mitochondrial protein

More general terms

• protein

More specific terms

• 1,25-dihydroxyvitamin D3 24-hydroxylase, mitochondrial; vitamin D3 24-hydroxylase; 24-OHase; cytochrome P450 24A1; P450-CC24 (CYP24A1, CYP24)
• 2,4-dienoyl CoA reductase
• 2,4-dienoyl-CoA reductase, mitochondrial (2,4-dienoyl-CoA reductase [NADPH], 4-enoyl-CoA reductase [NADPH], DECR1, DECR)
• 2-amino-3-ketobutyrate coenzyme A ligase, mitochondrial; AKB ligase; aminoacetone synthase; glycine acetyltransferase (GCAT, KBL)
• 2-oxoglutarate dehydrogenase E1 component DHKTD1, mitochondrial (dehydrogenase E1 & transketolase domain-containing protein 1, DHTKD1, KIAA1630)
• 2-oxoglutarate dehydrogenase E1 component-like, mitochondrial (alpha-ketoglutarate dehydrogenase-like, OGDHL, KIAA1290)
• 25-hydroxyvitamin D-1 alpha hydroxylase, mitochondrial; cytochrome P450 subfamily XXVIIB polypeptide 1; cytochrome p450 27B1; calcidiol 1-monooxygenase; 25-OHD-1 alpha-hydroxylase; 25-hydroxyvitamin D(3) 1-alpha-hydroxylase; VD3 1A hydroxylase; P450C1 ...
• 28S ribosomal protein S29, mitochondrial; S29mt; MRP-S29; death-associated protein 3; DAP-3; ionizing radiation resistance conferring protein (DAP3, MRPS29)
• 3,2-trans enoyl CoA isomerase; dodecenoyl CoA delta-isomerase (DCI, ECI1)
• 3-hydroxy-3-methylglutaryl coenzyme A lyase (HMG-CoA lyase, HMGCL)
• 3-hydroxyisobutyrate dehydrogenase, mitochondrial (HIBADH)
• 3-hydroxyisobutyryl-CoA hydrolase, mitochondrial; HIBYL-CoA-H; hib-coa hydrolase (hibch)
• 3-mercaptopyruvate sulfurtransferase (MST, MPST TST2)
• 3-methylcrotonyl-CoA carboxylase (MCCase, 3-methylcrotonyl-CoA:carbon dioxide ligase)
• 3-methylcrotonyl-CoA carboxylase-alpha (3-methylcrotonyl-CoA:carbon dioxide ligase alpha subunit, 3-methylcrotonyl-CoA carboxylase 1, MCCase subunit alpha, 3- methylcrotonyl-CoA carboxylase biotin-containing subunit, MCCC1, MCCA)
• 3-methylcrotonyl-CoA carboxylase-beta (3-methylcrotonyl-CoA:carbon dioxide ligase beta subunit, 3-Methylcrotonyl-CoA carboxylase 2)
• 3-OH acyl CoA dehydrogenase
• 3-OH butyrate dehydrogenase; beta-hydroxybutyrate dehydrogenase (BDH1)
• 3-oxoacyl-[acyl-carrier-protein] synthase, mitochondrial
• 4-aminobutyrate aminotransferase, mitochondrial; gamma-amino-N-butyrate transaminase; GABA transaminase; GABA-T; GABA aminotransferase; GABA-AT; L-AIBAT; (S)-3-amino-2-methylpropionate transaminase (ABAT, GABAT)
• 5'(3')-deoxyribonucleotidase, mitochondrial; 5',3'-nucleotidase, mitochondrial; deoxy-5'-nucleotidase 2; dNT-2 (NT5M, DNT2)
• 5-phosphohydroxy-L-lysine phospho-lyase; alanine-glyoxylate aminotransferase 2-like 2 (PHYKPL, AGXT2L2, PP9286)
• 60 kD heat shock protein, mitochondrial; heat shock protein 60; HSP-60; Hsp60; 60 kD chaperonin; chaperonin 60; CPN60; mitochondrial matrix protein P1; P60 lymphocyte protein; HuCHA60 (HSPD1, HSP60)
• A-kinase anchor protein 1, mitochondrial; A-kinase anchor protein 149 kD; AKAP 149; dual specificity A-kinase-anchoring protein 1; D-AKAP-1; protein kinase A-anchoring protein 1; PRKA1; spermatid A-kinase anchor protein 84; S-AKAP84 (AKAP1, AKAP149, PR...
• A-kinase anchor protein 10, mitochondrial; AKAP-10; dual specificity A kinase-anchoring protein 2; D-AKAP-2; protein kinase A-anchoring protein 10; PRKA10 (AKAP10)
• Abhydrolase domain-containing protein 10, mitochondrial (ABHD10)
• acetyl CoA acetyltransferase-1 (acetyl-CoA acetyltransferase mitochondrial, T2, ACAT1 ACAT MAT)
• acetyl-coenzyme A synthetase 2-like, mitochondrial; acetyl-CoA ligase 2; acetyl-CoA synthetase 2; acyl-CoA synthetase short-chain family member 1 (ACSS1, ACAS2L, KIAA1846)
• ACN9 protein homolog, mitochondrial (ACN9)
• acyl-CoA dehydrogenase family member 9, mitochondrial; ACAD-9 (ACAD9)
• acyl-CoA synthetase family member 2, mitochondrial (ACSF2)
• acyl-CoA synthetase family member 3, mitochondrial (ACSF3)
• acyl-CoA synthetase short-chain family member 3, mitochondrial (ACSS3)
• acyl-coenzyme A synthetase ACSM1, mitochondrial (acyl-CoA synthetase medium-chain family member 1, middle-chain acyl-CoA synthetase 1, butyrate-CoA ligase 1, butyryl coenzyme A synthetase 1, lipoate-activating enzyme, ACSM1, BUCS1, LAE, MACS1)
• acyl-coenzyme A synthetase ACSM2A, mitochondrial (scyl-CoA synthetase medium-chain family member 2A, middle-chain acyl-CoA synthetase 2A, butyrate-CoA ligase 2A, butyryl coenzyme A synthetase 2A, ACSM2A, ACSM2, MACS2)
• acyl-coenzyme A synthetase ACSM2B, mitochondrial (acyl-CoA synthetase medium-chain family member 2B, middle-chain acyl-CoA synthetase 2B, butyrate--CoA ligase 2B, butyryl coenzyme A synthetase 2B, xenobiotic/medium-chain fatty acid-CoA ligase HXM-A, AC...
• acyl-coenzyme A synthetase ACSM3, mitochondrial (acyl-CoA synthetase medium-chain family member 3, middle-chain acyl-CoA synthetase 3, butyrate-CoA ligase 3, butyryl coenzyme A synthetase 3, protein SA homolog, ACSM3, SAH)
• acyl-coenzyme A synthetase ACSM4, mitochondrial (ACSM4)
• acyl-coenzyme A synthetase ACSM5, mitochondrial (ACSM5, MACS3)
• acyl-coenzyme A synthetase ACSM6, mitochondrial (ACSM6, C10orf129)
• Acyl-coenzyme A thioesterase 2, mitochondrial; acyl-CoA thioesterase 2; acyl-coenzyme A thioester hydrolase 2a; long-chain acyl-coA thioesterase 2 (ACOT2, PTE2, PTE2A, ZAP128, CTE-Ia)
• adenylate kinase-2 (AK2)
• adenylate kinase-4 (adenylate kinase 3-like, ATP-AMP transphosphorylase, AK3L1, AK3, AK4, AK3L2)
• adrenodoxin-like protein, mitochondrial (ferredoxin-1-like protein, FDX1L)
• age-related maculopathy susceptibility protein 2, mitochondrial (ARMS2)
• agmatinase, mitochondrial (agmatine ureohydrolase, AUH, AGMAT)
• ALA synthetase-1 (nonspecific 5-aminolevulinate synthetase mitochondrial, ALAS-H, ALAS1, ALAS3, ALASH, OK/SW-cl121)
• ALA synthetase-2 (ALAS2, erythroid-specific 5-aminolevulinate synthetase, erythrocytic aminolevulinate synthetase)
• alanine-glyoxylate aminotransferase 2, mitochondrial; AGT 2; (R)-3-amino-2-methylpropionate-pyruvate transaminase; beta-ALAAT II; beta-alanine-pyruvate aminotransferase; D-AIBAT (AGXT2, AGT2)
• Alanine-tRNA ligase, mitochondrial; mitochondrial Alanyl-tRNA synthetase; AlaRS (AARS2, AARSL, KIAA1270)
• aldehyde dehydrogenase 1 family, member B1 (aldehyde dehydrogenase 5, mitochondrial aldehyde dehydrogenase X, ALDH class 2, ALDH1B1, ALDH5, ALDHX)
• aldehyde dehydrogenase 4 family, member A1 (aldehyde dehydrogenase 4, pyrroline 5 carboxylate dehydrogenase, ALDH4A1, ALDH4, P5CDH)
• aldehyde dehydrogenase 6 family, member A1 (methylmalonate-semialdehyde dehydrogenase, ALDH6A1, MMSDH)
• alkylated DNA repair protein alkB homolog 1; alpha-ketoglutarate-dependent dioxygenase ABH1; DNA lyase ABH1 (ALKBH1, ABH, ABH1, ALKBH)
• alpha-aminoadipic semialdehyde synthase, mitochondrial; LKR/SDH; includes: lysine ketoglutarate reductase; LKR; LOR; saccharopine dehydrogenase; SDH (AASS)
• alpha-methylacyl-CoA racemase (2-methylacyl-CoA racemase, AMACR)
• alternative prion protein; AltPrP ()
• AMY-1-associating protein expressed in testis 1 (MAATS1, AAT-1, AAT1, C3orf15)
• apoptosis related protein in the TGF-beta signalling pathway (ARTS)
• apoptosis-inducing factor 1, mitochondrial; programmed cell death protein 8 (AIFM1, AIF, PDCD8)
• apoptosis-inducing factor-like protein (AIFL, AIFM3)
• arginine-tRNA ligase, mitochondrial; mitochondrial Arginyl-tRNA synthetase; ArgRS (RARS2 RARSL)
• asparagine-tRNA ligase, mitochondrial; mitochondrial Asparaginyl-tRNA synthetase; AsnRS (NARS2)
• Aspartate-tRNA ligase, mitochondrial; mitochondrial Aspartyl-tRNA synthetase; AspRS2 (DARS2)
• ATP synthase A chain (ATP synthase 6, protein-6, F0 a element, coupling factor 0-a, MT-ATP6, ATP6, ATPASE6, MTATP)
• ATP synthase C chain (FO c element; ATP synthase lipid-binding protein, mitochondrial; ATPase protein 9)
• ATP synthase coupling factor-6 (ATP5J, ATP5A, ATPM)
• ATP synthase coupling factor-B (ATP synthase subunit s, mitochondrial; mitochondrial ATP synthase regulatory component factor B; ATP5S; ATPW)
• ATP synthase mitochondrial F1 complex assembly factor 1, mitochondrial (ATP11 homolog, ATPAF1, ATP11)
• ATP synthase mitochondrial F1 complex assembly factor 2; ATP12 homolog (ATPAF2, ATP12, LP3663)
• ATP synthase oligomycin sensitivity conferral protein (mitochondrial ATP synthase O subunit, OSCP, ATP5O, ATPO)
• ATP synthase protein-8 (A6L, MT-ATP8, ATP8, ATPASE8, MTATP8)
• ATP synthase subunit epsilon-like protein, mitochondrial (ATP5EP2)
• ATP synthase, H+ transporting, mitochondrial F1 complex, alpha subunit, isoform 1, cardiac muscle
• ATP synthase, H+ transporting, mitochondrial F1 complex, beta polypeptide
• ATP synthase, H+ transporting, mitochondrial F1 complex, gamma polypeptide 1
• ATP-binding cassette sub-family B member 7, mitochondrial; ATP-binding cassette transporter 7; ABC transporter 7 protein (ABCB7 ABC7)
• ATP-dependent Clp protease ATP-binding subunit clpX-like, mitochondrial (CLPX)
• ATP-dependent Clp protease proteolytic subunit, mitochondrial; endopeptidase Clp (CLPP)
• ATP-dependent RNA helicase SUPV3L1, mitochondrial (SUV3-like protein 1, suppressor of var1 3-like protein 1, SUPV3L1, SUV3)
• ATPase inhibitor, mitochondrial; IF1 protein (ATPIF1, ATPI)
• Bcl-2 homologous antagonist/killer; apoptosis regulator BAK; Bcl-2-like protein 7; Bcl2-L-7 (BAK1, BAK, BCL2L7, CDN1)
• Bcl-2-binding component 3; JFY-1; p53 up-regulated modulator of apoptosis (BBC3, PUMA)
• BCL2/adenovirus E1B 19 kD protein-interacting protein 3-like; NIP3-like protein X; NIP3L; BCL2/adenovirus E1B 19 kD protein-interacting protein 3A; adenovirus E1B19K-binding protein B5 (BNIP3L, BNIP3A, BNIP3H, NIX)
• beta,beta-carotene 9',10'-dioxygenase; B-diox-II; beta-carotene dioxygenase 2 (BCO2, BCDO2)
• bifunctional methylenetetrahydrofolate dehydrogenase/cyclohydrolase, mitochondrial; includes: NAD-dependent methylenetetrahydrofolate dehydrogenase; methenyltetrahydrofolate cyclohydrolase (MTHFD2, NMDMC)
• brain mitochondrial carrier protein 1; BMCP-1; mitochondrial uncoupling protein 5; UCP 5; solute carrier family 25 member 14 (SLC25A14, BMCP1, UCP5, UNQ791/PRO1682)
• brain protein 44-like protein; protein HSPC040; CGI-129; PNAS-115 (BRP44L, MPC1)
• branched-chain amino-acid transaminase-2 (mitochondrial branched-chain amino acid aminotransferase, placental protein 18)
• C-1-tetrahydrofolate synthase, cytoplasmic; C1-THF synthase; includes: methylenetetrahydrofolate dehydrogenase; methenyltetrahydrofolate cyclohydrolase; formyltetrahydrofolate synthetase (MTHFD1, MTHFC, MTHFD)
• C-Myc-binding protein; associate of Myc 1; AMY-1 (MYCBP, AMY1)
• C1Q-binding protein, mitochondrial; GC1q-R protein; glycoprotein gC1qBP; C1qBP; hyaluronan-binding protein 1; mitochondrial matrix protein p32; p33 (C1QBP, GC1QBP, HABP1, SF2P32)
• Ca+2-binding mitochondrial carrier protein Aralar1; mitochondrial aspartate glutamate carrier 1; solute carrier family 25 member 12 (SLC25A12, ARALAR1)
• Ca+2-binding mitochondrial carrier protein SCaMC-1 (small Ca+2-binding mitochondrial carrier protein 1, mitochondrial ATP-Mg/Pi carrier protein 1, mitochondrial Ca(2+)-dependent solute carrier protein 1, solute carrier family 25 member 24, SLC25A24, AP...
• Ca+2-binding mitochondrial carrier protein SCaMC-2 (small Ca+2-binding mitochondrial carrier protein 2, mitochondrial ATP-Mg/Pi carrier protein 3, mitochondrial Ca+2-dependent solute carrier protein 3, solute carrier family 25 member 25, SLC25A25, APC3...
• Ca+2-binding mitochondrial carrier protein SCaMC-3 (small Ca+2-binding mitochondrial carrier protein 3, mitochondrial ATP-Mg/Pi carrier protein 2, mitochondrial Ca+2-dependent solute carrier protein 2, solute carrier family 25 member 23, SLC25A23, APC2...
• carbamoyl phosphate synthase-1 (NH3, CPS1)
• carbonic anhydrase-5 (mitochondrial carbonic anhydrase, Purkinje-cell carbonic anhydrase, CA5A)
• carbonic anhydrase-5B (carbonic anhydrase VB, mitochondrial carbonic anhydrase)
• cardiolipin synthase; CLS; protein GCD10 homolog (CRLS1, C20orf155, CLS1)
• CDGSH iron sulfur domain-containing protein 3, mitochondrial (mitoNEET-related protein 2, Miner2, CISD3)
• CDP-diacylglycerol-glycerol-3-phosphate 3-phosphatidyltransferase, mitochondrial; phosphatidylglycerophosphate synthase 1; phosphatidylglycerophosphate synthase; PGP synthase (PGS1)
• CHCH domain-containing protein C22orf16, mitochondrial; protein N27C7-4 (CHCHD10, C22orf16)
• choline dehydrogenase, mitochondrial; CDH (CHDH)
• citrate lyase beta subunit-like protein, mitochondrial; citrate lyase beta-like; beta-methylmalate synthase (CLYBL, CLB)
• citric acid cycle enzyme
• Co-chaperone protein HscB, mitochondrial (Hsc20, HSCB, HSC20)
• Coiled-coil domain-containing protein 123, mitochondrial (CCDC123, CEP89)
• coiled-coil-helix-coiled-coil-helix domain-containing protein 9, mitochondrial (CHCHD9, CHCHD2P9, C9orf49)
• complex 1 intermediate-associated protein 30, mitochondrial; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 1 (NDUFAF1, CIA30, CGI-65)
• Coproporphyrinogen-III oxidase, mitochondrial; COX; Coprogen oxidase; Coproporphyrinogenase (CPOX, CPO, CPX)
• coupling factor 1 or F1 ATPase
• cyclophilin F (cyclophilin 3, mitochondrial peptidyl-prolyl cis-trans isomerase, PPIF, CYP3)
• Cysteine desulfurase, mitochondrial (NFS1, NIFS, HUSSY-08)
• cysteine-tRNA ligase, mitochondrial; mitochondrial Cysteinyl-tRNA synthetase; CysRS (CARS2)
• cytochrome b-c1 complex subunit 6-like, mitochondrial (UQCRHL)
• cytochrome b2
• cytochrome c (CYCS)
• cytochrome C heme lyase; holocytochrome c synthase (HCCS, CCHL)
• cytochrome c oxidase copper chaperone (COX17)
• cytochrome c oxidase subunit 7A3, mitochondrial; cytochrome c oxidase subunit VIIa 3 (COX7A2P2, COX7A3, COX7AL2, COX7AP2)
• cytochrome C1 heme lyase
• cytochrome peroxidase
• cytosolic phospholipase A2 beta; cPLA2-beta; phospholipase A2 group IVB (PLA2G4B)
• D-lactate dehydrogenase, mitochondrial (lactate dehydrogenase D, DLD, LDHD)
• delta-1-pyrroline-5-carboxylate synthase; P5CS; aldehyde dehydrogenase family 18 member A1; includes: glutamate 5-kinase; GK; gamma-glutamyl kinase; gamma-glutamyl phosphate reductase; GPR; glutamate-5-semialdehyde dehydrogenase; glutamyl-gamma-semiald...
• diablo homolog, mitochondrial; second mitochondria-derived activator of caspase; smac protein; direct IAP-binding protein with low pI (DIABLO, SMAC)
• dihydrodipicolinate synthase-like, mitochondrial; DHDPS-like protein (HOGA1, C10orf65)
• dimethylglycine dehydrogenase, mitochondrial (ME2GLYDH, DMGDH)
• DNA polymerase gamma subunit 2, mitochondrial (mitochondrial DNA polymerase accessory subunit, PolG-beta, MtPolB, DNA polymerase gamma accessory 55 kDa subunit, POLG2, MTPOLB)
• DNA topoisomerase I, mitochondrial; TOP1mt (TOP1MT)
• DNA-(apurinic or apyrimidinic site) lyase 2; AP endonuclease XTH2; APEX nuclease 2; APEX nuclease-like 2; apurinic-apyrimidinic endonuclease 2; AP endonuclease 2 (APEX2, APE2, APEXL2, XTH2)
• DnaJ homolog subfamily A member 3, mitochondrial; DnaJ protein Tid-1; hTid-1; hepatocellular carcinoma-associated antigen 57; tumorous imaginal discs protein Tid56 homolog (DNAJA3 HCA57 TID1)
• dynamin-like 120 kD protein, mitochondrial; optic atrophy protein 1; contains: dynamin-like 120 kD protein, form S1 (OPA1, KIAA0567)
• E3 ubiquitin-protein ligase RNF144B; IBR domain-containing protein 2; RING finger protein 144B; p53-inducible RING finger protein (RNF144B, IBRDC2, P53RFP)
• electron transfer flavoprotein (ETF)
• electron transfer flavoprotein-alpha subunit (ETFA)
• electron transport enzyme
• elongation factor Ts, mitochondrial; EF-TsMt; EF-Ts (TSFM)
• elongation factor Tu, mitochondrial; EF-Tu; P43 (TUFM)
• elongation of very long chain fatty acids protein 6 (hELO2, ELOVL6)
• endonuclease G (ENDOG)
• endopeptidase Clp (ATP-dependent Clp protease)
• Enoyl-CoA hydratase domain-containing protein 2, mitochondrial (ECHDC2)
• enoyl-CoA hydratase domain-containing protein 3, mitochondrial (ECHDC3)
• ethanolamine-phosphate phospho-lyase; alanine-glyoxylate aminotransferase 2-like 1 (ETNPPL, AGXT2L1)
• Ethylmalonic encephalopathy protein 1 (ETHE1 protein, hepatoma subtracted clone one protein, ETHE1, HSCO)
• evolutionarily conserved signaling intermediate in Toll pathway, mitochondrial (protein SITPEC, ECSIT)
• F-type ATPase
• F1 ATPase-delta
• F1 ATPase-epsilon (ATP5E)
• FAD-linked sulfhydryl oxidase ALR; ugmenter of liver regeneration; hERV1; hepatopoietin (GFER, ALR, HERV1, HPO)
• ferritin, mitochondrial
• ferrochelatase, mitochondrial; heme synthase; protoheme ferro-lyase (FECH)
• flavoprotein AIF
• frataxin; Friedreich ataxia protein (FXN, FRDA, X25)
• glutamate dehydrogenase (GDH)
• glutamate-tRNA ligase, mitochondrial; mitochondrial glutamyl-tRNA synthetase; GluRS (EARS2, KIAA1970)
• glutaminase
• glutamine amidotransferase-like class 1 domain-containing protein 3A, mitochondrial (GATD3A, C21orf33)
• glutamine amidotransferase-like class 1 domain-containing protein 3B, mitochondrial; Keio novel protein-I; KNP-I; protein GT335; protein HES1 (GATD3B, HES1, KNPI)
• glutamyl-tRNA(Gln) amidotransferase (Glu-ADT)
• glutamyl-tRNA(Gln) amidotransferase subunit B, mitochondrial; Glu-AdT subunit B; cytochrome oxidase assembly factor PET112 homolog (PET112, PET112L, HSPC199)
• glutaredoxin-2, mitochondrial (GLRX2, GRX2, CGI-133)
• Glutaryl-CoA dehydrogenase, mitochondrial (GCD, GCDH)
• glycerol kinase 2; GK 2; glycerokinase 2; ATP:glycerol 3-phosphotransferase 2; glycerol kinase, testis specific 2 (GK2, GKP2, GKTA)
• glycerol kinase 3; GK 3; glycerokinase 3; ATP:glycerol 3-phosphotransferase 3; glycerol kinase, testis specific 1 (GK3P, GKP3, GKTB)
• glycerol-3-phosphate dehydrogenase, mitochondrial; GPDH-M; GPD-M; mtGPD (GPD2)
• glycine amidinotransferase, mitochondrial; L-arginine:glycine amidinotransferase (GATM)
• glycine cleavage system
• glycine cleavage system H protein (GCSH)
• glycine cleavage system T protein; aminomethyltransferase (GCVT, AMT GCST)
• glycine decarboxylase; glycine dehydrogenase [decarboxylating]; glycine cleavage system P protein (GLDC)
• glycine N-acyltransferase (acyl-CoA:glycine N-acyltransferase, AAc, aralkyl acyl-CoA N-acyltransferase, aralkyl acyl-CoA:amino acid N-acyltransferase, HRP-1[CLP], GLYAT, ACGNAT, CAT, GAT)
• glycine N-acyltransferase-like protein 1 (acyl-CoA:glycine N-acyltransferase-like protein 1, GLYATL1, GNAT)
• glycine N-acyltransferase-like protein 2 (acyl-CoA:glycine N-acyltransferase-like protein 2, GLYATL2)
• glyoxalase domain-containing protein 4 (GLOD4, C17orf25, CGI-150, My027)
• Graves disease carrier protein; GDC; Graves disease autoantigen; GDA; mitochondrial solute carrier protein homolog; solute carrier family 25 member 16 (SLC25A1, GDA)
• GrpE protein homolog 1, mitochondrial (mt-GrpE#1, HMGE, GRPEL1, GREPEL1)
• GrpE protein homolog 2, mitochondrial (Mt-GrpE#2, GRPEL2)
• GrpE, Yge1 (Mge1 or GrpEp) in fungi
• HCLS1-associated protein X-1; HS1-associating protein X-1; HAX-1; HS1-binding protein 1; HSP1BP-1 (HAX1 HS1BP1)
• heat shock protein 75 kD, mitochondrial; HSP 75; tumor necrosis factor type 1 receptor-associated protein; TRAP-1; TNFR-associated protein 1 (TRAP1, HSP75)
• hexaprenyldihydroxybenzoate methyltransferase, mitochondrial; 3,4-dihydroxy-5-hexaprenylbenzoate methyltransferase; DHHB methyltransferase; DHHB-MT; DHHB-MTase; 3-demethylubiquinone-10 3-methyltransferase; dihydroxyhexaprenylbenzoate methyltransferase ...
• histidine-tRNA ligase, mitochondrial; histidine-tRNA ligase-like; histidyl-tRNA synthetase; HisRS (HARS2, HARSL, HARSR, HO3)
• hydroxyacid-oxoacid transhydrogenase, mitochondrial (HOT, Fe-containing alcohol dehydrogenase, ADHFe1, alcohol dehydrogenase iron-containing protein 1, ADHFE1)
• hydroxyacyl-thioester dehydratase type 2, mitochondrial; HsHTD2; 3-hydroxyacyl-[acyl-carrier-protein] dehydratase (RPP14)
• immature colon carcinoma transcript 1 protein; peptidyl-tRNA hydrolase ICT1, mitochondrial; digestion substraction 1; DS-1 (ICT1, DS1)
• inorganic pyrophosphatase 2, mitochondrial; pyrophosphatase SID6-306; pyrophosphate phospho-hydrolase 2; PPase 2 (PPA2)
• iron-sulfur cluster assembly 1 homolog, mitochondrial (HESB-like domain-containing protein 2, iron sulfur assembly protein IscA, hIscA, ISCA1, HBLD2, GK004)
• iron-sulfur cluster assembly 2 homolog, mitochondrial (HESB-like domain-containing protein 1, ISCA2, HBLD1)
• iron-sulfur cluster assembly enzyme ISCU (NifU-like N-terminal domain-containing protein, NifU-like protein, ISCU, NIFUN)
• isobutyryl-CoA dehydrogenase, mitochondrial; activator-recruited cofactor 42 kD component; ARC42; acyl-CoA dehydrogenase family member 8; ACAD-8 (ACAD8, ARC42, IBD)
• isochorismatase domain-containing protein 2, mitochondrial (ISOC2)
• isoleucine-tRNA ligase, mitochondrial; mitochondrial isoleucyl-tRNA synthetase; IleRS (IARS2)
• kinesin light chain 3 (KLC3, KLC2-like, KLC2L)
• kinesin-like protein KLP6; kinesin-like protein 6 (KLP6)
• kynurenine/alpha-aminoadipate aminotransferase, mitochondrial; KAT/AadAT; 2-aminoadipate aminotransferase; 2-aminoadipate transaminase; alpha-aminoadipate aminotransferase; AadAT; kynurenine aminotransferase 2; kynurenine-oxoglutarate transaminase 2 (A...
• L-2-hydroxyglutarate dehydrogenase, mitochondrial (duranin, L2HGDH, C14orf160)
• L-threonine 3-dehydrogenase, mitochondrial (TDH)
• leucine-rich PPR motif-containing protein, mitochondrial; 130 kD leucine-rich protein; LRP 130; GP130 (LRPPRC, LRP130)
• leucine-tRNA ligase, mitochondrial; mitochondrial leucyl-tRNA synthetase; LeuRS (LARS2, KIAA0028)
• lipoyl synthase, mitochondrial; lipoate synthase; LS; lip-syn; lipoic acid synthase (LIAS, LAS, HUSSY-01)
• lipoyltransferase 1, mitochondrial; lipoate biosynthesis protein; lipoate-protein ligase; lipoyl ligase (LIPT1)
• lon protease homolog, mitochondrial; lon protease-like protein; LONP; LONHs; mitochondrial ATP-dependent protease lon; serine protease 15 (LONP1, PRSS15)
• Lon protein or Pim1p in fungi
• long-chain-fatty-acid--CoA ligase 6; long-chain acyl-CoA synthetase 6; LACS 6 (ACSL6, ACS2, FACL6, KIAA0837, LACS5)
• long-chain-fatty-acid-CoA ligase 3; long-chain acyl-CoA synthetase 3; LACS 3 (ACSL3, ACS3, FACL3, LACS3)
• long-chain-fatty-acid-CoA ligase 4; long-chain acyl-CoA synthetase 4; LACS 4 (ACSL4, ACS4, FACL4, LACS4)
• long-chain-fatty-acid-CoA ligase 5; long-chain acyl-CoA synthetase 5; LACS 5 (ACSL5, ACS5, FACL5, UNQ633/PRO1250)
• malate synthase, glyoxylate transacetylase or malate-condensingenzyme
• malic enzyme 2 (NAD-dependent malic enzyme, mitochondrial, NAD-ME, malic enzyme 2) (ME2)
• malic enzyme 3 (NADP-dependent malic enzyme mitochondrial, NADP-ME, ME3)
• malonyl-CoA decarboxylase, mitochondrial; MCD (MLYCD)
• malonyl-CoA-acyl carrier protein transacylase, mitochondrial; MCT; mitochondrial malonyltransferase; [acyl-carrier-protein] malonyltransferase (MCAT, MT)
• metaxin
• Metaxin-3 (MTX3)
• methionine aminopeptidase 1D, mitochondrial (MAP1D, METAP1D)
• methionine synthase reductase, mitochondrial (MSR, MTRR)
• methionine-R-sulfoxide reductase B3, mitochondrial (MsrB3, MSRB3)
• methionine-tRNA ligase, mitochondrial; methionyl-tRNA synthetase 2; mitochondrial methionyl-tRNA synthetase; MtMetRS (MARS2)
• Methionyl-tRNA formyltransferase, mitochondrial (MtFMT, MTFMT, FMT, FMT1)
• methylglutaconyl-CoA hydratase, mitochondrial; AU-specific RNA-binding enoyl-CoA hydratase; AU-binding protein/enoyl-CoA hydratase (AUH)
• methylmalonic aciduria type A protein, mitochondrial (MMAA)
• methylmalonic aciduria type B protein; cob(I)yrinic acid a,c-diamide adenosyltransferase, mitochondrial; cob(I)alamin adenosyltransferase (MMAB)
• methylmalonyl Coenzyme A mutase; succinyl CoA mutase; methylmalonyl CoA isomerase (MUT, MCM)
• methyltransferase LOC751071, mitochondrial (METTL12)
• mimitin; Myc-induced mitochondrial protein; B17.2-like; B17.2L; NDUFA12-like protein (MMTN, NDUFA12L, NDUFAF2)
• mitochondrial 18 kD protein (MTP18, MTFP1)
• mitochondrial aspartate aminotransferase (glutamate oxaloacetate transaminase 2, mAspAT, fatty acid-binding protein, FABP-1, FABPpm, GOT2, AST2)
• mitochondrial carnitine/acylcarnitine carrier protein; carnitine/acylcarnitine translocase; CAC; solute carrier family 25 member 20 (SLC25A20, CAC, CACT)
• mitochondrial coiled-coil domain protein 1 (MCCD1)
• mitochondrial dimethyladenosine transferase 1 (S-adenosylmethionine-6-N',N'-adenosyl(rRNA) dimethyltransferase 1, mitochondrial 12S rRNA dimethylase 1, mitochondrial transcription factor B1, mtTFB1, h-mtTFB1, h-mtTFB, TFB1M, hTFB1M)
• mitochondrial dimethyladenosine transferase 2 (S-adenosylmethionine-6-N', N'-adenosyl(rRNA) dimethyltransferase 2, mitochondrial 12S rRNA dimethylase 2, mitochondrial transcription factor B2, mtTFB2, h-mtTFB2, h-mtTFB, hTFB2M, hepatitis C virus NS5A-t...
• mitochondrial elongation factor G1; elongation factor G, mitochondrial; EF-Gmt; elongation factor G 1, mitochondrial; mEF-G 1; elongation factor G1; hEFG1 (GFM1, EFG, EFG1, GFM)
• mitochondrial elongation factor G2; ribosome-releasing factor 2, mitochondrial; RRF2mt; elongation factor G 2, mitochondrial; EF-G2mt; mEF-G 2; elongation factor G2; hEFG2 (GFM2, EFG2, MSTP027)
• mitochondrial enolase superfamily member 1 (antisense RNA to thymidylate synthase, rTS, ENOSF1, RTS, TYMSAS)
• mitochondrial fission regulator 2; DUF729 domain-containing protein 1 (MTFR2, DUFD1, FAM54A)
• mitochondrial genome maintenance exonuclease 1 (MGME1, C20orf72, DDK1)
• mitochondrial GTP:AMP phosphotransferase (adenylate kinase 3 alpha like, AK3, AK6, AKL3L)
• mitochondrial GTPase 1 (GTP-binding protein 7, MTG1, GTPBP7)
• mitochondrial import inner membrane translocase subunit TIM16; mitochondria-associated granulocyte macrophage CSF-signaling molecule; presequence translocated-associated motor subunit PAM16 (PAM16, MAGMAS, TIM16, TIMM16, CGI-136)
• mitochondrial import inner membrane translocase subunit Tim23B (TIMM23B)
• mitochondrial import receptor subunit TOM5 homolog (TOMM5, C9orf105, TOM5)
• mitochondrial intermediate peptidase (MIP, MIPEP)
• mitochondrial intermembrane space import & assembly protein 40 (coiled-coil-helix-coiled-coil-helix domain-containing protein 4, CHCHD4, MIA40)
• mitochondrial membrane protein
• mitochondrial Na+/hydrogen exchanger NHA2; mitochondrial Na+/H+ exchanger NHA2; Na+/hydrogen exchanger-like domain-containing protein 2; Na+/H+ exchanger-like domain-containing protein 2; NHE domain-containing protein 2 (NHEDC2, NHA2, SLC9B2)
• mitochondrial proteolipid
• mitochondrial ribonuclease P
• mitochondrial ribonuclease P protein 3; mitochondrial Rnase P protein 3 (KIAA0391 ,MRPP3)
• mitochondrial ribosomal protein
• mitochondrial trifunctional enzyme complex (trifunctional protein complex, TFP complex, trifunctional enzyme)
• mitochondrial tRNA-specific 2-thiouridylase 1; MTO2 homolog (TRMU, MTU1)
• mitochondrial tumor suppressor 1 (angiotensin-2 type 2 receptor-interacting protein, MTUS1, ATIP, GK1, KIAA1288, MTSG1)
• mitochondrial-derived peptide MOTS-c; mitochondrial open reading frame of the 12S rRNA-c (MT-RNR1)
• mitochondrial-processing peptidase subunit alpha (alpha-MPP, P-55, PMPCA, KIAA0123, MPPA)
• mitochondrial-processing peptidase subunit beta (PMPCB)
• MMP37-like protein, mitochondrial (TAMM41, C3orf31)
• Mn+2 superoxide dismutase (SOD-2, Mn-SOD, SOD2)
• monofunctional C1-tetrahydrofolate synthase, mitochondrial (formyltetrahydrofolate synthetase, MTHFD1L, FTHFSDC1)
• MOSC domain-containing protein 1, mitochondrial (MOSC1, MARC1)
• MOSC domain-containing protein 2, mitochondrial (MOSC2, MARC2)
• MTCP-1 protein; mature T-cell proliferation-1 type A; MTCP-1 type A (P8MTCP1, MTCP1, C61B, MTCP1NB, CMC4)
• mTERF domain-containing protein 1, mitochondrial (MTERFD1)
• mTERF domain-containing protein 3, mitochondrial (mitochondrial transcription termination factor-like protein, mTERFL, MTERFD3)
• Na+/K+/Ca+2 exchanger 6, mitochondrial; Na+/K+/Ca+2-exchange protein 6; Na+/Ca+2 exchanger protein, mitochondrial; solute carrier family 24 member 6; solute carrier family 8 member B1 (SLC8B1, NCKX6, NCLX, SLC24A6)
• NAD kinase 2, mitochondrial; mitochondrial NAD kinase; NAD kinase domain-containing protein 1, mitochondrial (NADK2, C5orf33, MNADK, NADKD1)
• NADH dehydrogenase [ubiquinone] 1 subunit C2, isoform 2; NDUFC2-KCTD14 readthrough transcript protein (NDUFC2-KCTD14)
• NADP isocitrate dehydrogenase 2 (isocitrate dehydrogenase [NADP] mitochondrial)
• neurolysin, mitochondrial; angiotensin-binding protein; microsomal endopeptidase; MEP; mitochondrial oligopeptidase M; neurotensin endopeptidase (NLN, AGTBP, KIAA1226)
• Noxa
• nuclease EXOG, mitochondrial; endonuclease G-like 1; endo G-like 1 (EXOG, ENDOGL1, ENDOGL2, ENGL)
• nucleoside diphosphate kinase mitochondrial (NDP kinase mitochondrial, NDK, nm23-H4, NME4)
• nucleoside diphosphate-linked moiety X motif 17, mitochondrial (Nudix motif 17, NUDT17)
• nucleoside diphosphate-linked moiety X motif 8, mitochondrial (Nudix motif 8, NUDT8)
• octanoyltransferase, mitochondrial; octanoyl-[acyl-carrier-protein]-protein N-octanoyltransferase; lipoyl/octanoyl transferase; lipoate-protein ligase B (LIPT2)
• oligoribonuclease, mitochondrial (small fragment nuclease, RNA exonuclease 2 homolog, REXO2, SFN, SMFN, CGI-114)
• ornithine aminotransferase (Ornithine-oxo-acid aminotransferase, OAT)
• ornithine carbamoyltransferase (OTCase, ornithine transcarbamylase, OTC)
• p53 upregulated modulator of apoptosis (PUMA)
• p53-regulated apoptosis-inducing protein 1 (p53AIP1) (TP53AIP1)
• peptide chain release factor 1, mitochondrial (MTRF1)
• peptide chain release factor 1-like, mitochondrial (mitochondrial translational release factor 1-like, mtRF1a, MTRF1L, MTRF1A)
• Peptide deformylase, mitochondrial (polypeptide deformylase, PDF, PDF1A)
• peptidyl-tRNA hydrolase 2, mitochondrial (PTH-2, Bcl-2 inhibitor of transcription 1, PTRH2, BIT1, PTH2, CGI-147)
• peroxiredoxin-5, mitochondrial; peroxiredoxin V; Prx-V; peroxisomal antioxidant enzyme; PLP; thioredoxin reductase; thioredoxin peroxidase PMP20; antioxidant enzyme B166; AOEB166; TPx type VI; liver tissue 2D-page spot 71B; Alu corepressor 1 (PRDX5, AC...
• phenylalanine-tRNA ligase, mitochondrial; mitochondrial phenylalanyl-tRNA synthetase; PheRS (FARS2, FARS1, HSPC320)
• phosphatidate cytidylyltransferase 2; CDP-DAG synthase 2; CDP-DG synthase 2; CDP-diacylglycerol synthase 2; CDS 2; CDP-diglyceride pyrophosphorylase 2; CDP-diglyceride synthase 2; CTP:phosphatidate cytidylyltransferase 2 (CDS2)
• phosphatidylserine decarboxylase proenzyme [Contains: Phosphatidylserine decarboxylase alpha chain; Phosphatidylserine decarboxylase beta chain] (PISD)
• phosphoenolpyruvate carboxykinase 2 (mitochondrial phosphoenolpyruvate carboxykinase, PEPCK-M, PCK2, PEPCK2)
• Poly(A) RNA polymerase, mitochondrial (PAP, mtPAP, polynucleotide adenylyltransferase, PAP-associated domain- containing protein 1, PAPD1)
• polymerase 1 & transcript release factor; ; Cavin-1 (PTRF, FKSG13)
• polyribonucleotide nucleotidyltransferase 1 (PNPase 1, polynucleotide phosphorylase-like protein, PNPase old-35, 3'-5' RNA exonuclease OLD35, PNPT1, PNPASE)
• pproline-tRNA ligase, mitochondrial; mitochondrial prolyl-tRNA synthetase; ProRS (PARS2)
• pre-mRNA-splicing factor ATP-dependent RNA helicase DHX32 (DEAH box protein 32, DEAD/H helicase-like protein 1, DHLP1, DEAD/H box 32, HuDDX32)
• presequence protease, mitochondrial (hPreP, pitrilysin metalloproteinase 1, metalloprotease 1, hMP1, PITRM1, KIAA1104, MP1)
• protein C2orf25, mitochondrial (MMADHC, C2orf25, CL25022, HSPC161, My011)
• protein C2orf47, mitochondrial (C2orf47)
• protein CEBPZOS; CEBPZ antisense RNA 1; CEBPZ opposite strand (CEBPZOS, CEBPZ-AS1)
• protein COQ10 A, mitochondrial (COQ10A)
• protein COQ10 B, mitochondrial (COQ10B)
• protein EMI5 homolog, mitochondrial (SDHAF2, C11orf79)
• protein FAM136A (FAM136A)
• protein FLJ45275, mitochondrial (FLJ45275)
• protein FLJ75552, mitochondrial (C15ORF62)
• protein GDF5OS, mitochondrial; growth/differentiation factor 5 opposite strand transcript (GDF5OS)
• protein LETM2, mitochondrial (leucine zipper-EF-hand-containing transmembrane protein 1-like, LETM2)
• protein MTO1 homolog, mitochondrial (MTO1)
• protein PET117 homolog, mitochondrial (PET117)
• protein phosphatase 1K, mitochondrial (protein phosphatase 2C isoform kappa, PP2C-kappa, PP2C-like mitochondrial protein, PPM1K, PP2CM)
• protein preY, mitochondrial (PREY, PYURF)
• protein RSM22 homolog, mitochondrial; methyltransferase 11 domain-containing protein 1; false p73 target gene protein (METT11D1, METTL17)
• protein SCO1 homolog, mitochondrial (SCO1, SCOD1)
• protein SCO2 homolog, mitochondrial (SCO2)
• protein SHMOOSE; small human mitochondrial ORF over serine tRNA
• protein tyrosine phosphatase mitochondrial 1; PTEN-like phosphatase; phosphoinositide lipid phosphatase (PTPMT1, MOSP, PNAS-129)
• protoheme IX farnesyltransferase, mitochondrial; heme O synthase (COX10)
• protoporphyrinogen oxidase; PPO (PPOX)
• PTEN upstream open reading frame MP31; micropeptide 31 (MP31)
• pyruvate carboxylase, mitochondrial (pyruvic carboxylase, PCB, PC)
• pyruvate dehydrogenase component x (dihydrolipoamide dehydrogenase-binding protein of pyruvate dehydrogenase complex, E3-binding protein, E3BP, proX, PDHX, PDX1)
• pyruvate dehydrogenase kinase
• pyruvate dehydrogenase phosphatase catalytic subunit; [pyruvate dehydrogenase [Lipoamide-phosphatase]]
• pyruvate dehydrogenase phosphatase regulatory subunit, mitochondrial (PDPr, PDPR, KIAA1990)
• radical S-adenosyl methionine domain-containing protein 1, mitochondrial (oxygen-independent coproporphyrinogen III oxidase-like protein RSAD1, RSAD1)
• reticulon-4-interacting protein 1 (NOGO-interacting mitochondrial protein, RTN4IP1, NIMP)
• ribosome recycling factor, mitochondrial (MRRF)
• ribosome-binding factor A, mitochondrial (RBFA, C18orf22)
• RNA (guanine-9-)-methyltransferase domain-containing protein 1, mitochondrial; renal carcinoma antigen NY-REN-49; HBV pre-S2 trans-regulated protein 2 (RG9MTD1, TRMT10C)
• RNA polymerase mitochondrial (MTRPOL, POLRMT)
• rRNA methyltransferase 1, mitochondrial (MRM1)
• S-adenosylmethionine mitochondrial carrier protein (mitochondrial S-adenosylmethionine transporter, solute carrier family 25 member 26, SLC25A26, SAMC)
• sarcosine dehydrogenase, mitochondrial (SarDH, BPR-2)
• serine hydroxymethyltransferase, mitochondrial; SHMT; glycine hydroxymethyltransferase; serine methylase (SHMT2)
• serine-pyruvate aminotransferase; SPT; alanine-glyoxylate aminotransferase; AGT (AGXT, AGT1 SPAT)
• serine-tRNA ligase, mitochondrial; SerRSmt; mitochondrial Seryl-tRNA synthetase; SerRS; Seryl-tRNA(Ser/sec) synthetase (SARS2, SARSM)
• SH3 domain-binding protein 5; SH3BP-5; SH3 domain-binding protein that preferentially associates with BTK (SH3BP5, SAB)
• short chain enoyl CoA hydratase (SCEH, ECHS1)
• single-stranded DNA-binding protein, mitochondrial; Mt-SSB; MtSSB; PWP1-interacting protein 17 (SSBP1, SSBP)
• sirtuin 3 (NAD-dependent deacetylase sirtuin, silent mating type information regulation 2 homolog 3 mitochondrial, SIRT3)
• solute carrier family 25 member 10; mitochondrial dicarboxylate carrier (SLC25A10, DIC)
• solute carrier family 25 member 41 (SLC25A41)
• solute carrier family 27 member 1; long-chain fatty acid transport protein 1; fatty acid transport protein 1 (SLC27A1, FATP-1, ACSVL5, FATP1)
• solute carrier family 27 member 3 (SLC27A3, long-chain fatty acid transport protein 3, fatty acid transport protein 3, FATP-3, very long-chain acyl-CoA synthetase homolog 3, VLCS-3, ACSVL3, FATP3, UNQ367/PRO703)
• solute carrier family 35 member F6; ANT2-binding protein; ANT2BP; transport & Golgi organization 9 homolog (SLC35F6, C2orf18, UNQ3047/PRO9863)
• sperm mitochondrial-associated cysteine-rich protein (SMCP, MCS, MCSP)
• SRA stem-loop-interacting RNA-binding protein, mitochondrial (SLIRP, C14orf156, DC23, DC50, PD04872)
• stress-70 protein, mitochondrial; 75 kD glucose-regulated protein; GRP 75; heat shock 70 kD protein 9; peptide-binding protein 74; PBP74; mortalin; MOT (HSPA9, GRP75, HSPA9B)
• succinyl-CoA sunthetase alpha subunit (succinyl-CoA ligase [GDP-forming] alpha-chain, SCS-alpha, SUCLG1)
• succinyl-CoA sunthetase betaG subunit
• succinyl-CoA:3-ketoacid-coenzyme A transferase 1(somatic-type succinyl CoA:3-oxoacid CoA-transferase, Scot-S, OXCT1, OXCT, SCOT)
• succinyl-CoA:3-ketoacid-coenzyme A transferase 2 (testis-specific succinyl CoA:3-oxoacid CoA-transferase, SCOT-t, OXCT2)
• Sulfide:quinone oxidoreductase, mitochondrial (SQRDL)
• sulfite oxidase, mitochondrial (SUOX)
• surfeit locus protein 1 (SURF1, SURF-1)
• surfeit locus protein 4 (SURF4, SURF-4)
• thiolase
• thioredoxin reductase 2, mitochondrial; selenoprotein Z; selZ; TR-beta; thioredoxin reductase TR3 (TXNRD2, KIAA1652, TRXR2)
• thioredoxin-2 (TRX2, mitochondrial thioredoxin, TXN2)
• thioredoxin-dependent peroxide reductase, mitochondrial; peroxiredoxin-3; PRX III; antioxidant protein 1; AOP-1; protein MER5 homolog; HBC189 (PRDX3, AOP1)
• thiosulfate sulfurtransferase; rhodanese (TST)
• threonine-tRNA ligase, mitochondrial; mitochondrial threonyl-tRNA synthetase; ThrRS; threonyl-tRNA synthetase-like 1 (TARS2, TARSL1)
• thymidine kinase 2, mitochondrial; Mt-TK (TK2)
• TOMM20-like protein 1 (TOMM20L, UNQ9438/PRO34772)
• trans-2-enoyl-CoA reductase, mitochondrial; nuclear receptor-binding factor 1; HsNrbf-1; NRBF-1 (MECR, NBRF1, CGI-63)
• transcription factor A, mitochondrial; mtTFA; mitochondrial transcription factor 1; MtTF1; transcription factor 6; TCF-6; transcription factor 6-like 2 (TFAM, TCF6, TCF6L2)
• transcription termination factor, mitochondrial (mitochondrial transcription termination factor 1, mTERF)
• translation initiation factor IF-2, mitochondrial; IF-2Mt; IF2(mt); IF-2(Mt) (MTIF2)
• translation initiation factor IF-3, mitochondrial (IF-3Mt, IF3mt, MTIF3)
• trifunctional enzyme beta subunit, mitochondrial; TP-beta; hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase beta subunit (HADHB)
• trimethyllysine hydroxylase (TMLHE, trimethyllysine dioxygenase mitochondrial, epsilon-trimethyllysine 2-oxoglutarate dioxygenase, TML-alpha-ketoglutarate dioxygenase, TML hydroxylase, TML dioxygenase, TMLD)
• tRNA modification GTPase GTPBP3, mitochondrial; GTP-binding protein 3, mitochondrial GTP-binding protein 1 (GTPBP3, MTGP1)
• tRNA-nucleotidyltransferase 1, mitochondrial (mitochondrial tRNA nucleotidyl transferase CCA-adding, mt tRNA adenylyltransferase, mt tRNA CCA-pyrophosphorylase, mt tRNA CCA-diphosphorylase, mt CCA-adding enzyme, TRNT1)
• tryptophan-tRNA ligase, mitochondrial; (Mt)TrpRS; mitochondrial tryptophanyl-tRNA synthetase; TrpRS (WARS2)
• twinkle protein, mitochondrial; T7 gp4-like protein with intramitochondrial nucleoid localization; T7-like mitochondrial DNA helicase; Progressive external ophthalmoplegia 1 protein (PEO1, C10orf2)
• tyrosine-tRNA ligase, mitochondrial; mitochondrial tyrosyl-tRNA synthetase; TyrRS (YARS2)
• ubiquinol-cytochrome-c reductase complex assembly factor 2; breast cancer-associated protein SGA-81M; mitochondrial nucleoid factor 1; mitochondrial protein M19 (UQCC2 C6orf125 MNF1)
• ubiquinone biosynthesis methyltransferase COQ5
• ubiquinone biosynthesis protein COQ7 homolog; coenzyme Q biosynthesis protein 7 homolog; timing protein clk-1 homolog (COQ7)
• Ubiquinone biosynthesis protein COQ9, mitochondrial (COQ9, C16orf49, HSPC326, PSEC0129)
• UMP-CMP kinase 2, mitochondrial (CMPK2)
• UPF0317 protein C14orf159, mitochondrial (C14orf159)
• UPF0511 protein C2orf56, mitochondrial (NDUFAF7, C2orf56)
• uracil DNA glycosylase-1 (UDG, UNG, DGU, UNG1 UNG15)
• Valyl-tRNA synthetase, mitochondrial (valine-tRNA ligase, ValRS, valyl-tRNA synthetase-like, VARS2, KIAA1885 VARS2L VARSL)
• YrdC domain-containing protein, mitochondrial (Ischemia/reperfusion-inducible protein homolog, hIRIP, dopamine receptor-interacting protein 3, YRDC, DRIP3, IRIP)
• [3-methyl-2-oxobutanoate dehydrogenase [lipoamide kinase, mitochondrial; branched-chain alpha-ketoacid dehydrogenase kinase; BCKD-kinase; BCKDHKIN (BCKDK)]]