcord, kidney, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
PHYHphytanoyl-CoA ­2-hydroxylaseLN1, LNAP1, PAHX, PHYH1, RD, O14832, gene:526410targetP signal, induction, MS/MS++MatrixMetabolism > Lipid metabolismkidney, liver, smallintestine
NDUFAF2NADH:ubiquinone ­oxidoreductase ­complex ­assembly ­factor ­2B17.2L, MC1DN10, MMTN, NDUFA12L, mimitin, Q8N183, gene:9194210literature, APEX_matrix, induction, coexpression, MS/MS++MIMOXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors		all 14
ECSITECSIT ­signaling ­integratorSITPEC, Q9BQ95, gene:5129510literature, APEX_matrix, targetP signal, mito protein domain, induction, coexpression+MIMOXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors		brainstem, spinalcord, kidney, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
PARLpresenilin ­associated ­rhomboid ­likePRO2207, PSARL, PSARL1, PSENIP2, RHBDS1, Q9H300, gene:5548610literature, yeast mito homolog++, mito protein domain, MS/MSMIMProtein import, sorting and homeostasis > Protein homeostasis > Proteases;
Mitochondrial dynamics and surveillance > Mitophagy;
Mitochondrial dynamics and surveillance > Autophagy		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
MRPL42mitochondrial ­ribosomal ­protein ­L42HSPC204, L31MT, L42MT, MRP-L31, MRP-L42, MRP-S32, MRPL31, MRPS32, PTD007, RPML31, S32MT, Q9Y6G3, gene:2897710APEX_matrix, GFP, mito protein domain+, induction, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeliver, adipose, largeintestine, testis
DNAJC4DnaJ ­heat ­shock ­protein ­family ­(Hsp40) ­member ­C4DANJC4, HSPF2, MCG18, Q9NNZ3, gene:333810GFP, targetP signal+, Rickettsial homolog, mito protein domain, coexpressionMembraneProtein import, sorting and homeostasis > Protein homeostasislargeintestine
PAM16presequence ­translocase ­associated ­motor ­16CGI-136, MAGMAS, SMDMDM, TIM16, TIMM16, Q9Y3D7, gene:5102510literature, APEX_matrix, yeast mito homolog, MS/MS++MIMProtein import, sorting and homeostasis > Protein import and sorting > Import motorall 14
ALDH3A2aldehyde ­dehydrogenase ­3 ­family ­member ­A2ALDH10, FALDH, SLS, P51648, gene:22410targetP signal+, mito protein domain, coexpressionMembraneMetabolism > Detoxification > ROS and glutathione metabolismspinalcord, kidney, liver, smallintestine, largeintestine, stomach, placenta, testis
ACSL6acyl-CoA ­synthetase ­long ­chain ­family ­member ­6ACS2, FACL6, LACS 6, LACS2, LACS5, Q9UKU0, gene:2330510Rickettsial homolog, mito protein domain, MS/MS++MOMMetabolism > Lipid metabolism > Fatty acid oxidationcerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, largeintestine, stomach, testis
MRS2magnesium ­transporter ­MRS2HPT, MRS2L, Q9HD23, gene:5738010APEX_matrix, GFP, targetP signal, yeast mito homolog++, coexpressionMIMSmall molecule transport;
Signaling		cerebrum, cerebellum, brainstem, spinalcord, skeletalmuscle, smallintestine, largeintestine, stomach, placenta, testis
ABHD10abhydrolase ­domain ­containing ­10, ­depalmitoylaseQ9NUJ1, gene:553479literature, APEX_IMS, APEX_matrix, Rickettsial homolog, mito protein domain, MS/MS++MatrixMetabolism > Detoxification > Xenobiotic metabolismcerebrum, cerebellum, brainstem, spinalcord, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
HINT3histidine ­triad ­nucleotide ­binding ­protein ­3HINT4, Q9NQE9, gene:1351149Rickettsial homolog, mito protein domain, induction, coexpression+unknownnonebrainstem, heart, skeletalmuscle
PXMP4peroxisomal ­membrane ­protein ­4PMP24, Q9Y6I8, gene:112649targetP signal, mito protein domain+, coexpressionunknownnonekidney, liver, adipose
OSGEPL1O-sialoglycoprotein ­endopeptidase ­like ­1OSGEPL, Qri7, Q9H4B0, gene:641729APEX_matrix, targetP signal, yeast mito homolog, Rickettsial homolog, mito protein domain, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mt-tRNA modificationsnone
NUDT2nudix ­hydrolase ­2APAH1, P50583, gene:3189mito protein domain, coexpression, MS/MS++MatrixMetabolism > Nucleotide metabolism > Nucleotide synthesis and processingall 14
CYB5Bcytochrome ­b5 ­type ­BCYB5-M, CYPB5M, OMB5, O43169, gene:807779literature, mito protein domain, coexpression, MS/MS++MOMMetabolism > Metals and cofactors > Heme-containing proteins;
Metabolism > Detoxification > Xenobiotic metabolism;
Metabolism > Detoxification > Amidoxime reducing complex;
Metabolism > Electron carriers > Cytochromes		all 14
GRSF1G-rich ­RNA ­sequence ­binding ­factor ­1Q12849, gene:29269APEX_matrix, mito protein domain, induction, coexpression, MS/MSMatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > Polycistronic mtRNA processing;
Mitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay;
Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly		adipose, smallintestine, largeintestine, stomach, placenta, testis
MRPS18Bmitochondrial ­ribosomal ­protein ­S18BC6orf14, HSPC183, HumanS18a, MRP-S18-2, MRPS18-2, PTD017, S18amt, Q9Y676, gene:289739literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, brainstem, spinalcord, liver, heart, adipose, smallintestine, largeintestine, placenta, testis
DNAJC30DnaJ ­heat ­shock ­protein ­family ­(Hsp40) ­member ­C30WBSCR18, Q96LL9, gene:842779APEX_matrix, GFP, targetP signal+, Rickettsial homolog, mito protein domain, MS/MSMIMnonecerebrum, cerebellum, brainstem, spinalcord, heart, skeletalmuscle, adipose, largeintestine, stomach
CATcatalaseP04040, gene:8479yeast mito homolog++, mito protein domain, coexpressionIMSMetabolism > Detoxification > ROS and glutathione metabolismspinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
DGUOKdeoxyguanosine ­kinaseMTDPS3, NCPH, PEOB4, dGK, Q16854, gene:17169literature, APEX_matrix, targetP signal, mito protein domain, MS/MS++MatrixMetabolism > Nucleotide metabolism > Nucleotide synthesis and processingcerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
ACOT9acyl-CoA ­thioesterase ­9ACATE2, CGI-16, MT-ACT48, MTACT48, Q9Y305, gene:235979literature, targetP signal, mito protein domain, MS/MS++MIMMetabolism > Lipid metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
ACSS1acyl-CoA ­synthetase ­short ­chain ­family ­member ­1ACAS2L, ACECS1, AceCS2L, Q9NUB1, gene:845329literature, APEX_matrix, targetP signal, mito protein domain, MS/MS++MIMMetabolism > Lipid metabolism > Fatty acid oxidationcerebrum, cerebellum, brainstem, spinalcord, kidney, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
RFKriboflavin ­kinaseRIFK, Q969G6, gene:553129targetP signal, yeast mito homolog++, MS/MSMatrixMetabolism > Vitamin metabolism > Vitamin B2 metabolismbrainstem, spinalcord, kidney, adipose, smallintestine, largeintestine
STARD7StAR ­related ­lipid ­transfer ­domain ­containing ­7FAME2, GTT1, Q9NQZ5, gene:569109APEX_IMS, targetP signal, mito protein domain, induction, coexpression+IMSMetabolism > Lipid metabolism > Phospholipid metabolism;
Small molecule transport		none
CHPT1choline ­phosphotransferase ­1CPT, CPT1, Q8WUD6, gene:569949targetP signal, mito protein domain, induction, coexpression+MIMMetabolism > Lipid metabolism > Phospholipid metabolismnone
MT-ATP8ATP ­synthase ­F0 ­subunit ­8ATPase8, MTATP8, P03928, gene:45099literature, mito protein domain+, MS/MS++MIMOXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits		all 14
TIMMDC1translocase ­of ­inner ­mitochondrial ­membrane ­domain ­containing ­1C3orf1, MC1DN31, Q9NPL8, gene:513009literature, APEX_IMS, APEX_matrix, mito protein domain+, MS/MS++MIMOXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors		cerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
SPATA19spermatogenesis ­associated ­19CT132, SPAS1, spergen1, Q7Z5L4, gene:2199389mito protein domain+, MS/MS++unknownnonetestis
HTATIP2HIV-1 ­Tat ­interactive ­protein ­2CC3, SDR44U1, TIP30, Q9BUP3, gene:105539yeast mito homolog, mito protein domain+, MS/MSMembranenonecerebrum, cerebellum, brainstem, liver, adipose, placenta, testis
AKR7A2aldo-keto ­reductase ­family ­7 ­member ­A2AFAR, AFAR1, AFB1-AR1, AKR7, O43488, gene:85749APEX_matrix, targetP signal, coexpression, MS/MS++MatrixMetabolism > Amino acid metabolism > GABA metabolism;
Metabolism > Detoxification > Xenobiotic metabolism		all 14
DUTdeoxyuridine ­triphosphatasedUTPase, P33316, gene:18549literature, targetP signal, Rickettsial homolog, MS/MS++MatrixMetabolism > Nucleotide metabolism > Nucleotide synthesis and processingcerebrum, spinalcord, liver, heart, adipose, largeintestine, stomach, placenta
MT-CO3cytochrome ­c ­oxidase ­IIICOIII, MTCO3, P00414, gene:45149literature, yeast mito homolog++, Rickettsial homolog, mito protein domainMIMOXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits		all 14
DHRS7Bdehydrogenase/reductase ­7BCGI-93, SDR32C1, Q6IAN0, gene:259799Rickettsial homolog, mito protein domain, induction, coexpressionMembranenonecerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
MTRF1mitochondrial ­translation ­release ­factor ­1MRF1, MTTRF1, RF1, O75570, gene:96179literature, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domainMatrixMitochondrial central dogma > Translation > Translation factorsadipose
TMEM143transmembrane ­protein ­143Q96AN5, gene:552609targetP signal, coexpression+, MS/MSMIMnonecerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta
NT5M5',3'-nucleotidase, ­mitochondrialdNT-2, dNT2, mdN, Q9NPB1, gene:569539literature, targetP signal, mito protein domain, induction, MS/MSMatrixMetabolism > Nucleotide metabolism > Nucleotide synthesis and processingcerebrum, cerebellum, brainstem, spinalcord, skeletalmuscle, adipose, testis
RHOT1ras ­homolog ­family ­member ­T1ARHT1, MIRO-1, MIRO1, Q8IXI2, gene:552889literature, mito protein domain+, MS/MS+MOMSignaling > Calcium homeostasis > EF hand proteins;
Mitochondrial dynamics and surveillance > Trafficking		all 14
NME4NME/NM23 ­nucleoside ­diphosphate ­kinase ­4NDPK-D, NM23H4, nm23-H4, O00746, gene:48339APEX_matrix, GFP, targetP signal, Rickettsial homolog, mito protein domain, MS/MSMIMMetabolism > Nucleotide metabolism > Nucleotide synthesis and processingadipose, largeintestine, placenta, testis
MTHFSmethenyltetrahydrofolate ­synthetaseHsT19268, NEDMEHM, P49914, gene:105889targetP signal, Rickettsial homolog, mito protein domain, MS/MSMatrixMetabolism > Vitamin metabolism > Folate and 1-C metabolismcerebrum, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta
TIMM29translocase ­of ­inner ­mitochondrial ­membrane ­29C19orf52, TIM29, Q9BSF4, gene:905809targetP signal+, coexpression, MS/MSMIMProtein import, sorting and homeostasis > Protein import and sorting > TIM22 carrier pathwaycerebrum, cerebellum, brainstem, spinalcord, liver, heart, adipose, largeintestine, placenta, testis
PNPOpyridoxamine ­5'-phosphate ­oxidaseHEL-S-302, PDXPO, Q9NVS9, gene:551639APEX_matrix, yeast mito homolog++, coexpression, MS/MSMatrixMetabolism > Vitamin metabolism > Vitamin B6 metabolismbrainstem, liver
MICU2mitochondrial ­calcium ­uptake ­21110008L20Rik, EFHA1, Q8IYU8, gene:2211549literature, APEX_IMS, GFP, targetP signal+, mito protein domain, MS/MS+MIMSignaling > Calcium homeostasis > Calcium cycle;
Signaling > Calcium homeostasis > EF hand proteins;
Small molecule transport > Calcium uniporter		smallintestine, largeintestine, placenta, testis
PCBD2pterin-4 ­alpha-carbinolamine ­dehydratase ­2DCOH2, DCOHM, PHS2, Q9H0N5, gene:841059targetP signal+, induction, MS/MSunknownMetabolism > Metals and cofactors > Tetrahydrobiopterin synthesiscerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
QRSL1glutaminyl-tRNA ­amidotransferase ­subunit ­QRSL1COXPD40, GatA, Q9H0R6, gene:552789literature, APEX_matrix, GFP, yeast mito homolog, Rickettsial homolog, mito protein domain, coexpression, MS/MSMatrixMitochondrial central dogma > Translation > mt-tRNA synthetasesbrainstem, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
ADCK1aarF ­domain ­containing ­kinase ­1Q86TW2, gene:571439yeast mito homolog+, Rickettsial homolog, mito protein domain, MS/MSunknownnonecerebrum, spinalcord, kidney, heart, skeletalmuscle, adipose, stomach, placenta
PRDX6peroxiredoxin ­61-Cys, AOP2, HEL-S-128m, NSGPx, PRX, aiPLA2, p29, P30041, gene:95889yeast mito homolog++, Rickettsial homolog, mito protein domainunknownMetabolism > Lipid metabolism > Phospholipid metabolism;
Metabolism > Detoxification > ROS and glutathione metabolism		cerebrum, cerebellum, brainstem, spinalcord, liver, adipose, smallintestine, largeintestine, placenta, testis
NIPSNAP1nipsnap ­homolog ­1Q9BPW8, gene:85089APEX_matrix, targetP signal, coexpression, MS/MS++MatrixMitochondrial dynamics and surveillance > Mitophagy;
Mitochondrial dynamics and surveillance > Autophagy		all 14
GARS1glycyl-tRNA ­synthetase ­1CMT2D, DSMAV, GARS, GlyRS, HMN5, SMAD1, P41250, gene:26179targetP signal, yeast mito homolog, mito protein domain, inductionMatrixMitochondrial central dogma > Translation > mt-tRNA synthetasescerebrum, cerebellum, brainstem, spinalcord, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
ACSM2Aacyl-CoA ­synthetase ­medium ­chain ­family ­member ­2AA-923A4.1, ACSM2, Q08AH3, gene:1238769targetP signal, mito protein domain, MS/MS+MatrixMetabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Detoxification > Xenobiotic metabolism		kidney, liver, heart
SLC25A33solute ­carrier ­family ­25 ­member ­33BMSC-MCP, PNC1, Q9BSK2, gene:842759literature, yeast mito homolog+, mito protein domain+MIMMetabolism > Nucleotide metabolism > Nucleotide import;
Small molecule transport > SLC25A family		brainstem, spinalcord, placenta, testis
LYRM9LYR ­motif ­containing ­9C17orf108, HSD24, A8MSI8, gene:2012299targetP signal, mito protein domain+, MS/MSunknownnonecerebellum, heart, adipose, testis
RAB24RAB24, ­member ­RAS ­oncogene ­familyQ969Q5, gene:539179mito protein domain, induction, coexpression, MS/MSMOMMitochondrial dynamics and surveillance > Fission;
Mitochondrial dynamics and surveillance > Autophagy		cerebrum, cerebellum, brainstem, spinalcord, skeletalmuscle, adipose, stomach, testis
PARS2prolyl-tRNA ­synthetase ­2, ­mitochondrialEIEE75, MT-PRORS, proRS, Q7L3T8, gene:259738APEX_matrix, GFP, targetP signal, yeast mito homolog, Rickettsial homolog, mito protein domainMatrixMitochondrial central dogma > Translation > mt-tRNA synthetasessmallintestine, largeintestine, stomach
MRPL52mitochondrial ­ribosomal ­protein ­L52Q86TS9, gene:1227048literature, APEX_matrix, targetP signal, coexpression, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, spinalcord, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
PUS1pseudouridine ­synthase ­1MLASA1, Q9Y606, gene:803248APEX_matrix, targetP signal, yeast mito homolog+, mito protein domain, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications		none
GOT2glutamic-oxaloacetic ­transaminase ­2KAT4, KATIV, KYAT4, mitAAT, P00505, gene:28068literature, APEX_IMS, APEX_matrix, targetP signal, mito protein domain, MS/MS++MIMMetabolism > Carbohydrate metabolism > Malate-aspartate shuttle;
Metabolism > Amino acid metabolism > Glutamate metabolism;
Metabolism > Amino acid metabolism > Glyoxylate metabolism;
Metabolism > Amino acid metabolism > Kynurenine metabolism;
Metabolism > Sulfur metabolism		all 14
NAXDNAD(P)HX ­dehydrataseCARKD, LP3298, PEBEL2, Q8IW45, gene:557398GFP, targetP signal, mito protein domain, MS/MS++MIMMetabolism > Metals and cofactors > NAD biosynthesis and metabolismall 14
MT-ND3NADH ­dehydrogenase, ­subunit ­3 ­(complex ­I)MTND3, P03897, gene:45378literature, Rickettsial homolog, mito protein domain+, MS/MSMIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		cerebrum, cerebellum, brainstem, spinalcord, kidney, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta
ACCS1-aminocyclopropane-1-carboxylate ­synthase ­homolog ­(inactive)ACS, PHACS, Q96QU6, gene:846808yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpressionunknownnonenone
PTRH2peptidyl-tRNA ­hydrolase ­2BIT1, CFAP37, CGI-147, IMNEPD, PTH, PTH 2, PTH2, Q9Y3E5, gene:516518literature, yeast mito homolog, mito protein domain, MS/MS++MOMnoneall 14
PGS1phosphatidylglycerophosphate ­synthase ­1Q32NB8, gene:94898targetP signal, yeast mito homolog++, mito protein domainMIMMetabolism > Lipid metabolism > Cardiolipin synthesis;
Metabolism > Lipid metabolism > Phospholipid metabolism		cerebrum, spinalcord, placenta
OXR1oxidation ­resistance ­1CHEGDD, Nbla00307, TLDC3, Q8N573, gene:550748literature, yeast mito homolog+, mito protein domain, MS/MSMOMMetabolism > Detoxification > ROS and glutathione metabolismcerebrum, cerebellum, brainstem
LYPLAL1lysophospholipase ­like ­1Q96AV0, Q5VWZ2, gene:1270188mito protein domain, MS/MS++unknownMetabolism > Lipid metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, largeintestine, placenta, testis
DNAJC28DnaJ ­heat ­shock ­protein ­family ­(Hsp40) ­member ­C28C21orf55, C21orf78, Q9NX36, gene:549438Rickettsial homolog, mito protein domain, coexpression+unknownnonestomach
FPGSfolylpolyglutamate ­synthaseQ05932, gene:23568APEX_matrix, GFP, targetP signal, yeast mito homolog, Rickettsial homolog, mito protein domainMatrixMetabolism > Vitamin metabolism > Folate and 1-C metabolismnone
TMEM205transmembrane ­protein ­205UNQ501, Q6UW68, gene:3748828yeast mito homolog, mito protein domain+, coexpressionMembranenoneliver, adipose
SLC25A53solute ­carrier ­family ­25 ­member ­53MCART6, Q5H9E4, gene:4016128yeast mito homolog+, mito protein domain+MIMSmall molecule transport > SLC25A familytestis
AIFM3apoptosis ­inducing ­factor ­mitochondria ­associated ­3AIFL, Q96NN9, gene:1502098literature, yeast mito homolog, Rickettsial homolog, mito protein domain+MIMMetabolism > Metals and cofactors > Fe-S-containing proteins;
Mitochondrial dynamics and surveillance > Apoptosis		none
MGST1microsomal ­glutathione ­S-transferase ­1GST12, MGST, MGST-I, P10620, gene:42578mito protein domain, coexpression, MS/MS++MembraneMetabolism > Detoxification > Xenobiotic metabolism;
Metabolism > Detoxification > ROS and glutathione metabolism		cerebrum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
MTFP1mitochondrial ­fission ­process ­1HSPC242, MTP18, Q9UDX5, gene:515378literature, induction, coexpression, MS/MS+MembraneMitochondrial dynamics and surveillance > Fissionall 14
COA4cytochrome ­c ­oxidase ­assembly ­factor ­4 ­homologCHCHD8, CMC3, E2IG2, Q9NYJ1, gene:512878yeast mito homolog++, mito protein domain, MS/MSIMSOXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors		placenta
NIF3L1NGG1 ­interacting ­factor ­3 ­like ­1ALS2CR1, CALS-7, MDS015, Q9GZT8, gene:604918APEX_matrix, targetP signal, yeast mito homolog, coexpression, MS/MS+Matrixnonecerebrum, cerebellum, brainstem, spinalcord, skeletalmuscle, adipose, largeintestine, placenta, testis
PUSL1pseudouridine ­synthase ­like ­1Q8N0Z8, gene:1267898APEX_matrix, yeast mito homolog+, Rickettsial homolog, mito protein domainMatrixMitochondrial central dogma > Translationcerebrum, spinalcord, largeintestine, placenta
SDSLserine ­dehydratase ­likeSDH 2, SDS-RS1, TDH, cSDH, Q96GA7, gene:1136758yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpressionunknownMetabolism > Amino acid metabolism > Serine metabolismnone
BAXBCL2 ­associated ­X, ­apoptosis ­regulatorBCL2L4, Q07812, gene:5818literature, mito protein domain, coexpression, MS/MS++MOMMitochondrial dynamics and surveillance > Apoptosiscerebrum, cerebellum, brainstem, adipose, largeintestine, placenta, testis
MAVSmitochondrial ­antiviral ­signaling ­proteinCARDIF, IPS-1, IPS1, VISA, Q7Z434, gene:575068literature, induction, coexpression+, MS/MSMOMSignaling > Immune responsebrainstem, skeletalmuscle, adipose, placenta, testis
METTL17methyltransferase ­like ­17METT11D1, Q9H7H0, gene:647458GFP, yeast mito homolog++, mito protein domain+MatrixMitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications;
Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly		none
ACACBacetyl-CoA ­carboxylase ­betaACC2, ACCB, HACC275, O00763, gene:328Rickettsial homolog, mito protein domain, coexpression+MOMMetabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Vitamin metabolism > Biotin utilizing proteins		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, largeintestine, stomach, placenta, testis
ARG2arginase ­2P78540, gene:3848literature, APEX_matrix, targetP signal+, mito protein domain, MS/MSMatrixMetabolism > Amino acid metabolismkidney, adipose, smallintestine, largeintestine, placenta
MTPAPmitochondrial ­poly(A) ­polymerasePAPD1, SPAX4, TENT6, Q9NVV4, gene:551497literature, APEX_matrix, targetP signal+, mito protein domain, inductionMatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications;
Mitochondrial central dogma > mtRNA metabolism > mt-mRNA modifications;
Mitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay		cerebrum, cerebellum, spinalcord, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
CCDC51coiled-coil ­domain ­containing ­51MITOK, Q96ER9, gene:797147APEX_matrix, targetP signal, MS/MS++MIMSmall molecule transportall 14
SUGCTsuccinyl-CoA:glutarate-CoA ­transferaseC7orf10, DERP13, GA3, ORF19, Q9HAC7, gene:797837APEX_matrix, targetP signal, mito protein domain, coexpression, MS/MS+MatrixMetabolism > Amino acid metabolismkidney, liver, testis
DNM1Ldynamin ­1 ­likeDLP1, DRP1, DVLP, DYMPLE, EMPF, EMPF1, HDYNIV, OPA5, O00429, gene:100597literature, yeast mito homolog+, mito protein domain, inductionMOMMitochondrial dynamics and surveillance > Fissioncerebrum, cerebellum, brainstem, spinalcord
NRDCnardilysin ­convertaseNRD1, hNRD1, hNRD2, O43847, gene:48987targetP signal, Rickettsial homolog, mito protein domain+MatrixProtein import, sorting and homeostasis > Protein homeostasis > Chaperones;
Protein import, sorting and homeostasis > Protein import and sorting		testis
TMEM126Btransmembrane ­protein ­126BHT007, MC1DN29, Q8IUX1, gene:558637targetP signal, mito protein domain+, MS/MSMIMOXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors		cerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
NSUN4NOP2/Sun ­RNA ­methyltransferase ­4SHTAP, Q96CB9, gene:3873387APEX_matrix, targetP signal, mito protein domain, induction, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications;
Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly		placenta
ACSS3acyl-CoA ­synthetase ­short ­chain ­family ­member ­3Q9H6R3, gene:796117Rickettsial homolog, mito protein domain, MS/MSMatrixMetabolism > Lipid metabolismkidney, liver, adipose, placenta, testis
FAM210Bfamily ­with ­sequence ­similarity ­210 ­member ­B5A3, C20orf108, dJ1167H4.1, Q96KR6, gene:1161517targetP signal, yeast mito homolog, coexpression, MS/MSMIMMetabolism > Metals and cofactors > Iron homeostasiscerebrum, cerebellum, brainstem, spinalcord, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
RBFAribosome ­binding ­factor ­AC18orf22, HsT169, Q8N0V3, gene:798637APEX_matrix, targetP signal+, Rickettsial homolog, coexpressionMatrixMitochondrial central dogma > Translation > Mitochondrial ribosome assemblynone
METAP1Dmethionyl ­aminopeptidase ­type ­1D, ­mitochondrialMAP 1D, MAP1D, MetAP 1D, Metap1l, Q6UB28, gene:2540427literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, coexpressionMatrixMitochondrial central dogma > Translation > fMet processing;
Protein import, sorting and homeostasis > Protein homeostasis > Proteases		none
AHCYL1adenosylhomocysteinase ­like ­1DCAL, IRBIT, PPP1R78, PRO0233, XPVKONA, O43865, gene:107687targetP signal, mito protein domain+, inductionMOMMitochondrial dynamics and surveillance > Organelle contact sitesbrainstem, spinalcord, smallintestine, stomach
NIPSNAP3Anipsnap ­homolog ­3AHSPC299, NIPSNAP4, TASSC, Q9UFN0, gene:259347APEX_matrix, targetP signal+, MS/MS++Matrixnonecerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
MTARC1mitochondrial ­amidoxime ­reducing ­component ­1MARC1, MOSC1, Q5VT66, gene:647577mito protein domain+, MS/MSMOMMetabolism > Metals and cofactors > Molybdenum cofactor synthesis and proteins;
Metabolism > Detoxification > Xenobiotic metabolism;
Metabolism > Detoxification > Amidoxime reducing complex		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
FUNDC1FUN14 ­domain ­containing ­1Q8IVP5, gene:1393417yeast mito homolog, mito protein domain+, MS/MSMOMMitochondrial dynamics and surveillance > Mitophagy;
Mitochondrial dynamics and surveillance > Autophagy		cerebrum, cerebellum, brainstem, spinalcord, kidney, skeletalmuscle, adipose, largeintestine, stomach, testis
ACSL1acyl-CoA ­synthetase ­long ­chain ­family ­member ­1ACS1, FACL1, FACL2, LACS, LACS1, LACS2, P33121, gene:21807Rickettsial homolog, mito protein domain, coexpressionMOMMetabolism > Lipid metabolism > Fatty acid oxidationall 14
COX20cytochrome ­c ­oxidase ­assembly ­factor ­COX20FAM36A, Q5RI15, gene:1162287literature, APEX_IMS, GFP, yeast mito homolog++, MS/MSMIMOXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors		all 14
HSDL1hydroxysteroid ­dehydrogenase ­like ­1SDR12C3, Q3SXM5, gene:836937GFP, Rickettsial homolog, mito protein domain, MS/MS++unknownnonecerebrum, cerebellum, brainstem, spinalcord, adipose, smallintestine, largeintestine, stomach, placenta, testis
CBR3carbonyl ­reductase ­3HEL-S-25, SDR21C2, hCBR3, O75828, gene:8747targetP signal, Rickettsial homolog, mito protein domain, MS/MSMatrixMetabolism > Detoxification > Xenobiotic metabolismcerebrum, spinalcord, adipose, smallintestine, largeintestine, stomach
MTERF4mitochondrial ­transcription ­termination ­factor ­4MTERFD2, Q7Z6M4, gene:1309167APEX_matrix, targetP signal, mito protein domain+, coexpressionMatrixMitochondrial central dogma > Translation > Mitochondrial ribosome assemblynone
NUDT19nudix ­hydrolase ­19RP2, A8MXV4, gene:3909167APEX_matrix, targetP signal, mito protein domain, inductionMatrixMetabolism > Metals and cofactors > Coenzyme A metabolismkidney, liver, largeintestine, placenta
HEBP1heme ­binding ­protein ­1HBP, HEBP, Q9NRV9, gene:508657APEX_IMS, mito protein domain, coexpression, MS/MS++IMSnonecerebrum, cerebellum, liver, adipose, placenta
PTPMT1protein ­tyrosine ­phosphatase ­mitochondrial ­1DUSP23, MOSP, PLIP, PNAS-129, Q8WUK0, gene:1149717literature, APEX_matrix, mito protein domain, coexpression, MS/MS++MIMMetabolism > Lipid metabolism > Cardiolipin synthesis;
Metabolism > Lipid metabolism > Phospholipid metabolism		all 14
PGAM5PGAM ­family ­member ­5, ­mitochondrial ­serine/threonine ­protein ­phosphataseBXLBV68, Q96HS1, gene:1921117APEX_IMS, mito protein domain, coexpression, MS/MS++MOMMitochondrial dynamics and surveillance > Mitophagy;
Mitochondrial dynamics and surveillance > Autophagy		cerebrum, cerebellum, brainstem, spinalcord, skeletalmuscle, adipose, largeintestine, placenta, testis
DTYMKdeoxythymidylate ­kinaseCDC8, PP3731, TMPK, TYMK, P23919, gene:18417Rickettsial homolog, MS/MS+MatrixMetabolism > Nucleotide metabolism > Nucleotide synthesis and processingcerebrum, cerebellum, brainstem, spinalcord, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
KARS1lysyl-tRNA ­synthetase ­1CMTRIB, DFNB89, KARS, KARS2, KRS, Q15046, gene:37357literature, GFP, targetP signal, Rickettsial homolog, mito protein domain, coexpressionMatrixMitochondrial central dogma > Translation > mt-tRNA synthetasescerebrum, cerebellum, brainstem, spinalcord, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
STARsteroidogenic ­acute ­regulatory ­proteinSTARD1, P49675, gene:67707literature, targetP signal, mito protein domain, coexpression, MS/MSMOM, IMSMetabolism > Lipid metabolism > Cholesterol, bile acid, steroid synthesistestis
DNAJC19DnaJ ­heat ­shock ­protein ­family ­(Hsp40) ­member ­C19PAM18, TIM14, TIMM14, Q96DA6, gene:1311187literature, APEX_matrix, mito protein domain, MS/MS++MIMProtein import, sorting and homeostasis > Protein import and sorting > Import motorall 14
OTCornithine ­carbamoyltransferaseOCTD, P00480, gene:50097literature, targetP signal, mito protein domain, MS/MS+MatrixMetabolism > Amino acid metabolism > Urea cyclecerebellum, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta
COX7A2Lcytochrome ­c ­oxidase ­subunit ­7A2 ­likeCOX7AR, COX7RP, EB1, SIG81, O14548, gene:91677literature, APEX_IMS, APEX_matrix, mito protein domain+, MS/MS+MIMOXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits;
OXPHOS > OXPHOS assembly factors;
OXPHOS > Respirasome assembly		all 14
MTERF3mitochondrial ­transcription ­termination ­factor ­3CGI-12, MTERFD1, Q96E29, gene:510017literature, APEX_matrix, GFP, targetP signal, mito protein domain+, coexpressionMatrixMitochondrial central dogma > Translation > Mitochondrial ribosome assemblynone
SFXN2sideroflexin ­2SLC56A2, Q96NB2, gene:1189807mito protein domain+, coexpressionMIMSmall molecule transport > Sideroflexinskidney, liver, adipose, largeintestine, stomach, placenta, testis
FASNfatty ­acid ­synthaseFAS, OA-519, SDR27X1, P49327, gene:21947Rickettsial homolog, mito protein domain, coexpressionIMSMetabolism > Lipid metabolismcerebrum, cerebellum, brainstem, spinalcord, liver, skeletalmuscle, adipose, smallintestine, stomach, placenta, testis
PINK1PTEN ­induced ­kinase ­1BRPK, PARK6, Q9BXM7, gene:650187literature, targetP signal+, mito protein domain, coexpressionMOMMitochondrial dynamics and surveillance > Mitophagy;
Mitochondrial dynamics and surveillance > Autophagy;
Signaling		none
TMEM177transmembrane ­protein ­177Q53S58, gene:807757targetP signal, coexpression, MS/MSMIMOXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors		cerebrum, adipose, stomach, placenta, testis
TXNRD1thioredoxin ­reductase ­1GRIM-12, TR, TR1, TRXR1, TXNR, Q16881, gene:72966GFP, Rickettsial homolog, mito protein domain, coexpression, MS/MSunknownMetabolism > Detoxification > ROS and glutathione metabolism;
Metabolism > Detoxification > Selenoproteins		skeletalmuscle, smallintestine, placenta, testis
THNSL1threonine ­synthase ­like ­1TSH1, Q8IYQ7, gene:798966APEX_matrix, mito protein domain, MS/MS++Matrixnonecerebrum, cerebellum, brainstem, spinalcord, skeletalmuscle, adipose, largeintestine, stomach, testis
FDPSfarnesyl ­diphosphate ­synthaseFPPS, FPS, POROK9, P14324, gene:22246targetP signal, mito protein domain, MS/MS+MIMMetabolism > Lipid metabolism > Cholesterol-associatedliver, adipose, smallintestine, largeintestine, placenta
IDI1isopentenyl-diphosphate ­delta ­isomerase ­1IPP1, IPPI1, Q13907, gene:34226targetP signal, mito protein domain, MS/MS+MatrixMetabolism > Lipid metabolism > Cholesterol-associatedcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
TOP3ADNA ­topoisomerase ­III ­alphaMGRISCE2, PEOB5, TOP3, ZGRF7, Q13472, gene:71566APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domainMatrixMitochondrial central dogma > mtDNA maintenance > mtDNA replicationtestis
FTMTferritin ­mitochondrialMTF, Q8N4E7, gene:940336literature, targetP signal+, mito protein domain, MS/MSMatrixMetabolism > Metals and cofactors > Iron homeostasistestis
LDHAL6Blactate ­dehydrogenase ­A ­like ­6BLDH6B, LDHAL6, LDHL, Q9BYZ2, gene:924836targetP signal, Rickettsial homolog, mito protein domain, MS/MSMatrixMetabolism > Carbohydrate metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
MTFR1mitochondrial ­fission ­regulator ­1CHPPR, FAM54A2, Q15390, gene:96506literature, targetP signal, mito protein domain+, coexpressionMembraneMitochondrial dynamics and surveillance > Fissionnone
BCL2L13BCL2 ­like ­13BCL-RAMBO, Bcl2-L-13, MIL1, Q9BXK5, gene:237866literature, mito protein domain, coexpression, MS/MS+MOMMitochondrial dynamics and surveillance > Mitophagy;
Mitochondrial dynamics and surveillance > Autophagy;
Mitochondrial dynamics and surveillance > Apoptosis		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
TRMT10CtRNA ­methyltransferase ­10C, ­mitochondrial ­RNase ­P ­subunitCOXPD30, HNYA, MRPP1, RG9MTD1, Q7L0Y3, gene:549316literature, APEX_matrix, GFP, targetP signal+, mito protein domain, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > Polycistronic mtRNA processing;
Mitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications		largeintestine, placenta
ACOT7acyl-CoA ­thioesterase ­7ACH1, ACT, BACH, CTE-II, LACH, LACH1, hBACH, O00154, gene:113326targetP signal+, mito protein domain, inductionMatrixMetabolism > Lipid metabolismbrainstem, spinalcord, adipose, largeintestine
TFB1Mtranscription ­factor ­B1, ­mitochondrialCGI-75, CGI75, mtTFB, mtTFB1, Q8WVM0, gene:511066literature, APEX_matrix, Rickettsial homolog, mito protein domain, MS/MS+MatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications;
Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly		brainstem, largeintestine, placenta, testis
ARF5ADP ­ribosylation ­factor ­5P84085, gene:3816targetP signal, mito protein domain, coexpression, MS/MSunknownnonecerebrum, cerebellum, brainstem, spinalcord, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
MCUBmitochondrial ­calcium ­uniporter ­dominant ­negative ­subunit ­betaCCDC109B, Q9NWR8, gene:550136literature, targetP signal, mito protein domain+MIMSignaling > Calcium homeostasis > Calcium cycle;
Small molecule transport > Calcium uniporter		placenta
TWNKtwinkle ­mtDNA ­helicaseATXN8, C10orf2, IOSCA, MTDPS7, PEO, PEO1, PEOA3, PRLTS5, SANDO, SCA8, TWINL, Q96RR1, gene:566526literature, APEX_matrix, targetP signal, induction, coexpressionMatrixMitochondrial central dogma > mtDNA maintenance > mtDNA replication;
Mitochondrial central dogma > mtDNA maintenance > mtDNA nucleoid		none
MMADHCmetabolism ­of ­cobalamin ­associated ­DC2orf25, CL25022, cblD, Q9H3L0, gene:272496APEX_matrix, targetP signal, induction, coexpressionMatrixMetabolism > Vitamin metabolism > Vitamin B12 metabolismnone
PYCR2pyrroline-5-carboxylate ­reductase ­2HLD10, P5CR2, Q96C36, gene:299206APEX_matrix, mito protein domain, MS/MS++MatrixMetabolism > Amino acid metabolism > Proline metabolismcerebrum, cerebellum, brainstem, spinalcord, adipose, largeintestine, stomach, placenta, testis
TSTD1thiosulfate ­sulfurtransferase ­like ­domain ­containing ­1KAT, TST, Q8NFU3, gene:1001311876Rickettsial homolog, mito protein domain, MS/MSunknownMetabolism > Sulfur metabolismkidney, smallintestine, largeintestine
LYRM1LYR ­motif ­containing ­1A211C6.1, O43325, gene:571496GFP, mito protein domain+, MS/MSunknownnonebrainstem, testis
OSBPL1Aoxysterol ­binding ­protein ­like ­1AORP-1, ORP1, OSBPL1B, Q9BXW6, gene:1148766Rickettsial homolog, mito protein domain, induction, MS/MSunknownMetabolism > Lipid metabolism > Cholesterol, bile acid, steroid synthesis;
Metabolism > Lipid metabolism > Phospholipid metabolism		cerebrum, liver, largeintestine, placenta
RECQL4RecQ ­like ­helicase ­4RECQ4, O94761, gene:94016targetP signal, Rickettsial homolog, mito protein domain, coexpressionMatrixMitochondrial central dogma > mtDNA maintenance > mtDNA repairnone
PET100PET100 ­cytochrome ­c ­oxidase ­chaperoneC19orf79, P0DJ07, gene:1001318016literature, yeast mito homolog++, MS/MSMIMOXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors		cerebrum, spinalcord, kidney, liver, heart, smallintestine, largeintestine, placenta
GDAP1ganglioside ­induced ­differentiation ­associated ­protein ­1CMT4, CMT4A, CMTRIA, Q8TB36, gene:543326APEX_IMS, mito protein domain, MS/MS++MOMnonecerebrum, cerebellum, brainstem, spinalcord
MTIF3mitochondrial ­translational ­initiation ­factor ­3IF3mt, Q9H2K0, gene:2194026literature, APEX_matrix, Rickettsial homolog, mito protein domain, coexpressionMatrixMitochondrial central dogma > Translation > Translation factorslargeintestine
UNGuracil ­DNA ­glycosylaseDGU, HIGM4, HIGM5, UDG, UNG1, UNG15, UNG2, P13051, gene:73746literature, targetP signal, yeast mito homolog, mito protein domain, inductionMatrixMitochondrial central dogma > mtDNA maintenance > mtDNA repairnone
GLYATglycine-N-acyltransferaseACGNAT, CAT, GAT, Q6IB77, gene:102496literature, mito protein domain, coexpression, MS/MS+MatrixMetabolism > Amino acid metabolism > Glycine metabolism;
Metabolism > Detoxification > Xenobiotic metabolism		spinalcord, kidney, liver, placenta
ISOC2isochorismatase ­domain ­containing ­2Q96AB3, gene:797636APEX_matrix, targetP signal, MS/MS++Matrixnoneall 14
ATP5MC1ATP ­synthase ­membrane ­subunit ­c ­locus ­1ATP5A, ATP5G, ATP5G1, P05496, gene:5166literature, yeast mito homolog+, Rickettsial homolog, mito protein domainMIMOXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits		none
QDPRquinoid ­dihydropteridine ­reductaseDHPR, HDHPR, PKU2, SDR33C1, P09417, gene:58606APEX_matrix, targetP signal, mito protein domain, inductionMatrixMetabolism > Metals and cofactors > Tetrahydrobiopterin synthesis;
Metabolism > Vitamin metabolism > Folate and 1-C metabolism		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, stomach, placenta, testis
COQ8Bcoenzyme ­Q8BADCK4, NPHS9, Q96D53, gene:799346APEX_matrix, GFP, yeast mito homolog+, Rickettsial homolog, mito protein domainMIMMetabolism > Metals and cofactors > Coenzyme Q metabolismadipose, smallintestine, largeintestine, placenta
GTPBP10GTP ­binding ­protein ­10ObgH2, UG0751c10, A4D1E9, gene:858656APEX_matrix, yeast mito homolog+, Rickettsial homolog, mito protein domainMatrixMitochondrial central dogma > Translation > Mitochondrial ribosome assemblylargeintestine, placenta
TSPOtranslocator ­proteinBPBS, BZRP, DBI, IBP, MBR, PBR, PBS, PKBS, PTBR, mDRC, pk18, P30536, gene:7066literature, mito protein domain, MS/MS++MOMMetabolism > Lipid metabolism > Cholesterol, bile acid, steroid synthesisspinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
FTH1ferritin ­heavy ­chain ­1FHC, FTH, FTHL6, HFE5, PIG15, PLIF, P02794, gene:24956mito protein domain, MS/MS++unknownMetabolism > Metals and cofactors > Iron homeostasiscerebrum, cerebellum, brainstem, spinalcord, liver, adipose, smallintestine, largeintestine, stomach, placenta
TRMT1tRNA ­methyltransferase ­1MRT68, TRM1, Q9NXH9, gene:556216targetP signal+, mito protein domain, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mt-tRNA modificationsnone
ADCK2aarF ­domain ­containing ­kinase ­2AARF, Q7Z695, gene:909566GFP, targetP signal, yeast mito homolog, Rickettsial homolog, mito protein domainMIMnonenone
TSTD3thiosulfate ­sulfurtransferase ­like ­domain ­containing ­3H0UI37, gene:1001308906mito protein domain, induction, MS/MSunknownnonecerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
MRPS36mitochondrial ­ribosomal ­protein ­S36DC47, MRP-S36, P82909, gene:922596literature, APEX_matrix, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosome;
Metabolism > Carbohydrate metabolism > TCA cycle		all 14
PDP2pyruvate ­dehyrogenase ­phosphatase ­catalytic ­subunit ­2PDPC 2, PPM2B, PPM2C2, Q9P2J9, gene:575466APEX_matrix, yeast mito homolog+, mito protein domain, coexpressionMatrixMetabolism > Carbohydrate metabolism > Pyruvate metabolismadipose
AGPAT51-acylglycerol-3-phosphate ­O-acyltransferase ­51AGPAT5, LPAATE, Q9NUQ2, gene:553266literature, Rickettsial homolog, mito protein domain, induction, MS/MSMOMMetabolism > Lipid metabolism > Cardiolipin synthesis;
Metabolism > Lipid metabolism > Phospholipid metabolism		cerebellum, brainstem, spinalcord, adipose, smallintestine, largeintestine, placenta, testis
MCRIP2MAPK ­regulated ­corepressor ­interacting ­protein ­2C16orf14, FAM195A, c349E10.1, Q9BUT9, gene:843315induction, coexpression+unknownSignalingnone
UCP2uncoupling ­protein ­2BMIQ4, SLC25A8, UCPH, P55851, gene:73515literature, yeast mito homolog+, mito protein domain+MIMSmall molecule transport > SLC25A familynone
SLC25A37solute ­carrier ­family ­25 ­member ­37HT015, MFRN, MFRN1, MSC, MSCP, PRO1278, PRO1584, PRO2217, Q9NYZ2, gene:513125literature, yeast mito homolog+, mito protein domain+MIMMetabolism > Metals and cofactors > Iron homeostasis;
Small molecule transport > SLC25A family		none
SLC25A28solute ­carrier ­family ­25 ­member ­28MFRN2, MRS3/4, MRS4L, NPD016, Q96A46, gene:818945literature, yeast mito homolog+, mito protein domain+MIMMetabolism > Metals and cofactors > Iron homeostasis;
Small molecule transport > SLC25A family		none
SLC25A48solute ­carrier ­family ­25 ­member ­48Q6ZT89, gene:1533285yeast mito homolog+, mito protein domain+MIMSmall molecule transport > SLC25A familynone
SLC25A43solute ­carrier ­family ­25 ­member ­43Q8WUT9, gene:2034275yeast mito homolog+, mito protein domain+MIMSmall molecule transport > SLC25A familynone
SLC25A47solute ­carrier ­family ­25 ­member ­47C14orf68, HDMCP, HMFN1655, Q6Q0C1, gene:2836005literature, yeast mito homolog+, mito protein domain+MIMSmall molecule transport > SLC25A familynone
SLC25A41solute ­carrier ­family ­25 ­member ­41APC4, Q8N5S1, gene:2844275yeast mito homolog+, mito protein domain+MIMMetabolism > Nucleotide metabolism > Nucleotide import;
Small molecule transport > SLC25A family		none
SLC25A34solute ­carrier ­family ­25 ­member ­34Q6PIV7, gene:2847235yeast mito homolog+, mito protein domain+MIMSmall molecule transport > SLC25A familynone
EPHX2epoxide ­hydrolase ­2ABHD20, CEH, SEH, P34913, gene:20535Rickettsial homolog, mito protein domain, coexpressionunknownMetabolism > Detoxification > Xenobiotic metabolism;
Metabolism > Lipid metabolism		kidney, liver, heart, skeletalmuscle, adipose, smallintestine, stomach
NUDT5nudix ­hydrolase ­5YSA1, YSA1H, YSAH1, hNUDT5, Q9UKK9, gene:111645yeast mito homolog++, mito protein domain, coexpressionunknownMetabolism > Nucleotide metabolism > Nucleotide synthesis and processingnone
MICU1mitochondrial ­calcium ­uptake ­1CALC, CBARA1, EFHA3, MPXPS, ara CALC, Q9BPX6, gene:103675literature, APEX_IMS, GFP, targetP signal, mito protein domain, MS/MS+MIMSignaling > Calcium homeostasis > Calcium cycle;
Signaling > Calcium homeostasis > EF hand proteins;
Small molecule transport > Calcium uniporter		cerebrum, cerebellum, brainstem, spinalcord, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
SOD1superoxide ­dismutase ­1ALS, ALS1, HEL-S-44, IPOA, SOD, STAHP, hSod1, homodimer, P00441, gene:66475literature, mito protein domain, induction, coexpressionIMSMetabolism > Detoxification > ROS and glutathione metabolismall 14
SERHL2serine ­hydrolase ­like ­2dJ222E13.1, Q9H4I8, gene:2531905Rickettsial homolog, mito protein domain, MS/MSunknownnonekidney, adipose, smallintestine, largeintestine, stomach, placenta, testis
NDUFAF3NADH:ubiquinone ­oxidoreductase ­complex ­assembly ­factor ­32P1, C3orf60, E3-3, MC1DN18, Q9BU61, gene:259155literature, targetP signal, mito protein domain, MS/MSMIMOXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors		all 14
BOLA3bolA ­family ­member ­3MMDS2, Q53S33, gene:3889625targetP signal, mito protein domain, MS/MSMatrixMetabolism > Metals and cofactors > Fe-S cluster biosynthesis;
Metabolism > Metals and cofactors > Fe-S-containing proteins		cerebrum, spinalcord, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
OCIAD2OCIA ­domain ­containing ­2Q56VL3, gene:1322995MS/MS++unknownSignalingall 14
RSAD1radical ­S-adenosyl ­methionine ­domain ­containing ­1HemW, Q9HA92, gene:553165APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, coexpressionMIMMetabolism > Metals and cofactors > Fe-S-containing proteinsnone
CHCHD6coiled-coil-helix-coiled-coil-helix ­domain ­containing ­6CHCM1, MICOS25, Mic25, PPP1R23, Q9BRQ6, gene:843035literature, APEX_IMS, APEX_matrix, coexpression, MS/MS++MIMMitochondrial dynamics and surveillance > Cristae formation > MICOS complexcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
FASTKD2FAST ­kinase ­domains ­2COXPD44, KIAA0971, Q9NYY8, gene:228685literature, APEX_matrix, induction, coexpression, MS/MSMatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > Polycistronic mtRNA processing;
Mitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay;
Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly		skeletalmuscle, adipose, smallintestine, largeintestine, placenta, testis
PEX11Bperoxisomal ­biogenesis ­factor ­11 ­betaPEX11-BETA, PEX14B, O96011, gene:87995targetP signal, coexpression, MS/MSMembranenonecerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, placenta, testis
TMEM186transmembrane ­protein ­186C16orf51, Q96B77, gene:258805targetP signal, coexpression, MS/MSMIMOXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors		cerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
PIF1PIF1 ­5'-to-3' ­DNA ­helicaseC15orf20, PIF, Q9H611, gene:801195literature, targetP signal+, yeast mito homolog, mito protein domainMatrixMitochondrial central dogma > mtDNA maintenancenone
FABP1fatty ­acid ­binding ­protein ­1FABPL, L-FABP, P07148, gene:21685mito protein domain, MS/MS++unknownMetabolism > Metals and cofactors > Heme synthesis and processing;
Metabolism > Lipid metabolism		liver, smallintestine
ABCD1ATP ­binding ­cassette ­subfamily ­D ­member ­1ABC42, ALD, ALDP, AMN, P33897, gene:2155Rickettsial homolog, mito protein domain, inductionMembraneSmall molecule transport > ABC transportersadipose
PLPBPpyridoxal ­phosphate ­binding ­proteinEPVB6D, PROSC, O94903, gene:112125APEX_matrix, coexpression, MS/MS++MatrixMetabolism > Vitamin metabolism > Vitamin B6 metabolismall 14
DBIdiazepam ­binding ­inhibitor, ­acyl-CoA ­binding ­proteinACBD1, ACBP, CCK-RP, EP, P07108, gene:16225mito protein domain, induction, coexpressionunknownMetabolism > Lipid metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
PNPT1polyribonucleotide ­nucleotidyltransferase ­1COXPD13, DFNB70, OLD35, PNPASE, old-35, Q8TCS8, gene:871785literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domainMatrix, IMSMitochondrial central dogma > mtRNA metabolism > mtRNA stability and decayall 14
TOP1MTDNA ­topoisomerase ­I ­mitochondrialQ969P6, gene:1164475literature, APEX_matrix, targetP signal, mito protein domain, coexpressionMatrixMitochondrial central dogma > mtDNA maintenance > mtDNA replication;
Mitochondrial central dogma > mtRNA metabolism > Transcription		none
RNASEH1ribonuclease ­H1H1RNA, PEOB2, RNH1, O60930, gene:2462435literature, APEX_matrix, targetP signal, Rickettsial homolog, coexpressionMatrixMitochondrial central dogma > mtDNA maintenance > mtDNA replication;
Mitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay		none
ABCA9ATP ­binding ­cassette ­subfamily ­A ­member ­9EST640918, Q8IUA7, gene:103505GFP, yeast mito homolog, Rickettsial homolog, mito protein domainMembraneSmall molecule transport > ABC transporterscerebrum, placenta
SCP2sterol ­carrier ­protein ­2NLTP, NSL-TP, SCP-2, SCP-CHI, SCP-X, SCPX, P22307, gene:63425Rickettsial homolog, mito protein domain+MIMMetabolism > Lipid metabolism > Cholesterol, bile acid, steroid synthesisbrainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, placenta, testis
NT5DC35'-nucleotidase ­domain ­containing ­3TU12B1-TY, TU12B1TY, Q86UY8, gene:515595APEX_matrix, targetP signal, MS/MS++Matrixnonecerebrum, cerebellum, brainstem, spinalcord, skeletalmuscle, stomach, placenta, testis
CMC4C-X9-C ­motif ­containing ­4C6.1B, MTCP1, MTCP1B, MTCP1NB, p8, p8MTCP1, P56277, gene:1002721475yeast mito homolog++, mito protein domain+IMSnonenone
LDHBlactate ­dehydrogenase ­BHEL-S-281, LDH-B, LDH-H, LDHBD, TRG-5, P07195, gene:39455Rickettsial homolog, mito protein domain, inductionunknownMetabolism > Amino acid metabolism > Glyoxylate metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
YBEYybeY ­metalloendoribonucleaseC21orf57, P58557, gene:540595APEX_matrix, targetP signal, Rickettsial homolog, MS/MSMatrixMitochondrial central dogma > mtRNA metabolism > Polycistronic mtRNA processing;
Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly		largeintestine, placenta, testis
HINT1histidine ­triad ­nucleotide ­binding ­protein ­1HINT, NMAN, PKCI-1, PRKCNH1, P49773, gene:30945Rickettsial homolog, mito protein domain, coexpressionunknownMetabolism > Nucleotide metabolism > Nucleotide synthesis and processingcerebrum, cerebellum, brainstem, spinalcord, kidney, smallintestine, placenta, testis
MUL1mitochondrial ­E3 ­ubiquitin ­protein ­ligase ­1C1orf166, GIDE, MAPL, MULAN, RNF218, Q969V5, gene:795945literature, mito protein domain+, inductionMOMProtein import, sorting and homeostasis > Protein homeostasis;
Mitochondrial dynamics and surveillance > Fission;
Signaling		skeletalmuscle, largeintestine
TK2thymidine ­kinase ­2MTDPS2, MTTK, PEOB3, SCA31, O00142, gene:70845literature, targetP signal, mito protein domain, MS/MSMatrixMetabolism > Nucleotide metabolism > Nucleotide synthesis and processingcerebrum, cerebellum, brainstem, adipose
AGMATagmatinaseQ9BSE5, gene:798145literature, targetP signal, mito protein domain, MS/MS+MatrixMetabolism > Amino acid metabolismliver, smallintestine
CMPK2cytidine/uridine ­monophosphate ­kinase ­2NDK, TMPK2, TYKi, UMP-CMPK2, Q5EBM0, gene:1296074literature, targetP signal+, MS/MSMatrixMetabolism > Nucleotide metabolism > Nucleotide synthesis and processingspinalcord, smallintestine
BIDBH3 ­interacting ­domain ­death ­agonistFP497, P55957, gene:6374literature, targetP signal+, mito protein domain, coexpressionMOMMitochondrial dynamics and surveillance > Apoptosisnone
ARL2ADP ­ribosylation ­factor ­like ­GTPase ­2ARFL2, P36404, gene:4024literature, mito protein domain, coexpression, MS/MS+IMSMitochondrial dynamics and surveillance > Fusioncerebrum, brainstem, spinalcord, adipose, largeintestine
SYNJ2BPsynaptojanin ­2 ­binding ­proteinARIP2, OMP25, P57105, gene:553334APEX_IMS, mito protein domain, coexpression, MS/MS+MOMMitochondrial dynamics and surveillance > Organelle contact sitescerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
MFFmitochondrial ­fission ­factorC2orf33, EMPF2, GL004, Q9GZY8, gene:569474literature, induction, MS/MSMOMMitochondrial dynamics and surveillance > Fissioncerebrum, cerebellum, brainstem, spinalcord, adipose, largeintestine, placenta, testis
DCXRdicarbonyl ­and ­L-xylulose ­reductaseDCR, HCR2, HCRII, KIDCR, P34H, PNTSU, SDR20C1, XR, Q7Z4W1, gene:511814APEX_matrix, targetP signal, Rickettsial homolog, mito protein domainMatrixMetabolism > Carbohydrate metabolismcerebrum, cerebellum, spinalcord, heart, adipose, largeintestine, stomach, placenta, testis
NUDT9nudix ­hydrolase ­9NUDT10, Q9BW91, gene:533434APEX_matrix, mito protein domain, coexpression, MS/MSMatrixMetabolism > Nucleotide metabolism > Nucleotide synthesis and processingcerebrum, cerebellum, placenta, testis
AMACRalpha-methylacyl-CoA ­racemaseAMACRD, CBAS4, P504S, RACE, RM, Q9UHK6, gene:236004literature, mito protein domain, coexpression, MS/MSMatrixMetabolism > Lipid metabolism > Fatty acid oxidationkidney, liver, adipose, smallintestine, testis
VPS13Dvacuolar ­protein ­sorting ­13 ­homolog ­DSCAR4, Q5THJ4, gene:551874manual, mito protein domain, induction, coexpressionMOMMitochondrial dynamics and surveillance > Mitophagy;
Mitochondrial dynamics and surveillance > Autophagy		cerebrum, cerebellum, brainstem, spinalcord, kidney, skeletalmuscle
NUDT6nudix ­hydrolase ­6ASFGF2, FGF-AS, FGF2AS, GFG-1, GFG1, P53370, gene:111624APEX_matrix, targetP signal, mito protein domain, coexpressionMatrixnonenone
DMAC2distal ­membrane ­arm ­assembly ­complex ­2ATP5SL, Q9NW81, gene:551014APEX_matrix, GFP, targetP signal, induction, MS/MSMIMOXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors		cerebrum, cerebellum, brainstem, stomach
MRM2mitochondrial ­rRNA ­methyltransferase ­2FJH1, FTSJ2, HEL97, MTDPS17, RRMJ2, Q9UI43, gene:299604literature, Rickettsial homolog, mito protein domain, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications;
Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly		cerebrum
BAK1BCL2 ­antagonist/killer ­1BAK, BAK-LIKE, BCL2L7, CDN1, Q16611, gene:5784literature, mito protein domain, MS/MS++MOMMitochondrial dynamics and surveillance > Apoptosisbrainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
DHODHdihydroorotate ­dehydrogenase ­(quinone)DHOdehase, POADS, URA1, Q02127, gene:17234literature, mito protein domain, MS/MS++MIMMetabolism > Nucleotide metabolism > Nucleotide synthesis and processing;
Metabolism > Electron carriers > Q-linked reactions, other		cerebrum, spinalcord, kidney, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
MAOBmonoamine ­oxidase ­BP27338, gene:41294literature, mito protein domain, MS/MS++MOMMetabolism > Amino acid metabolism > Catechol metabolism;
Metabolism > Detoxification > Xenobiotic metabolism		cerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
MGST3microsomal ­glutathione ­S-transferase ­3GST-III, O14880, gene:42594APEX_IMS, mito protein domain, MS/MS++MOMMetabolism > Lipid metabolism > Eicosanoid metabolismall 14
ACP6acid ­phosphatase ­6, ­lysophosphatidicACPL1, LPAP, PACPL1, Q9NPH0, gene:512054literature, APEX_IMS, mito protein domain, MS/MS++IMSMetabolism > Lipid metabolism > Phospholipid metabolismcerebrum, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
MRPL48mitochondrial ­ribosomal ­protein ­L48CGI-118, HSPC290, L48MT, MRP-L48, Q96GC5, gene:516424literature, APEX_matrix, mito protein domain, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
AGXTalanine--glyoxylate ­and ­serine--pyruvate ­aminotransferaseAGT, AGT1, AGXT1, PH1, SPAT, SPT, TLH6, P21549, gene:1894literature, yeast mito homolog, mito protein domain, MS/MSMatrixMetabolism > Amino acid metabolism > Serine metabolism;
Metabolism > Amino acid metabolism > Glyoxylate metabolism		liver
CYB5R3cytochrome ­b5 ­reductase ­3B5R, DIA1, P00387, gene:17274literature, mito protein domain, MS/MS+MOMMetabolism > Detoxification > Xenobiotic metabolism;
Metabolism > Detoxification > Amidoxime reducing complex;
Metabolism > Lipid metabolism		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
DDX28DEAD-box ­helicase ­28MDDX28, Q9NUL7, gene:557944literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domainMatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly		spinalcord, adipose
PNKDPNKD ­metallo-beta-lactamase ­domain ­containingBRP17, DYT8, FKSG19, FPD1, KIPP1184, MR-1, MR-1S, MR1, PDC, PKND1, PNKD1, R1, TAHCCP2, Q8N490, gene:259534literature, targetP signal, mito protein domain, MS/MSMIMOXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors		cerebrum, cerebellum, spinalcord, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
MICU3mitochondrial ­calcium ­uptake ­family ­member ­3EFHA2, Q86XE3, gene:2860974manual, targetP signal, mito protein domain, MS/MSMIMSignaling > Calcium homeostasis > Calcium cycle;
Signaling > Calcium homeostasis > EF hand proteins;
Small molecule transport > Calcium uniporter		cerebrum, cerebellum, brainstem, spinalcord, skeletalmuscle, stomach
BNIP3BCL2 ­interacting ­protein ­3NIP3, Q12983, gene:6644literature, mito protein domain+, MS/MSMOMMitochondrial dynamics and surveillance > Apoptosiscerebrum, cerebellum, brainstem, heart, skeletalmuscle, adipose
MTX3metaxin ­3Q5HYI7, gene:3457784manual, mito protein domain+, MS/MSMOMMitochondrial dynamics and surveillance > Intramitochondrial membrane interactionscerebrum, cerebellum, brainstem, spinalcord
MUTYHmutY ­DNA ­glycosylaseMYH, Q9UIF7, gene:45954GFP, targetP signal+, Rickettsial homolog, mito protein domainMatrixMitochondrial central dogma > mtDNA maintenancenone
GTPBP6GTP ­binding ­protein ­6 ­(putative)PGPL, O43824, gene:82254APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domainMatrixnonenone
CYP11A1cytochrome ­P450 ­family ­11 ­subfamily ­A ­member ­1CYP11A, CYPXIA1, P450SCC, P05108, gene:15834literature, mito protein domain, MS/MS+MIMMetabolism > Lipid metabolism > Cholesterol, bile acid, steroid synthesis;
Metabolism > Metals and cofactors > Heme-containing proteins		adipose, placenta, testis
OCIAD1OCIA ­domain ­containing ­1ASRIJ, OCIA, TPA018, Q9NX40, gene:549404APEX_IMS, GFP, MS/MS++MOMnoneall 14
METTL8methyltransferase ­like ­8TIP, Q9H825, gene:798284APEX_matrix, targetP signal, Rickettsial homolog, mito protein domainMatrixMitochondrial central dogma > mtRNA metabolismnone
SMIM20small ­integral ­membrane ­protein ­20C4orf52, MITRAC7, PNX, Q8N5G0, gene:3892034literature, coexpression, MS/MSMIMOXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, placenta, testis
RPUSD4RNA ­pseudouridine ­synthase ­D4Q96CM3, gene:848814APEX_matrix, GFP, Rickettsial homolog, mito protein domain, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications;
Mitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications		placenta
SMIM8small ­integral ­membrane ­protein ­8C6orf162, dJ102H19.2, Q96KF7, gene:571504manual, induction, coexpression, MS/MSMOMnonecerebrum, cerebellum, brainstem, spinalcord, kidney, adipose, placenta, testis
NME6NME/NM23 ­nucleoside ­diphosphate ­kinase ­6IPIA-ALPHA, NDK 6, NM23-H6, O75414, gene:102013literature, APEX_matrix, GFP, Rickettsial homolog, mito protein domain, coexpressionMatrixMetabolism > Nucleotide metabolism > Nucleotide synthesis and processinglargeintestine
FKBP8FKBP ­prolyl ­isomerase ­8FKBP38, FKBPr38, Q14318, gene:237703literature, mito protein domain, coexpression, MS/MSMOMMitochondrial dynamics and surveillance > Organelle contact sites;
Mitochondrial dynamics and surveillance > Mitophagy;
Mitochondrial dynamics and surveillance > Autophagy		cerebrum, brainstem, spinalcord, largeintestine, placenta, testis
ACSM4acyl-CoA ­synthetase ­medium ­chain ­family ­member ­4P0C7M7, gene:3413923manual, targetP signal, Rickettsial homolog, mito protein domainMatrixMetabolism > Lipid metabolism > Fatty acid oxidationnone
NLRX1NLR ­family ­member ­X1CLR11.3, DLNB26, NOD26, NOD5, NOD9, Q86UT6, gene:796713APEX_matrix, targetP signal, MS/MSMatrixSignaling > Immune responseall 14
FOXRED1FAD ­dependent ­oxidoreductase ­domain ­containing ­1FP634, H17, MC1DN19, Q96CU9, gene:555723literature, mito protein domain, coexpression+MIMOXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors		adipose, largeintestine, placenta, testis
TFB2Mtranscription ­factor ­B2, ­mitochondrialHkp1, mtTFB2, Q9H5Q4, gene:642163literature, APEX_matrix, targetP signal+, mito protein domainMatrixMitochondrial central dogma > mtDNA maintenance > mtDNA replication;
Mitochondrial central dogma > mtRNA metabolism > Transcription		placenta
PPM1Kprotein ­phosphatase, ­Mg2+/Mn2+ ­dependent ­1KBDP, MSUDMV, PP2Ckappa, PP2Cm, PTMP, UG0882E07, Q8N3J5, gene:1529263literature, targetP signal+, mito protein domainMatrixMetabolism > Amino acid metabolism > BCAA metabolism;
Metabolism > Amino acid metabolism > BCAA dehydrogenase complex		cerebellum, liver, heart, adipose
ATP5MC2ATP ­synthase ­membrane ­subunit ­c ­locus ­2ATP5A, ATP5G2, Q06055, gene:5173literature, yeast mito homolog+, Rickettsial homolog, mito protein domainMIMOXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits		none
MT-ND1NADH ­dehydrogenase, ­subunit ­1 ­(complex ­I)MTND1, P03886, gene:45353literature, Rickettsial homolog, mito protein domain, MS/MSMIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		all 14
TAZtafazzinBTHS, CMD3A, EFE, EFE2, G4.5, LVNCX, Taz1, Q16635, gene:69013literature, yeast mito homolog++, mito protein domainIMSMetabolism > Lipid metabolism > Cardiolipin synthesisnone
MSRB3methionine ­sulfoxide ­reductase ­B3DFNB74, Q8IXL7, gene:2538273literature, yeast mito homolog+, mito protein domainunknownMetabolism > Detoxification > ROS and glutathione metabolismnone
ACOT11acyl-CoA ­thioesterase ­11BFIT, STARD14, THEA, THEM1, Q8WXI4, gene:260273literature, targetP signal, mito protein domain, coexpressionMatrixMetabolism > Lipid metabolism > Fatty acid oxidationnone
NIPSNAP3Bnipsnap ­homolog ­3BFP944, NIPSNAP3, SNAP1, Q9BS92, gene:553353GFP, targetP signal, MS/MS+Matrixnonecerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
NTHL1nth ­like ­DNA ­glycosylase ­1FAP3, NTH1, OCTS3, hNTH1, P78549, gene:49133literature, yeast mito homolog, Rickettsial homolog, mito protein domainunknownMetabolism > Metals and cofactors > Fe-S-containing proteinsnone
MARCHF5membrane ­associated ­ring-CH-type ­finger ­5MARCH-V, MARCH5, MITOL, RNF153, Q9NX47, gene:547083literature, mito protein domain, coexpression, MS/MSMOMMitochondrial dynamics and surveillance > Mitophagy;
Mitochondrial dynamics and surveillance > Autophagy		cerebrum, cerebellum, brainstem, spinalcord, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
SFXN4sideroflexin ­4BCRM1, COXPD18, SLC56A4, Q6P4A7, gene:1195593literature, mito protein domain+MIMSmall molecule transport > Sideroflexinscerebellum, spinalcord
TRMT5tRNA ­methyltransferase ­5COXPD26, KIAA1393, TRM5, Q32P41, gene:575703literature, yeast mito homolog, mito protein domain, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mt-tRNA modificationsnone
SLC25A6solute ­carrier ­family ­25 ­member ­6AAC3, ANT, ANT 2, ANT 3, ANT3, ANT3Y, P12236, gene:2933literature, yeast mito homolog+, mito protein domain+MIMMetabolism > Nucleotide metabolism > Nucleotide import;
Signaling > Calcium homeostasis > Mitochondrial permeability transition pore;
Small molecule transport > SLC25A family		none
SLC25A52solute ­carrier ­family ­25 ­member ­52MCART2, Q3SY17, gene:1474073manual, yeast mito homolog+, mito protein domain+MIMMetabolism > Metals and cofactors > NAD biosynthesis and metabolism;
Small molecule transport > SLC25A family		none
OGG18-oxoguanine ­DNA ­glycosylaseHMMH, HOGG1, MUTM, OGH1, O15527, gene:49683literature, targetP signal, yeast mito homolog, mito protein domainMatrixMitochondrial central dogma > mtDNA maintenance > mtDNA repairnone
ACOD1aconitate ­decarboxylase ­1CAD, IRG1, A6NK06, gene:7302493literature, yeast mito homolog, mito protein domain+MatrixMetabolism > Carbohydrate metabolism > Itaconate metabolism;
Signaling > Immune response		none
QTRT1queuine ­tRNA-ribosyltransferase ­catalytic ­subunit ­1FP3235, TGT, TGUT, Q9BXR0, gene:818903APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mt-tRNA modificationsnone
AIFM2apoptosis ­inducing ­factor ­mitochondria ­associated ­2AMID, FSP1, PRG3, Q9BRQ8, gene:848833literature, Rickettsial homolog, mito protein domain, coexpressionMembraneMetabolism > Detoxification > ROS and glutathione metabolism;
Mitochondrial dynamics and surveillance > Apoptosis		none
PRDX2peroxiredoxin ­2HEL-S-2a, NKEF-B, NKEFB, PRP, PRX2, PRXII, PTX1, TDPX1, TPX1, TSA, P32119, gene:70013GFP, Rickettsial homolog, mito protein domain, coexpressionunknownMetabolism > Detoxification > ROS and glutathione metabolismcerebrum, cerebellum, brainstem, spinalcord, adipose, smallintestine, stomach, placenta, testis
FBXL4F-box ­and ­leucine ­rich ­repeat ­protein ­4FBL4, FBL5, MTDPS13, Q9UKA2, gene:262352literature, mito protein domain, coexpressionIMSnoneadipose
GPAMglycerol-3-phosphate ­acyltransferase, ­mitochondrialGPAT, GPAT1, Q9HCL2, gene:576782literature, mito protein domain, coexpressionMembraneMetabolism > Lipid metabolism > Cardiolipin synthesis;
Metabolism > Lipid metabolism > Phospholipid metabolism		cerebellum, spinalcord, kidney, liver, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
DMAC2Ldistal ­membrane ­arm ­assembly ­complex ­2 ­likeATP5S, ATPW, FB, HSU79253, Q99766, gene:271092literature, APEX_matrix, MS/MS++MIMOXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
RMDN3regulator ­of ­microtubule ­dynamics ­3FAM82A2, FAM82C, RMD-3, RMD3, ptpip51, Q96TC7, gene:551772literature, APEX_IMS, GFP, MS/MS++MOMMitochondrial dynamics and surveillance > Organelle contact sitescerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, largeintestine, stomach, placenta, testis
PRXL2Aperoxiredoxin ­like ­2AAdrx, C10orf58, FAM213A, PAMM, Q9BRX8, gene:842932GFP, MS/MS++MembraneMetabolism > Detoxification > ROS and glutathione metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, placenta, testis
ATP5MDATP ­synthase ­membrane ­subunit ­DAPITDAPIT, HCVFTP2, MC5DN6, USMG5, bA792D24.4, Q96IX5, gene:848332GFP, MS/MS++MIMOXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits;
Mitochondrial dynamics and surveillance > Cristae formation		all 14
PET117PET117 ­cytochrome ­c ­oxidase ­chaperoneCSRP2BP, Q6UWS5, gene:1003037552literature, yeast mito homolog, MS/MSMIMOXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors		cerebrum, cerebellum, liver, adipose, largeintestine, stomach, placenta
MAOAmonoamine ­oxidase ­ABRNRS, MAO-A, P21397, gene:41282literature, mito protein domain, MS/MS+MOMMetabolism > Amino acid metabolism > Catechol metabolism;
Metabolism > Detoxification > Xenobiotic metabolism		all 14
C15orf61chromosome ­15 ­open ­reading ­frame ­61A6NNL5, gene:1458532APEX_matrix, targetP signal, MS/MSMatrixnonebrainstem, spinalcord, skeletalmuscle, adipose, largeintestine, stomach, testis
POLQDNA ­polymerase ­thetaPRO0327, O75417, gene:107212literature, targetP signal, Rickettsial homolog, mito protein domainMatrixMitochondrial central dogma > mtDNA maintenancebrainstem, testis
BCO2beta-carotene ­oxygenase ­2B-DIOX-II, BCDO2, Q9BYV7, gene:838752literature, targetP signal, mito protein domain, MS/MSMIMMetabolism > Vitamin metabolism > Vitamin A metabolismsmallintestine
IFI27interferon ­alpha ­inducible ­protein ­27FAM14D, ISG12, ISG12A, P27, P40305, gene:34292literature, induction, coexpressionMembraneSignaling > Immune response;
Mitochondrial dynamics and surveillance > Apoptosis		none
CRYZcrystallin ­zetaQ08257, gene:14292APEX_matrix, mito protein domain, MS/MSMatrixnonespinalcord, kidney, liver, adipose, placenta, testis
PYCR1pyrroline-5-carboxylate ­reductase ­1ARCL2B, ARCL3B, P5C, P5CR, PIG45, PP222, PRO3, PYCR, P32322, gene:58312literature, mito protein domain, MS/MSMatrixMetabolism > Amino acid metabolism > Proline metabolismcerebrum, cerebellum, brainstem, spinalcord, adipose, largeintestine, stomach, placenta, testis
TMLHEtrimethyllysine ­hydroxylase, ­epsilonAUTSX6, BBOX2, TMLD, TMLH, TMLHED, XAP130, Q9NVH6, gene:552172literature, APEX_matrix, mito protein domain, MS/MSMatrixMetabolism > Metals and cofactors > Carnitine synthesis and transportkidney, adipose, smallintestine, largeintestine, stomach, placenta, testis
TOMM20translocase ­of ­outer ­mitochondrial ­membrane ­20MAS20, MOM19, TOM20, Q15388, gene:98042literature, yeast mito homolog+MOMProtein import, sorting and homeostasis > Protein import and sorting > TOM;
Mitochondrial dynamics and surveillance		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
YRDCyrdC ­N6-threonylcarbamoyltransferase ­domain ­containingDRIP3, IRIP, SUA5, Q86U90, gene:796932manual, targetP signal+, Rickettsial homologMatrixMitochondrial central dogma > mtRNA metabolism > mt-tRNA modificationsnone
COA7cytochrome ­c ­oxidase ­assembly ­factor ­7 ­(putative)C1orf163, RESA1, SCAN3, SELRC1, Q96BR5, gene:652602literature, APEX_IMS, mito protein domain, coexpression, MS/MSIMSOXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
DHRS2dehydrogenase/reductase ­2HEP27, SDR25C1, Q13268, gene:102022literature, targetP signal, Rickettsial homolog, mito protein domainMatrixMetabolism > Detoxification > Xenobiotic metabolismnone
MMAAmetabolism ­of ­cobalamin ­associated ­AcblA, Q8IVH4, gene:1667852manual, coexpression+MatrixMetabolism > Vitamin metabolism > Vitamin B12 metabolismcerebrum, cerebellum, brainstem, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
MIEF1mitochondrial ­elongation ­factor ­1AltMIEF1, HSU79252, MID51, MIEF1-MP, SMCR7L, dJ1104E15.3, Q9NQG6, gene:544712literature, mito protein domain, induction, coexpressionMOM, MatrixMitochondrial central dogma > Translation > Mitochondrial ribosome assembly;
Mitochondrial dynamics and surveillance > Fission		none
C15orf48chromosome ­15 ­open ­reading ­frame ­48COXFA4L3, FOAP-11, MIR147BHG, NMES1, Q9C002, gene:844191GFP, mito protein domain, MS/MS+Membranenonesmallintestine, largeintestine, stomach, placenta, testis
LIG3DNA ­ligase ­3LIG2, LIG3alpha, P49916, gene:39801literature, targetP signal, mito protein domain, coexpressionMatrixMitochondrial central dogma > mtDNA maintenance > mtDNA replication;
Mitochondrial central dogma > mtDNA maintenance > mtDNA repair		none
BNIP3LBCL2 ­interacting ­protein ­3 ­likeBNIP3a, NIX, O60238, gene:6651literature, mito protein domain+, coexpressionMOMMitochondrial dynamics and surveillance > Apoptosisnone
FASTKD5FAST ­kinase ­domains ­5dJ1187M17.5, Q7L8L6, gene:604931APEX_matrix, targetP signal, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > Polycistronic mtRNA processing;
Mitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay		skeletalmuscle, largeintestine, placenta
PARK7Parkinsonism ­associated ­deglycaseDJ-1, DJ1, GATD2, HEL-S-67p, Q99497, gene:113151literature, mito protein domain, coexpressionMOMProtein import, sorting and homeostasis > Protein homeostasis;
Mitochondrial dynamics and surveillance > Mitophagy;
Mitochondrial dynamics and surveillance > Autophagy		all 14
COA1cytochrome ­c ­oxidase ­assembly ­factor ­1 ­homologC7orf44, MITRAC15, Q9GZY4, gene:557441APEX_IMS, targetP signal, mito protein domain+MIMOXPHOS > Complex I > CI assembly factors;
OXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors		none
COX7B2cytochrome ­c ­oxidase ­subunit ­7B2Q8TF08, gene:1707121literature, targetP signal, mito protein domain+MIMOXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits		none
MT-ND4LNADH ­dehydrogenase, ­subunit ­4L ­(complex ­I)MTND4L, P03901, gene:45391literature, Rickettsial homolog, mito protein domainMIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		heart, skeletalmuscle
SIRT4sirtuin ­4SIR2L4, Q9Y6E7, gene:234091literature, targetP signal, mito protein domainMatrixMetabolism > Metals and cofactors > NAD biosynthesis and metabolismlargeintestine
UQCC2ubiquinol-cytochrome ­c ­reductase ­complex ­assembly ­factor ­2C6orf125, C6orf126, Cbp6, M19, MC3DN7, MNF1, bA6B20.2, Q9BRT2, gene:843001APEX_matrix, GFP, coexpression, MS/MSMIMOXPHOS > Complex III > CIII assembly factors;
OXPHOS > OXPHOS assembly factors		all 14
USP30ubiquitin ­specific ­peptidase ­30Q70CQ3, gene:847491literature, targetP signal, mito protein domainMOMProtein import, sorting and homeostasis > Protein homeostasis > Proteases;
Mitochondrial dynamics and surveillance > Mitophagy;
Mitochondrial dynamics and surveillance > Autophagy		adipose, placenta, testis
PDE12phosphodiesterase ­122'-PDE, 2-PDE, Q6L8Q7, gene:2016261APEX_matrix, targetP signal, mito protein domainMatrixMitochondrial central dogma > mtRNA metabolism > mt-mRNA modifications;
Mitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay		brainstem, smallintestine, largeintestine, testis
NME3NME/NM23 ­nucleoside ­diphosphate ­kinase ­3DR-nm23, NDPK-C, NDPKC, NM23-H3, NM23H3, c371H6.2, Q13232, gene:48321GFP, Rickettsial homolog, mito protein domainMOMMetabolism > Nucleotide metabolism > Nucleotide synthesis and processingcerebrum, cerebellum, brainstem, kidney, adipose, placenta, testis
DHX30DExH-box ­helicase ­30DDX30, NEDMIAL, RETCOR, Q7L2E3, gene:229071literature, mito protein domainMatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly		cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
FASTKFas ­activated ­serine/threonine ­kinaseFAST, Q14296, gene:109221GFP, induction, coexpressionMIMMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > Polycistronic mtRNA processing;
Mitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay		none
METTL15methyltransferase ­like ­15METT5D1, A6NJ78, gene:1960741APEX_matrix, Rickettsial homolog, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mt-rRNA modificationslargeintestine, placenta
CYP24A1cytochrome ­P450 ­family ­24 ­subfamily ­A ­member ­1CP24, CYP24, HCAI, HCINF1, P450-CC24, Q07973, gene:15911literature, targetP signal+, mito protein domainMIMMetabolism > Metals and cofactors > Heme-containing proteins;
Metabolism > Vitamin metabolism > Vitamin D metabolism		none
NOCTnocturninCCR4L, CCRN4L, Ccr4c, NOC, Q9UK39, gene:258191literature, targetP signal+, mito protein domainMatrixMetabolism > Metals and cofactors > NAD biosynthesis and metabolismnone
PANK2pantothenate ­kinase ­2C20orf48, HARP, HSS, NBIA1, PKAN, Q9BZ23, gene:800251literature, targetP signal+, mito protein domainIMSMetabolism > Metals and cofactors > Coenzyme A metabolismnone
CA5Acarbonic ­anhydrase ­5ACA5, CA5AD, CAV, CAVA, GS1-21A4.1, P35218, gene:7631literature, mito protein domain, MS/MSMatrixMetabolism > Amino acid metabolism > Urea cycleliver
NT5DC25'-nucleotidase ­domain ­containing ­2Q9H857, gene:649431APEX_matrix, targetP signal, MS/MSMatrixMetabolism > Nucleotide metabolism > Nucleotide synthesis and processingspinalcord, largeintestine, placenta
ATAD3BATPase ­family ­AAA ­domain ­containing ­3BAAA-TOB3, TOB3, Q5T9A4, gene:838581APEX_IMS, APEX_matrix, GFP, Rickettsial homolog, mito protein domain+MIMMitochondrial central dogma > mtDNA maintenance > mtDNA nucleoid;
Mitochondrial dynamics and surveillance > Organelle contact sites;
Mitochondrial dynamics and surveillance > Intramitochondrial membrane interactions		none
TRUB2TruB ­pseudouridine ­synthase ­family ­member ­2CLONE24922, O95900, gene:269951APEX_matrix, Rickettsial homolog, mito protein domainMatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications		none
PRKACAprotein ­kinase ­cAMP-activated ­catalytic ­subunit ­alphaPKACA, PPNAD4, P17612, gene:55660literature, mito protein domain, coexpressionMIMSignaling > cAMP-PKA signalingcerebrum, brainstem, spinalcord, adipose, testis
COMTD1catechol-O-methyltransferase ­domain ­containing ­1Q86VU5, gene:1188810GFP, MS/MS++Membranenoneall 14
CPT1Ccarnitine ­palmitoyltransferase ­1CCATL1, CPT1-B, CPT1P, CPTI-B, CPTIC, SPG73, Q8TCG5, gene:1261290literature, yeast mito homolog+, mito protein domainMOMMetabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Metals and cofactors > Carnitine synthesis and transport;
Metabolism > Metals and cofactors > Carnitine shuttle		none
C3orf33chromosome ­3 ­open ­reading ­frame ­33AC3-33, Q6P1S2, gene:2853150APEX_matrix, targetP signal, MS/MSMatrixnoneadipose, testis
MIGA2mitoguardin ­2C9orf54, FAM73B, Q7L4E1, gene:848950literature, coexpressionMOMMitochondrial dynamics and surveillance > Fusion;
Mitochondrial dynamics and surveillance > Organelle contact sites		cerebrum, cerebellum, brainstem, spinalcord, liver, testis
SND1staphylococcal ­nuclease ­and ­tudor ­domain ­containing ­1TDRD11, Tudor-SN, p100, Q7KZF4, gene:270440GFP, yeast mito homolog, mito protein domainunknownnonecerebrum, cerebellum, brainstem, liver, smallintestine, placenta, testis
GLUD2glutamate ­dehydrogenase ­2GDH2, GLUDP1, P49448, gene:27470literature, targetP signal+, mito protein domainMatrixMetabolism > Amino acid metabolism > Glutamate metabolism;
Metabolism > Amino acid metabolism > GABA metabolism		none
HPDL4-hydroxyphenylpyruvate ­dioxygenase ­like4-HPPD-L, GLOXD1, Q96IR7, gene:848420manual, targetP signal, inductionunknownnonenone
ANGEL2angel ­homolog ­2Ccr4d, KIAA0759L, Q5VTE6, gene:908060APEX_matrix, mito protein domain, MS/MSMatrixMitochondrial central dogma > mtRNA metabolism > mt-mRNA modificationsadipose, smallintestine, placenta, testis
ANTKMTadenine ­nucleotide ­translocase ­lysine ­methyltransferaseANT-KMT, C16orf24, FAM173A, Q9BQD7, gene:659900literature, coexpression, MS/MSMIMMetabolism > Nucleotide metabolismcerebrum, cerebellum, brainstem, spinalcord, adipose, largeintestine, placenta
UQCC3ubiquinol-cytochrome ­c ­reductase ­complex ­assembly ­factor ­3C11orf83, CCDS41658.1, MC3DN9, UNQ655, Q6UW78, gene:7909550literature, coexpression, MS/MSMIMOXPHOS > Complex III > CIII assembly factors;
OXPHOS > OXPHOS assembly factors		liver, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
PRDX4peroxiredoxin ­4AOE37-2, AOE372, HEL-S-97n, PRX-4, Q13162, gene:105490APEX_IMS, Rickettsial homolog, mito protein domainIMSMetabolism > Detoxification > ROS and glutathione metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
CDK5RAP1CDK5 ­regulatory ­subunit ­associated ­protein ­1C20orf34, C42, CGI-05, HSPC167, Q96SZ6, gene:516540literature, targetP signal, Rickettsial homolog, mito protein domainMatrixMitochondrial central dogma > mtRNA metabolism > mt-tRNA modificationsnone
PAICSphosphoribosylaminoimidazole ­carboxylase ­and ­phosphoribosylaminoimidazolesuccinocarboxamide ­synthaseADE2, ADE2H1, AIRC, PAIS, P22234, gene:106060APEX_matrix, Rickettsial homolog, coexpressionMatrixMetabolism > Nucleotide metabolism > Nucleotide synthesis and processingnone
MGARPmitochondria ­localized ­glutamic ­acid ­rich ­proteinC4orf49, CESP-1, HUMMR, OSAP, Q8TDB4, gene:847090literature, APEX_IMS, targetP signal, coexpressionMembraneMitochondrial dynamics and surveillance > Traffickingnone
HDHD3haloacid ­dehalogenase ­like ­hydrolase ­domain ­containing ­32810435D12Rik, C9orf158, Q9BSH5, gene:819320APEX_matrix, MS/MS++Matrixnonecerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
CYP11B2cytochrome ­P450 ­family ­11 ­subfamily ­B ­member ­2ALDOS, CPN2, CYP11B, CYP11BL, CYPXIB2, P-450C18, P450C18, P450aldo, P19099, gene:15850literature, targetP signal, mito protein domain, coexpressionMIMMetabolism > Lipid metabolism > Cholesterol, bile acid, steroid synthesis;
Metabolism > Metals and cofactors > Heme-containing proteins		none
AGPAT41-acylglycerol-3-phosphate ­O-acyltransferase ­41-AGPAT4, LPAAT-delta, dJ473J16.2, Q9NRZ5, gene:568950literature, mito protein domain, inductionMOMMetabolism > Lipid metabolism > Cardiolipin synthesis;
Metabolism > Lipid metabolism > Phospholipid metabolism		testis
RCC1LRCC1 ­likeWBSCR16, Q96I51, gene:81554-0literature, APEX_matrix, targetP signal, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mt-rRNA modificationsplacenta
FAM185Afamily ­with ­sequence ­similarity ­185 ­member ­AQ8N0U4, gene:222234-0APEX_matrix, targetP signal, coexpressionMatrixnonenone
PMAIP1phorbol-12-myristate-13-acetate-induced ­protein ­1APR, NOXA, Q13794, gene:5366-0literature, mito protein domain+MOMMitochondrial dynamics and surveillance > Apoptosisnone
TDRKHtudor ­and ­KH ­domain ­containingTDRD2, Q9Y2W6, gene:11022-0GFP, mito protein domain, MS/MSMembranenonecerebrum, cerebellum, brainstem, spinalcord, adipose, testis
SLC8B1solute ­carrier ­family ­8 ­member ­B1NCKX6, NCLX, SLC24A6, Q6J4K2, gene:80024-0literature, mito protein domain, inductionMIMSignaling > Calcium homeostasis > Calcium cycle;
Small molecule transport		placenta
CYP27B1cytochrome ­P450 ­family ­27 ­subfamily ­B ­member ­1CP2B, CYP1, CYP1alpha, CYP27B, P450c1, PDDR, VDD1, VDDR, VDDRI, VDR, O15528, gene:1594-1literature, targetP signal, mito protein domainMIMMetabolism > Metals and cofactors > Heme-containing proteins;
Metabolism > Vitamin metabolism > Vitamin D metabolism		none
POLG2DNA ­polymerase ­gamma ­2, ­accessory ­subunitHP55, MTDPS16, MTPOLB, PEOA4, POLB, POLG-BETA, POLGB, Q9UHN1, gene:11232-1literature, APEX_matrix, targetP signal, mito protein domainMatrixMitochondrial central dogma > mtDNA maintenance > mtDNA replication;
Mitochondrial central dogma > mtDNA maintenance > mtDNA nucleoid		none
THEM5thioesterase ­superfamily ­member ­5ACOT15, Q8N1Q8, gene:284486-1literature, targetP signal, mito protein domainMatrixMetabolism > Lipid metabolismnone
BIKBCL2 ­interacting ­killerBIP1, BP4, NBK, Q13323, gene:638-1manual, mito protein domain+MOMMitochondrial dynamics and surveillance > Apoptosisnone
MTERF1mitochondrial ­transcription ­termination ­factor ­1MTERF, Q99551, gene:7978-1literature, APEX_matrix, mito protein domain+MatrixMitochondrial central dogma > mtDNA maintenance > mtDNA nucleoid;
Mitochondrial central dogma > mtRNA metabolism > Transcription		none
MTFR2mitochondrial ­fission ­regulator ­2DUFD1, FAM54A, Q6P444, gene:113115-1manual, mito protein domain+MembraneMitochondrial dynamics and surveillance > Fissionnone
GPX4glutathione ­peroxidase ­4GPx-4, GSHPx-4, MCSP, PHGPx, SMDS, snGPx, snPHGPx, P36969, gene:2879-1literature, mito protein domainMIMMetabolism > Detoxification > ROS and glutathione metabolism;
Metabolism > Detoxification > Selenoproteins		all 14
TOMM20Ltranslocase ­of ­outer ­mitochondrial ­membrane ­20 ­likeUNQ9438, Q6UXN7, gene:387990-1manual, yeast mito homolog+MOMProtein import, sorting and homeostasis > Protein import and sortingnone
COMTcatechol-O-methyltransferaseHEL-S-98n, P21964, gene:1312-1GFP, induction, coexpressionMembraneMetabolism > Amino acid metabolism > Catechol metabolismliver
MYG1MYG1 ­exonucleaseC12orf10, Gamm1, MST024, MSTP024, MYG, Q86UA3, gene:60314-1literature, mito protein domain, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications;
Mitochondrial central dogma > mtRNA metabolism > mt-mRNA modifications		none
SPATA20spermatogenesis ­associated ­20HEL-S-98, SSP411, Tisp78, Q8TB22, gene:64847-1APEX_matrix, targetP signalMatrixnonetestis
OXCT23-oxoacid ­CoA-transferase ­2FKSG25, SCOTT, Q9BYC2, gene:64064-1manual, targetP signal+, mito protein domainMatrixMetabolism > Carbohydrate metabolism > Ketone metabolismnone
CCDC127coiled-coil ­domain ­containing ­127Q96BQ5, gene:133957-1APEX_IMS, MS/MSMembranenonecerebrum, cerebellum, brainstem, spinalcord, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
CRY1cryptochrome ­circadian ­regulator ­1DSPD, PHLL1, Q16526, gene:1407-1literature, mito protein domain, coexpressionunknownnonenone
ACSM2Bacyl-CoA ­synthetase ­medium ­chain ­family ­member ­2BACSM2, HXMA, HYST1046, Q68CK6, gene:348158-1manual, targetP signal, mito protein domainMatrixMetabolism > Lipid metabolism > Fatty acid oxidationnone
MYO19myosin ­XIXMYOHD1, Q96H55, gene:80179-1literature, mito protein domainMOMMitochondrial dynamics and surveillance > Traffickingadipose
MGME1mitochondrial ­genome ­maintenance ­exonuclease ­1C20orf72, DDK1, MTDPS11, bA504H3.4, Q9BQP7, gene:92667-2literature, APEX_matrix, GFP, targetP signalMatrixMitochondrial central dogma > mtDNA maintenance > mtDNA replication;
Mitochondrial central dogma > mtDNA maintenance > mtDNA stability and decay		largeintestine, placenta
SEPTIN4septin ­4ARTS, BRADEION, C17orf47, CE5B3, H5, MART, PNUTL2, SEP4, SEPT4, hCDCREL-2, hucep-7, O43236, gene:5414-2literature, mito protein domainMOMMitochondrial dynamics and surveillance > Apoptosisbrainstem, spinalcord
EFHD1EF-hand ­domain ­family ­member ­D1MST133, MSTP133, PP3051, SWS2, Q9BUP0, gene:80303-2literature, mito protein domain, inductionMIMSignaling > Calcium homeostasis > EF hand proteinsnone
RAB5IFRAB5 ­interacting ­factorC20orf24, PNAS-11, RCAF1, RIP5, Q9BUV8, gene:55969-2GFP, induction, coexpressionMIMOXPHOS > OXPHOS assembly factors;
OXPHOS > Respirasome assembly		none
APEX1apurinic/apyrimidinic ­endodeoxyribonuclease ­1APE, APE1, APEN, APEX, APX, HAP1, REF1, P27695, gene:328-2literature, Rickettsial homolog, mito protein domainMatrixMitochondrial central dogma > mtDNA maintenance > mtDNA repairnone
SPTLC2serine ­palmitoyltransferase ­long ­chain ­base ­subunit ­2HSN1C, LCB2, LCB2A, NSAN1C, SPT2, hLCB2a, O15270, gene:9517-2GFP, Rickettsial homolog, mito protein domainMembraneMetabolism > Lipid metabolism > Phospholipid metabolismnone
DMAC1distal ­membrane ­arm ­assembly ­complex ­1C9orf123, TMEM261, Q96GE9, gene:90871-2literature, MS/MSMIMOXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors		cerebrum, spinalcord, kidney, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
ATPSCKMTATP ­synthase ­c ­subunit ­lysine ­N-methyltransferaseFAM173B, JS-2, hFAM173B, Q6P4H8, gene:134145-2literature, coexpressionMIMOXPHOS > Complex V > CV assembly factors;
OXPHOS > OXPHOS assembly factors		placenta
NEU4neuraminidase ­4Q8WWR8, gene:129807-2GFP, targetP signalMOMMetabolism > Carbohydrate metabolismnone
CASP9caspase ­9APAF-3, APAF3, ICE-LAP6, MCH6, PPP1R56, P55211, gene:842-2literature, targetP signal, mito protein domainIMSProtein import, sorting and homeostasis > Protein homeostasis > Proteases;
Mitochondrial dynamics and surveillance > Apoptosis		none
AGKacylglycerol ­kinaseCATC5, CTRCT38, MTDPS10, MULK, Q53H12, gene:55750-2literature, APEX_IMS, mito protein domainMIMProtein import, sorting and homeostasis > Protein import and sorting > TIM22 carrier pathway;
Metabolism > Lipid metabolism > Phospholipid metabolism		cerebrum, cerebellum, brainstem, spinalcord, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
MCL1MCL1 ­apoptosis ­regulator, ­BCL2 ­family ­memberBCL2L3, EAT, MCL1-ES, MCL1L, MCL1S, Mcl-1, TM, bcl2-L-3, mcl1/EAT, Q07820, gene:4170-3manual, mito protein domainMOMMitochondrial dynamics and surveillance > Apoptosisadipose, largeintestine, placenta, testis
DUS2dihydrouridine ­synthase ­2DUS2L, SMM1, URLC8, Q9NX74, gene:54920-3GFP, Rickettsial homolog, mito protein domainMatrixMitochondrial central dogma > mtRNA metabolism > mt-tRNA modificationsnone
PLD6phospholipase ­D ­family ­member ­6ZUC, Q8N2A8, gene:201164-3literature, Rickettsial homolog, mito protein domainMOMMetabolism > Lipid metabolism > Cardiolipin synthesis;
Mitochondrial dynamics and surveillance > Fusion		none
TOMM34translocase ­of ­outer ­mitochondrial ­membrane ­34HTOM34P, TOM34, URCC3, Q15785, gene:10953-3literature, mito protein domainMOMProtein import, sorting and homeostasis > Protein import and sorting > TOMtestis
PRORPprotein ­only ­RNase ­P ­catalytic ­subunitKIAA0391, MRPP3, O15091, gene:9692-3APEX_matrixMatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > Polycistronic mtRNA processing		spinalcord, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
PLSCR3phospholipid ­scramblase ­3Q9NRY6, gene:57048-3literature, mito protein domainMIMMetabolism > Lipid metabolism > Cardiolipin synthesisnone
CKMT1Bcreatine ­kinase, ­mitochondrial ­1BCKMT, CKMT1, UMTCK, 0, gene:1159-3literature, APEX_IMS, targetP signal, mito protein domainMIMMetabolism > Nucleotide metabolism > Creatine metabolismnone
NDUFB1NADH:ubiquinone ­oxidoreductase ­subunit ­B1CI-MNLL, CI-SGDH, MNLL, O75438, gene:4707-3literature, mito protein domain+MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		none
ATP5MF-PTCD1ATP5MF-PTCD1 ­readthroughATP5J2-PTCD1, B4DJ38, gene:100526740-3APEX_matrix, mito protein domain+unknownnonenone
CASP8caspase ­8ALPS2B, CAP4, Casp-8, FLICE, MACH, MCH5, Q14790, gene:841-3literature, mito protein domainMOMProtein import, sorting and homeostasis > Protein homeostasis;
Mitochondrial dynamics and surveillance > Apoptosis		none
BCL2L1BCL2 ­like ­1BCL-XL/S, BCL2L, BCLX, Bcl-X, PPP1R52, Q07817, gene:598-3literature, mito protein domainMOMMitochondrial dynamics and surveillance > Apoptosiscerebrum, cerebellum, placenta
EXD2exonuclease ­3'-5' ­domain ­containing ­2C14orf114, EXDL2, Q9NVH0, gene:55218-3literature, mito protein domainMatrixMitochondrial central dogma > Translationcerebrum, cerebellum, testis
ARMC10armadillo ­repeat ­containing ­10PNAS-112, PNAS112, PSEC0198, SVH, Q8N2F6, gene:83787-4GFP, MS/MSMOMMitochondrial dynamics and surveillance > Fission;
Mitochondrial dynamics and surveillance > Trafficking		cerebrum, cerebellum, brainstem, spinalcord, liver, adipose, largeintestine, stomach, placenta, testis
MIEF2mitochondrial ­elongation ­factor ­2MID49, SMCR7, Q96C03, gene:125170-4literature, mito protein domainMOMMitochondrial dynamics and surveillance > Fissionnone
THG1LtRNA-histidine ­guanylyltransferase ­1 ­likeICF45, IHG-1, IHG1, SCAR28, THG1, hTHG1, Q9NWX6, gene:54974-4literature, targetP signal, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mt-tRNA modificationsnone
GOLPH3golgi ­phosphoprotein ­3GOPP1, GPP34, MIDAS, Vps74, Q9H4A6, gene:64083-4literature, inductionIMSnonenone
PREPLprolyl ­endopeptidase ­likeCMS22, Q4J6C6, gene:9581-4APEX_matrix, Rickettsial homologMatrixnonespinalcord, largeintestine
FASTKD3FAST ­kinase ­domains ­3Q14CZ7, gene:79072-4APEX_matrix, targetP signalMatrixMitochondrial central dogma > mtRNA metabolism > Polycistronic mtRNA processing;
Mitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay		none
CEP89centrosomal ­protein ­89CCDC123, CEP123, Q96ST8, gene:84902-4literature, targetP signalIMSOXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors		none
GPAT2glycerol-3-phosphate ­acyltransferase ­2, ­mitochondrialCT123, Q6NUI2, gene:150763-5manual MOMMetabolism > Lipid metabolism > Cardiolipin synthesis;
Metabolism > Lipid metabolism > Phospholipid metabolism		testis
NBR1NBR1 ­autophagy ­cargo ­receptor1A1-3B, IAI3B, M17S2, MIG19, Q14596, gene:4077-5APEX_IMS, coexpressionIMSMitochondrial dynamics and surveillance > Autophagycerebellum, liver
BOKBCL2 ­family ­apoptosis ­regulator ­BOKBCL2L9, BOKL, Q9UMX3, gene:666-5manual, mito protein domainMOMMitochondrial dynamics and surveillance > Apoptosisnone
FKBP10FKBP ­prolyl ­isomerase ­10BRKS1, FKBP65, OI11, OI6, PPIASE, hFKBP65, Q96AY3, gene:60681-5APEX_IMS, mito protein domainIMSProtein import, sorting and homeostasis > Protein homeostasis > Chaperonesnone
SETD9SET ­domain ­containing ­9C5orf35, Q8NE22, gene:133383-5APEX_matrix, targetP signalMatrixnonenone
CYP11B1cytochrome ­P450 ­family ­11 ­subfamily ­B ­member ­1CPN1, CYP11B, FHI, P450C11, P15538, gene:1584-5manual, targetP signal, mito protein domainMIMMetabolism > Lipid metabolism > Cholesterol, bile acid, steroid synthesis;
Metabolism > Metals and cofactors > Heme-containing proteins		none
CSKMTcitrate ­synthase ­lysine ­methyltransferaseCS-KMT, METTL12, U99HG, A8MUP2, gene:751071-5literature, targetP signal, mito protein domainMatrixSignalingnone
MIGA1mitoguardin ­1FAM73A, Q8NAN2, gene:374986-5literatureMOMMitochondrial dynamics and surveillance > Fusioncerebrum, cerebellum, brainstem, spinalcord, smallintestine, testis
RPIAribose ­5-phosphate ­isomerase ­ARPI, RPIAD, P49247, gene:22934-5APEX_matrixMatrixMetabolism > Carbohydrate metabolismplacenta
BBC3BCL2 ­binding ­component ­3JFY-1, JFY1, PUMA, Q96PG8, gene:27113-6manual MOMMitochondrial dynamics and surveillance > Apoptosisnone
BADBCL2 ­associated ­agonist ­of ­cell ­deathBBC2, BCL2L8, Q92934, gene:572-6literature, mito protein domainMOMMitochondrial dynamics and surveillance > Apoptosisnone
BCL2BCL2 ­apoptosis ­regulatorBcl-2, PPP1R50, P10415, gene:596-6literature, mito protein domainMOMMitochondrial dynamics and surveillance > Apoptosisnone
BCL2A1BCL2 ­related ­protein ­A1ACC-1, ACC-2, ACC1, ACC2, BCL2L5, BFL1, GRS, HBPA1, Q16548, gene:597-6manual, mito protein domainMOMMitochondrial dynamics and surveillance > Apoptosisnone
BCL2L2BCL2 ­like ­2BCL-W, BCL2-L-2, BCLW, PPP1R51, Q92843, gene:599-6literature, mito protein domainMOMMitochondrial dynamics and surveillance > Apoptosisnone
CASP3caspase ­3CPP32, CPP32B, SCA-1, P42574, gene:836-6literature, mito protein domainIMSProtein import, sorting and homeostasis > Protein homeostasis > Proteases;
Mitochondrial dynamics and surveillance > Apoptosis		none
DMPKDM1 ­protein ­kinaseDM, DM1, DM1PK, DMK, MDPK, MT-PK, Q09013, gene:1760-6literature, mito protein domainMOMnonenone
DNA2DNA ­replication ­helicase/nuclease ­2DNA2L, hDNA2, P51530, gene:1763-6literature, mito protein domainMatrixMitochondrial central dogma > mtDNA maintenance > mtDNA replication;
Metabolism > Metals and cofactors > Fe-S-containing proteins		none
MT-ND6NADH ­dehydrogenase, ­subunit ­6 ­(complex ­I)MTND6, P03923, gene:4541-6literature, mito protein domainMIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits		none
PRKNparkin ­RBR ­E3 ­ubiquitin ­protein ­ligaseAR-JP, LPRS2, PARK2, PDJ, O60260, gene:5071-6manual, mito protein domainMOMMitochondrial dynamics and surveillance > Mitophagy;
Mitochondrial dynamics and surveillance > Autophagy		none
ALKBH1alkB ­homolog ­1, ­histone ­H2A ­dioxygenaseABH, ABH1, ALKBH, alkB, hABH, Q13686, gene:8846-6literature, APEX_matrix, mito protein domainMatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications		none
PICK1protein ­interacting ­with ­PRKCA ­1PICK, PRKCABP, Q9NRD5, gene:9463-6literature, mito protein domainunknownnonenone
BCL2L10BCL2 ­like ­10BCL-B, Boo, Diva, bcl2-L-10, Q9HD36, gene:10017-6manual, mito protein domainMOMMitochondrial dynamics and surveillance > Apoptosisnone
AKAP10A-kinase ­anchoring ­protein ­10AKAP-10, D-AKAP-2, D-AKAP2, PRKA10, O43572, gene:11216-6literature, mito protein domainMOMSignaling > cAMP-PKA signalingnone
STYXL1serine/threonine/tyrosine ­interacting ­like ­1DUSP24, MK-STYX, MKSTYX, Q9Y6J8, gene:51657-6literature, mito protein domainMatrixMitochondrial dynamics and surveillance > Apoptosisnone
NSUN2NOP2/Sun ­RNA ­methyltransferase ­2MISU, MRT5, SAKI, TRM4, Q08J23, gene:54888-6literature, mito protein domainMatrixMitochondrial central dogma > mtRNA metabolism > mt-tRNA modificationsnone
ADCY10adenylate ­cyclase ­10HCA2, HEL-S-7a, SAC, SACI, Sacy, hsAC, Q96PN6, gene:55811-6literature, mito protein domainMembraneSignaling > cAMP-PKA signalingnone
SPHK2sphingosine ­kinase ­2SK 2, SK-2, SPK 2, SPK-2, Q9NRA0, gene:56848-6literature, mito protein domainMIMMetabolism > Lipid metabolism > Phospholipid metabolism;
Mitochondrial dynamics and surveillance > Apoptosis		none
NSUN3NOP2/Sun ­RNA ­methyltransferase ­3MST077, MSTP077, Q9H649, gene:63899-6APEX_matrix, mito protein domainMatrixMitochondrial central dogma > mtRNA metabolism > mt-tRNA modificationsnone
PABPC5poly(A) ­binding ­protein ­cytoplasmic ­5PABP5, Q96DU9, gene:140886-6literature, mito protein domainMatrixnonenone
PRSS35serine ­protease ­35C6orf158, dJ223E3.1, Q8N3Z0, gene:167681-6GFP, mito protein domainunknownProtein import, sorting and homeostasis > Protein homeostasisnone
PRIMPOLprimase ­and ­DNA ­directed ­polymeraseCCDC111, MYP22, Primpol1, Q96LW4, gene:201973-6literature, mito protein domainMatrixMitochondrial central dogma > mtDNA maintenance > mtDNA repairnone
SERAC1serine ­active ­site ­containing ­1Q96JX3, gene:84947-6literature, APEX_IMSMOMMetabolism > Lipid metabolism > Phospholipid metabolismcerebrum, cerebellum, skeletalmuscle, stomach, testis
STX17syntaxin ­17P56962, gene:55014-6APEX_IMS, coexpressionMOMMitochondrial dynamics and surveillance > Fission;
Mitochondrial dynamics and surveillance > Organelle contact sites;
Mitochondrial dynamics and surveillance > Autophagy		testis
TRMT61BtRNA ­methyltransferase ­61BQ9BVS5, gene:55006-7APEX_matrix, mito protein domainMatrixMitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications;
Mitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications;
Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly		none
PDE2Aphosphodiesterase ­2ACGS-PDE, PDE2A1, PED2A4, cGSPDE, O00408, gene:5138-7literatureMIMSignaling > cAMP-PKA signalingcerebrum, cerebellum, brainstem, largeintestine
PLGRKTplasminogen ­receptor ­with ­a ­C-terminal ­lysineAD025, C9orf46, MDS030, PLG-RKT, Plg-R(KT), Q9HBL7, gene:55848-7GFPMembranenonecerebrum, cerebellum, brainstem, spinalcord, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
ETFBKMTelectron ­transfer ­flavoprotein ­subunit ­beta ­lysine ­methyltransferaseC12orf72, ETFB-KMT, METTL20, Q8IXQ9, gene:254013-7literature, targetP signalMatrixMetabolism > Electron carriers > Q-linked reactions, othernone
RTL10retrotransposon ­Gag ­like ­10BOP, C22orf29, Q7L3V2, gene:79680-7manual, targetP signalMOMMitochondrial dynamics and surveillance > Apoptosisnone
BCL2L11BCL2 ­like ­11BAM, BIM, BOD, O43521, gene:10018-8manual MOMMitochondrial dynamics and surveillance > Apoptosisnone
SPIRE1spire ­type ­actin ­nucleation ­factor ­1Spir-1, Q08AE8, gene:56907-8literatureMOMMitochondrial dynamics and surveillance > Fission;
Mitochondrial dynamics and surveillance > Organelle contact sites		none
C2orf69chromosome ­2 ­open ­reading ­frame ­69Q8N8R5, gene:205327-8APEX_matrixMatrixnonenone
ARMCX2armadillo ­repeat ­containing ­X-linked ­2ALEX2, GASP9, Q7L311, gene:9823-10literatureMembranenonenone
ARMCX1armadillo ­repeat ­containing ­X-linked ­1ALEX1, GASP7, Q9P291, gene:51309-10literatureMOMMitochondrial dynamics and surveillance > Traffickingnone
POLBDNA ­polymerase ­betaP06746, gene:5423-10literatureMatrixMitochondrial central dogma > mtDNA maintenance > mtDNA repairnone
SNAP29synaptosome ­associated ­protein ­29CEDNIK, SNAP-29, O95721, gene:9342-10APEX_IMSIMSMitochondrial dynamics and surveillance > Autophagynone
ARMCX3armadillo ­repeat ­containing ­X-linked ­3ALEX3, GASP6, dJ545K15.2, Q9UH62, gene:51566-10literatureMOMMitochondrial dynamics and surveillance > Traffickingnone
ARMCX6armadillo ­repeat ­containing ­X-linked ­6GASP10, Q7L4S7, gene:54470-10literatureMembranenonenone
METTL4methyltransferase ­like ­4HsT661, Q8N3J2, gene:64863-10literatureMatrixMitochondrial central dogma > mtDNA maintenance > mtDNA modificationsnone
SPHKAPSPHK1 ­interactor, ­AKAP ­domain ­containingSKIP, Q2M3C7, gene:80309-10manual MIMSignaling > cAMP-PKA signalingnone
NAT8LN-acetyltransferase ­8 ­likeCML3, NACED, NAT8-LIKE, Q8N9F0, gene:339983-10literatureMembraneMetabolism > Amino acid metabolism > Glutamate metabolismnone
MCCD1mitochondrial ­coiled-coil ­domain ­1P59942, gene:401250-10manual unknownnonenone
PIGBOS1PIGB ­opposite ­strand ­1HP06981, A0A0B4J2F0, gene:101928527NAliteratureMOMMitochondrial dynamics and surveillance > Organelle contact sitesNA
HTD2hydroxyacyl-thioester ­dehydratase ­type ­2P86397, gene:109703458NAliteratureMatrixMetabolism > Lipid metabolism > Type II fatty acid synthesisNA
RP11_469A15.2RP11_469A15.20, gene:ENSG00000230623NAliteratureMIMNA