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Mitochondrial Biogenesis - Propithecus coquereli (Coquerel's sifaka)
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AMitochondrial DNA transcription, translation, and replication factors
B Mitochondrial DNA-encoded proteins
C Mitochondrial respiratory chain complex I
D 6458118 ND1; NADH dehydrogenase subunit 1 K03878 ND1; NADH-ubiquinone oxidoreductase chain 1 [EC:7.1.1.2]
D 6458121 ND2; NADH dehydrogenase subunit 2 K03879 ND2; NADH-ubiquinone oxidoreductase chain 2 [EC:7.1.1.2]
D 6458120 ND3; NADH dehydrogenase subunit 3 K03880 ND3; NADH-ubiquinone oxidoreductase chain 3 [EC:7.1.1.2]
D 6458127 ND4; NADH dehydrogenase subunit 4 K03881 ND4; NADH-ubiquinone oxidoreductase chain 4 [EC:7.1.1.2]
D 6458117 ND4L; NADH dehydrogenase subunit 4L K03882 ND4L; NADH-ubiquinone oxidoreductase chain 4L [EC:7.1.1.2]
D 6458126 ND5; NADH dehydrogenase subunit 5 K03883 ND5; NADH-ubiquinone oxidoreductase chain 5 [EC:7.1.1.2]
D 6458119 ND6; NADH dehydrogenase subunit 6 K03884 ND6; NADH-ubiquinone oxidoreductase chain 6 [EC:7.1.1.2]
C Mitochondrial respiratory chain complex III
D 6458122 CYTB; cytochrome b K00412 CYTB; ubiquinol-cytochrome c reductase cytochrome b subunit
C Cytochrome c oxidase
D 6458125 COX1; cytochrome c oxidase subunit I K02256 COX1; cytochrome c oxidase subunit 1 [EC:7.1.1.9]
D 6458115 COX2; cytochrome c oxidase subunit II K02261 COX2; cytochrome c oxidase subunit 2
D 6458124 COX3; cytochrome c oxidase subunit III K02262 COX3; cytochrome c oxidase subunit 3
C F-type ATPase
D 6458116 ATP8; ATP synthase F0 subunit 8 K02125 ATPeF08; F-type H+-transporting ATPase subunit 8
D 6458123 ATP6; ATP synthase F0 subunit 6 K02126 ATPeF0A; F-type H+-transporting ATPase subunit a
B Mitochondrial transcription and translation factors
C Mitochondrial RNA polymerase
D 105811294 POLRMT; DNA-directed RNA polymerase, mitochondrial isoform X1 K10908 POLRMT; DNA-directed RNA polymerase, mitochondrial [EC:2.7.7.6]
C Mitochondrial transcription factors
D 105821053 TFAM; transcription factor A, mitochondrial K11830 TFAM; transcription factor A, mitochondrial
D 105812403 TFB1M; dimethyladenosine transferase 1, mitochondrial K15266 TFB1M; dimethyladenosine transferase 1, mitochondrial [EC:2.1.1.-]
D 105810386 TFB2M; dimethyladenosine transferase 2, mitochondrial K17653 TFB2M; dimethyladenosine transferase 2, mitochondrial [EC:2.1.1.-]
C Mitochondrial translation factors
D 105814779 MTIF2; translation initiation factor IF-2, mitochondrial K02519 infB; translation initiation factor IF-2
D 105814799 MTIF3; LOW QUALITY PROTEIN: translation initiation factor IF-3, mitochondrial K02520 infC; translation initiation factor IF-3
D 105816788 TSFM; elongation factor Ts, mitochondrial K02357 tsf; elongation factor Ts
D 105805980 TUFM; elongation factor Tu, mitochondrial K02358 tuf; elongation factor Tu
D 105809157 GFM1; elongation factor G, mitochondrial K02355 fusA; elongation factor G
D 105826592 GFM2; ribosome-releasing factor 2, mitochondrial K02355 fusA; elongation factor G
D 105809692 MTERF3; transcription termination factor 3, mitochondrial K15031 MTERF; mitochondrial transcription termination factor family protein
D 105810667 MTERF2; LOW QUALITY PROTEIN: transcription termination factor 2, mitochondrial K15031 MTERF; mitochondrial transcription termination factor family protein
D 105820131 MTERF1; transcription termination factor 1, mitochondrial K15031 MTERF; mitochondrial transcription termination factor family protein
D 105824831 MTERF4; transcription termination factor 4, mitochondrial K15031 MTERF; mitochondrial transcription termination factor family protein
C RNase MRP
D 105809725 POP1; ribonucleases P/MRP protein subunit POP1 K01164 POP1; ribonuclease P/MRP protein subunit POP1 [EC:3.1.26.5]
D 105812559 POP4; ribonuclease P protein subunit p29 K03538 POP4; ribonuclease P protein subunit POP4 [EC:3.1.26.5]
D 105815059 POP5; ribonuclease P/MRP protein subunit POP5 K03537 POP5; ribonuclease P/MRP protein subunit POP5 [EC:3.1.26.5]
D 105822722 RPP30; ribonuclease P protein subunit p30 isoform X1 K03539 RPP1; ribonuclease P/MRP protein subunit RPP1 [EC:3.1.26.5]
D 105806015 POP7; ribonuclease P protein subunit p20 K14527 RPP20; ribonuclease P/MRP protein subunit RPP20 [EC:3.1.26.5]
D 105807266 RPP25; ribonuclease P protein subunit p25 K14525 RPP25; ribonucleases P/MRP protein subunit RPP25 [EC:3.1.26.5]
D 105820157 RPP25L; ribonuclease P protein subunit p25-like protein K14525 RPP25; ribonucleases P/MRP protein subunit RPP25 [EC:3.1.26.5]
D 105823698 RPP38; ribonuclease P protein subunit p38 K14523 RPP38; ribonucleases P/MRP protein subunit RPP38 [EC:3.1.26.5]
D 105812516 RPP40; ribonuclease P protein subunit p40 isoform X1 K14530 RPP40; ribonucleases P/MRP protein subunit RPP40 [EC:3.1.26.5]
C Mitochondrial ribonuclease P
D 105817211 TRMT10C; mitochondrial ribonuclease P protein 1 K17654 TRMT10C; mitochondrial ribonuclease P protein 1 [EC:2.1.1.-]
D 105823442 HSD17B10; 3-hydroxyacyl-CoA dehydrogenase type-2 K08683 HSD17B10; 3-hydroxyacyl-CoA dehydrogenase / 3-hydroxy-2-methylbutyryl-CoA dehydrogenase [EC:1.1.1.35 1.1.1.178]
D 105821490 KIAA0391; mitochondrial ribonuclease P protein 3 K17655 MRPP3; mitochondrial ribonuclease P protein 3
C Other mitochondrial DNA transcription and translation factors
D 105825491 putative protein MSS51 homolog, mitochondrial K17656 MSS51; mitochondrial splicing suppressor protein 51
D 105825492 putative protein MSS51 homolog, mitochondrial K17656 MSS51; mitochondrial splicing suppressor protein 51
D 105813533 TEFM; transcription elongation factor, mitochondrial K17658 TEFM; transcription elongation factor, mitochondrial
D 105805991 PTCD1; pentatricopeptide repeat-containing protein 1, mitochondrial K17710 PTCD1; pentatricopeptide repeat domain-containing protein 1
D 105826604 PTCD2; pentatricopeptide repeat-containing protein 2, mitochondrial K17711 PTCD2; pentatricopeptide repeat domain-containing protein 2
D 105810290 PTCD3; pentatricopeptide repeat domain-containing protein 3, mitochondrial K17659 PTCD3; pentatricopeptide repeat domain-containing protein 3
D 105824771 DHX30; putative ATP-dependent RNA helicase DHX30 isoform X1 K13185 DHX30; ATP-dependent RNA helicase DHX30 [EC:5.6.2.6]
D 105804918 ubiquinol-cytochrome-c reductase complex assembly factor 1 K17662 CBP3; cytochrome b pre-mRNA-processing protein 3
D 105815904 LACE1; LOW QUALITY PROTEIN: lactation elevated protein 1 K18798 AFG1; peroxisome-assembly ATPase [EC:3.6.4.7]
D 105815996 XRCC6BP1; mitochondrial inner membrane protease ATP23 homolog K18156 ATP23; mitochondrial inner membrane protease ATP23 [EC:3.4.24.-]
D 105821706 SUPV3L1; ATP-dependent RNA helicase SUPV3L1, mitochondrial K17675 SUPV3L1; ATP-dependent RNA helicase SUPV3L1/SUV3 [EC:5.6.2.6]
D 105825398 LACTB2; beta-lactamase-like protein 2 K16639 LACTB2; endoribonuclease LACTB2 [EC:3.1.27.-]
D 105809466 SLC25A31; ADP/ATP translocase 4 K05863 SLC25A4S; solute carrier family 25 (mitochondrial adenine nucleotide translocator), member 4/5/6/31
D 105810108 SLC25A4; ADP/ATP translocase 1 K05863 SLC25A4S; solute carrier family 25 (mitochondrial adenine nucleotide translocator), member 4/5/6/31
D 105817755 SLC25A5; ADP/ATP translocase 2 K05863 SLC25A4S; solute carrier family 25 (mitochondrial adenine nucleotide translocator), member 4/5/6/31
D 105820356 SLC25A6; ADP/ATP translocase 3 K05863 SLC25A4S; solute carrier family 25 (mitochondrial adenine nucleotide translocator), member 4/5/6/31
D 105824344 SLC25A28; mitoferrin-2 isoform X1 K15113 SLC25A28_37; solute carrier family 25 (mitochondrial iron transporter), member 28/37
D 105825280 SLC25A37; mitoferrin-1 K15113 SLC25A28_37; solute carrier family 25 (mitochondrial iron transporter), member 28/37
D 105822433 MALSU1; mitochondrial assembly of ribosomal large subunit protein 1 K25803 MALSU1; mitochondrial assembly of ribosomal large subunit protein 1
D 105813513 ERAL1; GTPase Era, mitochondrial K03595 era; GTPase
D 105807584 YARS2; tyrosine--tRNA ligase, mitochondrial K01866 YARS; tyrosyl-tRNA synthetase [EC:6.1.1.1]
D 105808600 YARS; tyrosine--tRNA ligase, cytoplasmic K01866 YARS; tyrosyl-tRNA synthetase [EC:6.1.1.1]
D 105821538 LARS; leucine--tRNA ligase, cytoplasmic K01869 LARS; leucyl-tRNA synthetase [EC:6.1.1.4]
D 105824653 LARS2; probable leucine--tRNA ligase, mitochondrial K01869 LARS; leucyl-tRNA synthetase [EC:6.1.1.4]
D 105821097 DARS2; aspartate--tRNA ligase, mitochondrial K01876 DARS2; aspartyl-tRNA synthetase [EC:6.1.1.12]
D 105807323 GARS; glycine--tRNA ligase K01880 GARS; glycyl-tRNA synthetase [EC:6.1.1.14]
D 105805342 RARS2; probable arginine--tRNA ligase, mitochondrial K01887 RARS; arginyl-tRNA synthetase [EC:6.1.1.19]
D 105819497 RARS; arginine--tRNA ligase, cytoplasmic isoform X1 K01887 RARS; arginyl-tRNA synthetase [EC:6.1.1.19]
D 105807107 LOW QUALITY PROTEIN: glutamyl-tRNA(Gln) amidotransferase subunit A, mitochondrial-like K02433 gatA; aspartyl-tRNA(Asn)/glutamyl-tRNA(Gln) amidotransferase subunit A [EC:6.3.5.6 6.3.5.7]
D 105815870 QRSL1; glutamyl-tRNA(Gln) amidotransferase subunit A, mitochondrial K02433 gatA; aspartyl-tRNA(Asn)/glutamyl-tRNA(Gln) amidotransferase subunit A [EC:6.3.5.6 6.3.5.7]
D 105822949 GATB; glutamyl-tRNA(Gln) amidotransferase subunit B, mitochondrial K02434 gatB; aspartyl-tRNA(Asn)/glutamyl-tRNA(Gln) amidotransferase subunit B [EC:6.3.5.6 6.3.5.7]
D 105815075 GATC; glutamyl-tRNA(Gln) amidotransferase subunit C, mitochondrial K02435 gatC; aspartyl-tRNA(Asn)/glutamyl-tRNA(Gln) amidotransferase subunit C [EC:6.3.5.6 6.3.5.7]
D 105818748 MTPAP; poly(A) RNA polymerase, mitochondrial K18060 MTPAP; poly(A) RNA polymerase, mitochondrial [EC:2.7.7.19]
D 105826445 PDE12; 2',5'-phosphodiesterase 12 K19612 PDE12; 2',5'-phosphodiesterase [EC:3.1.13.4 3.1.4.-]
D 105823484 MTG1; mitochondrial ribosome-associated GTPase 1 K19828 MTG1; mitochondrial GTPase 1
D 105807686 NOA1; nitric oxide-associated protein 1 K19832 NOA1; nitric oxide-associated protein 1
D 105810355 NSUN3; putative methyltransferase NSUN3 K21969 NSUN3; tRNA (cytosine34-C5)-methyltransferase [EC:2.1.1.-]
D 105814071 NSUN4; 5-methylcytosine rRNA methyltransferase NSUN4 K21970 NSUN4; 5-methylcytosine rRNA methyltransferase NSUN4 [EC:2.1.1.-]
D 105812406 RPUSD3; RNA pseudouridylate synthase domain-containing protein 3 K22537 RPUSD3; mitochondrial RNA pseudouridine synthase RPUSD3 [EC:5.4.99.-]
D 105825727 RPUSD4; LOW QUALITY PROTEIN: RNA pseudouridylate synthase domain-containing protein 4 K22538 RPUSD4; mitochondrial RNA pseudouridine synthase RPUSD4 [EC:5.4.99.-]
D 105807065 WBSCR16; Williams-Beuren syndrome chromosomal region 16 protein K23495 RCC1L; RCC1-like G exchanging factor-like protein
D 105809853 NGRN; neugrin K23496 NGRN; neugrin
D 105806653 TRUB2; probable tRNA pseudouridine synthase 2 K23497 TRUB2; mitochondrial mRNA pseudouridine synthase TRUB2 [EC:5.4.99.-]
D 105815584 FASTKD1; LOW QUALITY PROTEIN: FAST kinase domain-containing protein 1 K27754 FASTKD1; FAST kinase domain-containing protein 1
D 105823908 FASTKD2; FAST kinase domain-containing protein 2 K18190 FASTKD2; FAST kinase domain-containing protein 2
D 105821320 FASTKD3; FAST kinase domain-containing protein 3 K27755 FASTKD3; FAST kinase domain-containing protein 3
D 105812502 TBRG4; protein TBRG4 K27756 TBRG4; FAST kinase domain-containing protein 4
D 105806212 FASTKD5; LOW QUALITY PROTEIN: FAST kinase domain-containing protein 5 K27757 FASTKD5; FAST kinase domain-containing protein 5
D 105825629 probable peptide chain release factor C12orf65 homolog, mitochondrial K23498 COXPD7; peptide chain release factor
D 105819330 RMND1; LOW QUALITY PROTEIN: required for meiotic nuclear division protein 1 homolog K23499 RMND1; required for meiotic nuclear division protein 1
D 105822800 GRSF1; G-rich sequence factor 1 K24984 GRSF1; G-rich sequence factor 1
D 105814573 METTL15; probable methyltransferase-like protein 15 K25876 METTL15; 12S rRNA N4-methylcytidine (m4C) methyltransferase [EC:2.1.1.-]
D 105817403 UPF0160 protein MYG1, mitochondrial K25877 MYG1; MYG1 exonuclease [EC:3.1.-.-]
D 105815953 uncharacterized protein C6orf203 homolog K26186 MTRES1; mitochondrial transcription rescue factor 1
B Mitochondrial DNA replication factors
C Mitochondrial DNA polymerase
D 105809865 POLG; DNA polymerase subunit gamma-1 K02332 POLG; DNA polymerase gamma 1 [EC:2.7.7.7]
D 105822548 POLG2; DNA polymerase subunit gamma-2, mitochondrial K02333 POLG2; DNA polymerase gamma 2
D 105810074 PRIMPOL; DNA-directed primase/polymerase protein K22761 PRIMPOL; DNA-directed primase/polymerase protein [EC:2.7.7.102 2.7.7.7]
C Mitochondrial DNA maintenance factors
D 105806593 ENDOG; endonuclease G, mitochondrial K01173 ENDOG; endonuclease G, mitochondrial
D 105821265 MGME1; mitochondrial genome maintenance exonuclease 1 K19465 MGME1; mitochondrial genome maintenance exonuclease 1 [EC:3.1.-.-]
D 105806130 EXOG; nuclease EXOG, mitochondrial isoform X1 K15050 EXOG; nuclease EXOG, mitochondrial
D 105824317 twinkle protein, mitochondrial K17680 PEO1; twinkle protein [EC:5.6.2.3]
D 105810805 PIF1; ATP-dependent DNA helicase PIF1 K15255 PIF1; ATP-dependent DNA helicase PIF1 [EC:5.6.2.3]
C Other Mitochondrial DNA replication factors
D 105814708 SSBP1; single-stranded DNA-binding protein, mitochondrial K03111 ssb; single-strand DNA-binding protein
D 105820944 ATPase family AAA domain-containing protein 3 isoform X1 K17681 ATAD3A_B; ATPase family AAA domain-containing protein 3A/B
D 105821680 ubiquinol-cytochrome-c reductase complex assembly factor 2 K17682 MNF1; mitochondrial nucleoid factor 1
D 105824789 uncharacterized protein C17orf80 homolog isoform X1 K27759 MTNAP1; mitochondrial nucleoid-associated protein 1
D 105824590 GIMAP1; GTPase IMAP family member 1 K27758 GIMAP1_3_5; GTPase IMAP family member 1/3/5
D 105824591 GTPase IMAP family member 5-like K27758 GIMAP1_3_5; GTPase IMAP family member 1/3/5
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AMitochondrial protein import machinery
B Endoplasmic reticulum membrane and cytosol
C ER-mitochondria encounter structure (ERMES) complex
C Other ER-memrane and cytosol factors
D 105818094 DNAJA2; dnaJ homolog subfamily A member 2 K09503 DNAJA2; DnaJ homolog subfamily A member 2
D 105821250 TOMM34; mitochondrial import receptor subunit TOM34 K17766 TOM34; mitochondrial import receptor subunit TOM34
D 105813583 RHOT1; mitochondrial Rho GTPase 1 isoform X1 K07870 RHOT1; mitochondrial Rho GTPase 1 [EC:3.6.5.-]
D 105806470 HSPA6; heat shock 70 kDa protein 6 K03283 HSPA1_6_8; heat shock 70kDa protein 1/6/8
D 105810840 HSPA8; heat shock cognate 71 kDa protein K03283 HSPA1_6_8; heat shock 70kDa protein 1/6/8
D 105817877 heat shock 70 kDa protein 1 K03283 HSPA1_6_8; heat shock 70kDa protein 1/6/8
D 105817878 heat shock 70 kDa protein 1B K03283 HSPA1_6_8; heat shock 70kDa protein 1/6/8
D 105817879 HSPA1L; heat shock 70 kDa protein 1-like K03283 HSPA1_6_8; heat shock 70kDa protein 1/6/8
D 105816977 AIPL1; aryl-hydrocarbon-interacting protein-like 1 K17767 AIP; AH receptor-interacting protein
D 105827396 AIP; AH receptor-interacting protein isoform X1 K17767 AIP; AH receptor-interacting protein
D 105809915 FATE1; fetal and adult testis-expressed transcript protein K28235 FATE1; fetal and adult testis-expressed transcript protein
B Outer membrane
C Transporter outer membrane (TOM) complex
D 105820386 TOMM5; LOW QUALITY PROTEIN: mitochondrial import receptor subunit TOM5 homolog K17773 TOM5; mitochondrial import receptor subunit TOM5
D 105807485 TOMM6; mitochondrial import receptor subunit TOM6 homolog K17772 TOM6; mitochondrial import receptor subunit TOM6
D 105823202 TOMM20; mitochondrial import receptor subunit TOM20 homolog K17770 TOM20; mitochondrial import receptor subunit TOM20
D 105825908 TOMM20L; TOMM20-like protein 1 K17770 TOM20; mitochondrial import receptor subunit TOM20
D 105805297 TOMM22; mitochondrial import receptor subunit TOM22 homolog K17769 TOM22; mitochondrial import receptor subunit TOM22
D 105806450 TOMM40L; mitochondrial import receptor subunit TOM40B K11518 TOM40; mitochondrial import receptor subunit TOM40
D 105817645 TOMM40; mitochondrial import receptor subunit TOM40 homolog K11518 TOM40; mitochondrial import receptor subunit TOM40
D 105817175 TOMM70A; mitochondrial import receptor subunit TOM70 K17768 TOM70; mitochondrial import receptor subunit TOM70
C TOB/SAM complex
D 105826282 SAMM50; sorting and assembly machinery component 50 homolog isoform X1 K07277 SAM50; outer membrane protein insertion porin family
C Porin
D 105816029 VDAC1; voltage-dependent anion-selective channel protein 1 K05862 VDAC1; voltage-dependent anion channel protein 1
D 105825488 VDAC2; voltage-dependent anion-selective channel protein 2 isoform X1 K15040 VDAC2; voltage-dependent anion channel protein 2
D 105819681 VDAC3; voltage-dependent anion-selective channel protein 3 K15041 VDAC3; voltage-dependent anion channel protein 3
C ERMES complex
C Other outer membrane factor
D 105815521 MTX2; metaxin-2 K17776 MTX; metaxin
D 105821901 MTX1; metaxin-1 isoform X1 K17776 MTX; metaxin
D 105826493 MTX3; metaxin-3 K17776 MTX; metaxin
D 105813878 MYO19; unconventional myosin-XIX isoform X1 K25838 MYO19; myosin XIX
D 105814922 ARMCX1; LOW QUALITY PROTEIN: armadillo repeat-containing X-linked protein 1 K26187 ARMCX1; armadillo repeat-containing X-linked protein 1
D 105814916 ARMCX2; armadillo repeat-containing X-linked protein 2 K26188 ARMCX2; armadillo repeat-containing X-linked protein 2
D 105823823 armadillo repeat-containing X-linked protein 2-like K26188 ARMCX2; armadillo repeat-containing X-linked protein 2
D 105814943 ARMCX3; armadillo repeat-containing X-linked protein 3 K26189 ARMCX3; armadillo repeat-containing X-linked protein 3
D 105814946 ARMCX6; protein ARMCX6 K26190 ARMCX6; armadillo repeat-containing X-linked protein 6
D 105824662 MARC2; mitochondrial amidoxime reducing component 2 K27318 MTARC; mitochondrial amidoxime-reducing component [EC:1.7.-.-]
D 105824663 MARC1; mitochondrial amidoxime-reducing component 1 isoform X1 K27318 MTARC; mitochondrial amidoxime-reducing component [EC:1.7.-.-]
B Inter membrane space
C Transporter inner membrane (TIM) 9/10 complex
D 105825909 TIMM9; mitochondrial import inner membrane translocase subunit Tim9 isoform X1 K17777 TIM9; mitochondrial import inner membrane translocase subunit TIM9
D 105810058 TIMM10; mitochondrial import inner membrane translocase subunit Tim10 K17778 TIM10; mitochondrial import inner membrane translocase subunit TIM10
D 105818387 TIMM10B; mitochondrial import inner membrane translocase subunit Tim10 B K17779 TIM10B; mitochondrial import inner membrane translocase subunit TIM10B
C TIM8/13 complex
D 105814950 TIMM8A; mitochondrial import inner membrane translocase subunit Tim8 A isoform X1 K17780 TIM8; mitochondrial import inner membrane translocase subunit TIM8
D 105822266 TIMM8B; mitochondrial import inner membrane translocase subunit Tim8 B K17780 TIM8; mitochondrial import inner membrane translocase subunit TIM8
D 105811874 TIMM13; mitochondrial import inner membrane translocase subunit Tim13 K17781 TIM13; mitochondrial import inner membrane translocase subunit TIM13
C Mitochondrial intermembrane space assembly (MIA) complex
D 105824675 CHCHD4; mitochondrial intermembrane space import and assembly protein 40 K17782 MIA40; mitochondrial intermembrane space import and assembly protein 40
D 105813055 GFER; FAD-linked sulfhydryl oxidase ALR isoform X1 K17783 ERV1; mitochondrial FAD-linked sulfhydryl oxidase [EC:1.8.3.2]
B Inner mambrane
C MICOS complex
D 105806743 MICOS complex subunit MIC10-like K17784 MICOS10; MICOS complex subunit MIC10
D 105806966 MICOS complex subunit MIC10-like K17784 MICOS10; MICOS complex subunit MIC10
D 105818030 MICOS complex subunit MIC10-like K17784 MICOS10; MICOS complex subunit MIC10
D 105820461 MINOS1; MICOS complex subunit MIC10 K17784 MICOS10; MICOS complex subunit MIC10
D 105810282 IMMT; MICOS complex subunit MIC60 isoform X1 K17785 IMMT; MICOS complex subunit MIC60
D 105811922 protein QIL1 K24624 MICOS13; MICOS complex subunit MIC13
D 105815459 CHCHD3; MICOS complex subunit MIC19 K17563 CHCHD3; MICOS complex subunit MIC19
D 105819978 APOO; apolipoprotein O isoform X1 K24625 APOO; MICOS complex subunit MIC26
D 105812631 APOOL; MICOS complex subunit MIC27 K24626 APOOL; MICOS complex subunit MIC27
C MICOS complex (yeast)
D 105806743 MICOS complex subunit MIC10-like K17784 MICOS10; MICOS complex subunit MIC10
D 105806966 MICOS complex subunit MIC10-like K17784 MICOS10; MICOS complex subunit MIC10
D 105818030 MICOS complex subunit MIC10-like K17784 MICOS10; MICOS complex subunit MIC10
D 105820461 MINOS1; MICOS complex subunit MIC10 K17784 MICOS10; MICOS complex subunit MIC10
D 105810282 IMMT; MICOS complex subunit MIC60 isoform X1 K17785 IMMT; MICOS complex subunit MIC60
C TIM22 complex
D 105820741 TIMM22; mitochondrial import inner membrane translocase subunit Tim22 K17790 TIM22; mitochondrial import inner membrane translocase subunit TIM22
C TIM23 complex
D 105821649 TIMM23; mitochondrial import inner membrane translocase subunit Tim23 K17794 TIM23; mitochondrial import inner membrane translocase subunit TIM23
D 105816825 TIMM17B; mitochondrial import inner membrane translocase subunit Tim17-B K17795 TIM17; mitochondrial import inner membrane translocase subunit TIM17
D 105819648 TIMM17A; mitochondrial import inner membrane translocase subunit Tim17-A K17795 TIM17; mitochondrial import inner membrane translocase subunit TIM17
D 105820980 TIMM21; mitochondrial import inner membrane translocase subunit Tim21 K17796 TIM21; mitochondrial import inner membrane translocase subunit TIM21
D 105820590 TIMM50; mitochondrial import inner membrane translocase subunit TIM50 K17496 TIM50; mitochondrial import inner membrane translocase subunit TIM50
C Mitochondrial calcium uniporter complex
D 105825513 MCU; calcium uniporter protein, mitochondrial isoform X1 K20858 MCU; calcium uniporter protein, mitochondrial
D 105825495 MICU1; calcium uptake protein 1, mitochondrial K22827 MICU1; calcium uptake protein 1, mitochondrial
D 105808102 MICU2; calcium uptake protein 2, mitochondrial K22828 MICU2; calcium uptake protein 2, mitochondrial
D 105805268 SMDT1; essential MCU regulator, mitochondrial K22830 SMDT1; essential MCU regulator, mitochondrial
D 105817472 MCUR1; mitochondrial calcium uniporter regulator 1 K22137 MCUR1; mitochondrial calcium uniporter regulator 1
C Other inner membrane factors
D 105826218 LETM1; LETM1 and EF-hand domain-containing protein 1, mitochondrial isoform X1 K17800 LETM1; LETM1 and EF-hand domain-containing protein 1, mitochondrial
D 105814570 IMMP1L; mitochondrial inner membrane protease subunit 1 K09647 IMP1; mitochondrial inner membrane protease subunit 1 [EC:3.4.21.-]
D 105817153 IMMP2L; mitochondrial inner membrane protease subunit 2 K09648 IMP2; mitochondrial inner membrane protease subunit 2 [EC:3.4.21.-]
D 105808220 TMEM126A; transmembrane protein 126A K18157 TMEM126A; transmembrane protein 126A
D 105808425 MPC1L; mitochondrial pyruvate carrier 1-like protein K22138 MPC1; mitochondrial pyruvate carrier 1
D 105822326 MPC1; mitochondrial pyruvate carrier 1 K22138 MPC1; mitochondrial pyruvate carrier 1
D 105821133 MPC2; mitochondrial pyruvate carrier 2 K22139 MPC2; mitochondrial pyruvate carrier 2
D 105810221 CHCHD10; coiled-coil-helix-coiled-coil-helix domain-containing protein 10, mitochondrial K22759 CHCHD10; coiled-coil-helix-coiled-coil-helix domain-containing protein 10
D 105806690 OCIAD1; OCIA domain-containing protein 1 K28215 OCIAD1; OCIA domain-containing protein 1
B Matrix
C MMC/PAM proteins
D 105807930 TIMM44; mitochondrial import inner membrane translocase subunit TIM44 K17804 TIM44; mitochondrial import inner membrane translocase subunit TIM44
D 105807634 LOW QUALITY PROTEIN: stress-70 protein, mitochondrial-like K04043 dnaK; molecular chaperone DnaK
D 105811996 HSPA9; stress-70 protein, mitochondrial K04043 dnaK; molecular chaperone DnaK
D 105820295 GRPEL1; grpE protein homolog 1, mitochondrial K03687 GRPE; molecular chaperone GrpE
D 105821542 GRPEL2; grpE protein homolog 2, mitochondrial K03687 GRPE; molecular chaperone GrpE
D 105806338 DNAJC19; mitochondrial import inner membrane translocase subunit TIM14 K09539 DNAJC19; DnaJ homolog subfamily C member 19
D 105813102 PAM16; mitochondrial import inner membrane translocase subunit TIM16 isoform X1 K17805 PAM16; mitochondrial import inner membrane translocase subunit TIM16
C Other matrix factors
D 105818701 PMPCA; mitochondrial-processing peptidase subunit alpha isoform X1 K01412 PMPCA; mitochondrial-processing peptidase subunit alpha [EC:3.4.24.64]
D 105815189 PMPCB; mitochondrial-processing peptidase subunit beta isoform X1 K17732 PMPCB; mitochondrial-processing peptidase subunit beta [EC:3.4.24.64]
D 105814783 MIPEP; mitochondrial intermediate peptidase K01410 MIPEP; mitochondrial intermediate peptidase [EC:3.4.24.59]
D 105808089 HSPD1; 60 kDa heat shock protein, mitochondrial K04077 groEL; chaperonin GroEL [EC:5.6.1.7]
D 105808087 HSPE1; 10 kDa heat shock protein, mitochondrial K04078 groES; chaperonin GroES
D 105813131 DNAJA3; dnaJ homolog subfamily A member 3, mitochondrial K09504 DNAJA3; DnaJ homolog subfamily A member 3
D 105818998 TAMM41; phosphatidate cytidylyltransferase, mitochondrial K17807 TAM41; mitochondrial translocator assembly and maintenance protein 41
D 105818674 DNLZ; DNL-type zinc finger protein K17808 ZIM17; mitochondrial protein import protein ZIM17
D 105817513 ETHE1; persulfide dioxygenase ETHE1, mitochondrial K17725 ETHE1; sulfur dioxygenase [EC:1.13.11.18]
D 105813548 POLDIP2; polymerase delta-interacting protein 2 K17809 POLDIP2; polymerase delta-interacting protein 2
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AMitochondrial quality control factors
B Regulator of mitochondrial biogenesis
C Ubiquitas transcription factors
D 105823945 CREB1; cyclic AMP-responsive element-binding protein 1 K05870 CREB1; cyclic AMP-responsive element-binding protein 1
D 105809157 GFM1; elongation factor G, mitochondrial K02355 fusA; elongation factor G
D 105826592 GFM2; ribosome-releasing factor 2, mitochondrial K02355 fusA; elongation factor G
D 105818043 SP1; transcription factor Sp1 isoform X1 K04684 SP1; transcription factor Sp1
D 105814046 GA-binding protein subunit beta-1 K09454 GABPB; GA-binding protein transcription factor, beta
D 105821758 GABPB2; GA-binding protein subunit beta-2 K09454 GABPB; GA-binding protein transcription factor, beta
D 105821053 TFAM; transcription factor A, mitochondrial K11830 TFAM; transcription factor A, mitochondrial
C Nuclear respiratory factors
D 105826732 NRF1; nuclear respiratory factor 1 K11831 NRF1; nuclear respiratory factor 1
D 105815577 NFE2L2; nuclear factor erythroid 2-related factor 2 isoform X1 K05638 NFE2L2; nuclear factor erythroid 2-related factor 2
D 105820862 metallothionein-I, hippocampal K14739 MT1_2; metallothionein 1/2
D 105820863 metallothionein-2A K14739 MT1_2; metallothionein 1/2
D 105820864 metallothionein-2 K14739 MT1_2; metallothionein 1/2
D 105820865 metallothionein-1E K14739 MT1_2; metallothionein 1/2
C Coactivators
D 105826255 PPARGC1A; peroxisome proliferator-activated receptor gamma coactivator 1-alpha isoform X1 K07202 PPARGC1A; alpha peroxisome proliferator-activated receptor gamma coactivator 1-alpha
D 105821547 PPARGC1B; peroxisome proliferator-activated receptor gamma coactivator 1-beta isoform X1 K17962 PPARGC1B; peroxisome proliferator-activated receptor gamma coactivator 1-beta
D 105824336 PPRC1; peroxisome proliferator-activated receptor gamma coactivator-related protein 1 isoform X1 K17963 PPRC1; peroxisome proliferator-activated receptor gamma coactivator-related protein 1
D 105818181 CRTC1; CREB-regulated transcription coactivator 1 K15309 CRTC1; CREB-regulated transcription coactivator 1
D 105805288 EP300; histone acetyltransferase p300 K04498 EP300; E1A/CREB-binding protein [EC:2.3.1.48]
D 105813122 CREBBP; CREB-binding protein isoform X1 K04498 EP300; E1A/CREB-binding protein [EC:2.3.1.48]
C Proteins involved in Fe-S cluster biogenesis
D 105827592 NFS1; cysteine desulfurase, mitochondrial K04487 iscS; cysteine desulfurase [EC:2.8.1.7]
D 105807591 iron-sulfur cluster assembly enzyme ISCU, mitochondrial K22068 ISCU; iron-sulfur cluster assembly enzyme ISCU, mitochondrial
D 105820997 ISCU; iron-sulfur cluster assembly enzyme ISCU, mitochondrial K22068 ISCU; iron-sulfur cluster assembly enzyme ISCU, mitochondrial
D 105812517 LYRM4; LYR motif-containing protein 4 K22069 LYRM4; LYR motif-containing protein 4
D 105808040 FXN; frataxin, mitochondrial K19054 FXN; frataxin [EC:1.16.3.1]
D 105822215 FDX1; adrenodoxin, mitochondrial K22070 FDX1; ferredoxin-1, mitochondrial
D 105807848 FDX1L; adrenodoxin-like protein, mitochondrial K22071 FDX2; ferredoxin-2, mitochondrial
D 105824769 FDXR; NADPH:adrenodoxin oxidoreductase, mitochondrial K18914 FDXR; adrenodoxin-NADP+ reductase [EC:1.18.1.6]
D 105807634 LOW QUALITY PROTEIN: stress-70 protein, mitochondrial-like K04043 dnaK; molecular chaperone DnaK
D 105811996 HSPA9; stress-70 protein, mitochondrial K04043 dnaK; molecular chaperone DnaK
D 105810165 HSCB; iron-sulfur cluster co-chaperone protein HscB, mitochondrial K04082 hscB; molecular chaperone HscB
D 105812015 SIL1; nucleotide exchange factor SIL1 isoform X1 K14001 SIL1; nucleotide exchange factor SIL1
D 105814961 GLRX5; glutaredoxin-related protein 5, mitochondrial K07390 grxD; monothiol glutaredoxin
D 105814583 iron-sulfur cluster assembly 1 homolog, mitochondrial-like K22063 ISCA1; iron-sulfur cluster assembly 1
D 105822403 ISCA1; iron-sulfur cluster assembly 1 homolog, mitochondrial K22063 ISCA1; iron-sulfur cluster assembly 1
D 105819883 ISCA2; iron-sulfur cluster assembly 2 homolog, mitochondrial K22072 ISCA2; iron-sulfur cluster assembly 2
D 105808147 IBA57; putative transferase CAF17, mitochondrial K22073 IBA57; transferase CAF17, mitochondrial [EC:2.1.-.-]
D 105812060 NFU1; NFU1 iron-sulfur cluster scaffold homolog, mitochondrial K22074 NFU1; NFU1 iron-sulfur cluster scaffold homolog, mitochondrial
D 105821926 BOLA1; bolA-like protein 1 K22066 BOLA1; BolA-like protein 1
D 105821474 BOLA3; bolA-like protein 3 isoform X1 K22075 BOLA3; BolA-like protein 3
D 105824320 SFXN3; sideroflexin-3 K23500 SFXN1_3; sideroflexin-1/3
D 105824847 SFXN1; sideroflexin-1 K23500 SFXN1_3; sideroflexin-1/3
D 105824362 SFXN2; sideroflexin-2 K23501 SFXN2; sideroflexin-2
D 105808695 SFXN4; sideroflexin-4 K23502 SFXN4; sideroflexin-4
D 105812108 SFXN5; sideroflexin-5 K23503 SFXN5; sideroflexin-5
C Other regulator of mitochondrial biogenesis
D 105826795 CPT1A; LOW QUALITY PROTEIN: carnitine O-palmitoyltransferase 1, liver isoform K08765 CPT1A; carnitine O-palmitoyltransferase 1, liver isoform [EC:2.3.1.21]
D 105813938 CPT1B; carnitine O-palmitoyltransferase 1, muscle isoform K19523 CPT1B; carnitine O-palmitoyltransferase 1, muscle isoform [EC:2.3.1.21]
D 105809371 CPT1C; carnitine O-palmitoyltransferase 1, brain isoform isoform X1 K19524 CPT1C; carnitine O-palmitoyltransferase 1, brain isoform [EC:2.3.1.21]
D 105808524 DHTKD1; probable 2-oxoglutarate dehydrogenase E1 component DHKTD1, mitochondrial K15791 DHKTD1; 2-oxoadipate dehydrogenase E1 component [EC:1.2.4.-]
D 105824432 FBXL4; F-box/LRR-repeat protein 4 K10270 FBXL4; F-box and leucine-rich repeat protein 4
D 105824542 HCFC1; host cell factor 1 isoform X1 K14966 HCFC1; host cell factor 1
D 105815114 HCFC2; host cell factor 2 K27390 HCFC2; host cell factor 2
D 105818911 HTT; huntingtin isoform X1 K04533 HD; huntingtin
D 105827290 ESRRA; steroid hormone receptor ERR1 K08552 NR3B1; estrogen-related receptor alpha
D 105819803 ESRRB; steroid hormone receptor ERR2 K08553 NR3B2; estrogen-related receptor beta
D 105826906 ESRRG; estrogen-related receptor gamma isoform X1 K08554 NR3B3; estrogen-related receptor gamma
D 105812803 NRIP1; nuclear receptor-interacting protein 1 K17965 NRIP1; nuclear receptor-interacting protein 1
D 105806302 PRKAA2; 5'-AMP-activated protein kinase catalytic subunit alpha-2 K07198 PRKAA; 5'-AMP-activated protein kinase, catalytic alpha subunit [EC:2.7.11.31]
D 105814830 PRKAA1; 5'-AMP-activated protein kinase catalytic subunit alpha-1 isoform X1 K07198 PRKAA; 5'-AMP-activated protein kinase, catalytic alpha subunit [EC:2.7.11.31]
D 105811918 LONP1; lon protease homolog, mitochondrial K08675 PRSS15; ATP-dependent Lon protease [EC:3.4.21.53]
D 105821946 SIRT1; NAD-dependent protein deacetylase sirtuin-1 isoform X1 K11411 SIRT1; NAD-dependent protein deacetylase sirtuin 1 [EC:2.3.1.286]
D 105812418 SERAC1; protein SERAC1 K23504 SERAC1; protein SERAC1
D 105816822 UXT; protein UXT K26537 UXT; protein UXT
D 105820904 PERM1; PGC-1 and ERR-induced regulator in muscle protein 1 K26738 PERM1; PGC-1 and ERR-induced regulator in muscle protein 1
D 105824163 ES1 protein homolog, mitochondrial K27835 GATD3; glutamine amidotransferase-like class 1 domain-containing protein 3, mitochondrial
D 105818863 TANGO2; transport and Golgi organization protein 2 homolog K28098 TANGO2; transport and Golgi organization protein 2
D 105819368 UPF0565 protein C2orf69 homolog K28271 C2ORF69; mitochondrial protein C2orf69
D 105820563 FAM214B; protein FAM214B isoform X1 K28528 ATOS; protein Atossa
D 105821365 FAM214A; protein FAM214A K28528 ATOS; protein Atossa
D 105819520 DDX47; probable ATP-dependent RNA helicase DDX47 K14777 DDX47; ATP-dependent RNA helicase DDX47/RRP3 [EC:5.6.2.7]
B Mitochondrial respiratory chain complex assembly factors
C Complex-I assembly factors
D 105814273 NDUFAF1; complex I intermediate-associated protein 30, mitochondrial isoform X1 K18159 NDUFAF1; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 1
D 105826637 NDUFAF2; LOW QUALITY PROTEIN: mimitin, mitochondrial K18160 NDUFAF2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 2
D 105818374 NDUFAF3; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 3 K09008 NDUFAF3; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 3
D 105824443 NDUFAF4; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 4 K18161 NDUFAF4; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 4
D 105807133 NDUFAF5; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 5 K18162 NDUFAF5; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 5 [EC:2.1.1.-]
D 105809735 NDUFAF6; NADH dehydrogenase (ubiquinone) complex I, assembly factor 6 K18163 NDUFAF6; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 6
D 105818005 NDUFAF7; NADH dehydrogenase [ubiquinone] complex I, assembly factor 7 K18164 NDUFAF7; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 7
D 105826231 uncharacterized protein C17orf89 homolog K24726 NDUFAF8; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 8
D 105808223 TMEM126B; complex I assembly factor TMEM126B, mitochondrial K18165 TMEM126B; complex I assembly factor TMEM126B
D 105807785 ECSIT; evolutionarily conserved signaling intermediate in Toll pathway, mitochondrial K04405 ECSIT; evolutionarily conserved signaling intermediate in Toll pathway
D 105819006 ACAD9; acyl-CoA dehydrogenase family member 9, mitochondrial K15980 ACAD9; acyl-CoA dehydrogenase family member 9 [EC:1.3.99.-]
D 105813234 NUBPL; iron-sulfur protein NUBPL isoform X1 K03593 mrp; ATP-binding protein involved in chromosome partitioning
D 105825731 FOXRED1; FAD-dependent oxidoreductase domain-containing protein 1 isoform X1 K18166 FOXRED1; FAD-dependent oxidoreductase domain-containing protein 1
D 105815902 RTN4IP1; reticulon-4-interacting protein 1, mitochondrial isoform X1 K23164 RTN4IP1; reticulon-4-interacting protein 1, mitochondrial
D 105808326 TIMMDC1; complex I assembly factor TIMMDC1, mitochondrial isoform X1 K23505 TIMMDC1; complex I assembly factor TIMMDC1
C Complex-II assembly factors
D 105816216 SDHAF1; succinate dehydrogenase assembly factor 1, mitochondrial K18167 SDHAF1; succinate dehydrogenase assembly factor 1
D 105825058 SDHAF2; succinate dehydrogenase assembly factor 2, mitochondrial K18168 SDHAF2; succinate dehydrogenase assembly factor 2
C Complex-III assembly factors
D 105808818 mitochondrial chaperone BCS1 K08900 BCS1; mitochondrial chaperone BCS1
D 105816110 TTC19; tetratricopeptide repeat protein 19, mitochondrial K18169 TTC19; tetratricopeptide repeat protein 19, mitochondrial
D 105804918 ubiquinol-cytochrome-c reductase complex assembly factor 1 K17662 CBP3; cytochrome b pre-mRNA-processing protein 3
D 105821680 ubiquinol-cytochrome-c reductase complex assembly factor 2 K17682 MNF1; mitochondrial nucleoid factor 1
D 105827131 ubiquinol-cytochrome-c reductase complex assembly factor 3 K23165 UQCC3; ubiquinol-cytochrome-c reductase complex assembly factor 3
D 105813119 protein CCSMST1 K27339 UQCC4; ubiquinol-cytochrome c reductase complex assembly factor 4
D 105827510 SMIM4; small integral membrane protein 4 K27340 UQCC5; ubiquinol-cytochrome c reductase complex assembly factor 5
D 105815126 uncharacterized protein C12orf73 homolog K27341 UQCC6; ubiquinol-cytochrome c reductase complex assembly factor 6
D 105809007 uncharacterized protein C7orf73 homolog K27319 STMP1; short transmembrane mitochondrial protein 1
C Complex-IV assembly factors
D 105810558 CMC1; COX assembly mitochondrial protein homolog K18171 CMC1; COX assembly mitochondrial protein 1
D 105813457 CMC2; COX assembly mitochondrial protein 2 homolog K18172 CMC2; COX assembly mitochondrial protein 2
D 105812469 cytochrome c oxidase assembly factor 1 homolog K18173 COA1; cytochrome c oxidase assembly factor 1
D 105820260 cytochrome c oxidase assembly factor 3 homolog, mitochondrial K18175 CCDC56; cytochrome c oxidase assembly factor 3, animal type
D 105811525 cytochrome c oxidase assembly factor 4 homolog, mitochondrial K18177 COA4; cytochrome c oxidase assembly factor 4
D 105808340 cytochrome c oxidase assembly factor 5 isoform X1 K18178 COA5; cytochrome c oxidase assembly factor 5
D 105823121 cytochrome c oxidase assembly factor 6 homolog K18179 COA6; cytochrome c oxidase assembly factor 6
D 105806298 cytochrome c oxidase assembly factor 7 K18180 COA7; cytochrome c oxidase assembly factor 7
D 105816119 protoheme IX farnesyltransferase, mitochondrial K02257 COX10; heme o synthase [EC:2.5.1.141]
D 105827499 cytochrome c oxidase assembly protein COX11, mitochondrial K02258 COX11; cytochrome c oxidase assembly protein subunit 11
D 105816042 cytochrome c oxidase assembly protein COX14 K18181 COX14; cytochrome c oxidase assembly factor 14
D 105824326 cytochrome c oxidase assembly protein COX15 homolog isoform X1 K02259 COX15; heme a synthase [EC:1.17.99.9]
D 105808553 cytochrome c oxidase copper chaperone K02260 COX17; cytochrome c oxidase assembly protein subunit 17
D 105822809 mitochondrial inner membrane protein COX18 K17797 COX18; mitochondrial inner membrane protein COX18
D 105806031 cytochrome c oxidase assembly protein COX19 K18183 COX19; cytochrome c oxidase assembly protein subunit 19
D 105810146 cytochrome c oxidase protein 20 homolog K18184 COX20; cytochrome c oxidase assembly protein subunit 20
D 105810392 cytochrome c oxidase protein 20 homolog K18184 COX20; cytochrome c oxidase assembly protein subunit 20
D 105825491 putative protein MSS51 homolog, mitochondrial K17656 MSS51; mitochondrial splicing suppressor protein 51
D 105825492 putative protein MSS51 homolog, mitochondrial K17656 MSS51; mitochondrial splicing suppressor protein 51
D 105807864 PET100; protein PET100 homolog, mitochondrial K18186 PET100; protein PET100, animal type
D 105821272 PET117; protein PET117 homolog, mitochondrial K18188 PET117; protein PET117
D 105814404 mitochondrial inner membrane protein OXA1L isoform X1 K03217 yidC; YidC/Oxa1 family membrane protein insertase
D 105826687 SURF1; surfeit locus protein 1 isoform X1 K14998 SURF1; surfeit locus 1 family protein
D 105818081 protein SCO1 homolog, mitochondrial K07152 SCO1; protein SCO1
D 105813933 protein SCO2 homolog, mitochondrial isoform X1 K23755 SCO2; protein SCO2
D 105825037 translational activator of cytochrome c oxidase 1 K18189 TACO1; translational activator of cytochrome c oxidase 1
D 105817947 LRPPRC; leucine-rich PPR motif-containing protein, mitochondrial K17964 LRPPRC; leucine-rich PPR motif-containing protein, mitochondrial
D 105812614 APOPT1; apoptogenic protein 1, mitochondrial K23506 APOPT1; apoptogenic protein 1
D 105809007 uncharacterized protein C7orf73 homolog K27319 STMP1; short transmembrane mitochondrial protein 1
D 105827510 SMIM4; small integral membrane protein 4 K27340 UQCC5; ubiquinol-cytochrome c reductase complex assembly factor 5
D 105827318 TMEM223; transmembrane protein 223 K27342 TMEM223; transmembrane protein 223
D 105817741 HIGD1C; HIG1 domain family member 1C K28216 HIGD1A_C; HIG1 domain family member 1A/C, mitochondrial
D 105818537 HIGD1A; HIG1 domain family member 1A, mitochondrial K28216 HIGD1A_C; HIG1 domain family member 1A/C, mitochondrial
D 105823441 HIG1 domain family member 1A, mitochondrial K28216 HIGD1A_C; HIG1 domain family member 1A/C, mitochondrial
D 105822509 HIGD2A; HIG1 domain family member 2A, mitochondrial K28217 HIGD2A; HIG1 domain family member 2A, mitochondrial
C Complex-V assembly factors
D 105814106 ATPAF1; ATP synthase mitochondrial F1 complex assembly factor 1 isoform X1 K07555 ATPeAF1; ATP synthase mitochondrial F1 complex assembly factor 1
D 105826966 ATPAF2; ATP synthase mitochondrial F1 complex assembly factor 2 K07556 ATPeAF2; ATP synthase mitochondrial F1 complex assembly factor 2
D 105825416 TMEM70; transmembrane protein 70, mitochondrial isoform X1 K17966 TMEM70; transmembrane protein 70, mitochondrial
D 105820167 6.8 kDa mitochondrial proteolipid K18193 MP68; 6.8 kDa mitochondrial proteolipid
B Mitochondrial dynamics
C Fission and Fusion factors
D 105806239 MFN1; mitofusin-1 K21356 MFN1; mitofusin 1 [EC:3.6.5.-]
D 105824956 MFN2; mitofusin-2 K06030 MFN2; mitofusin 2 [EC:3.6.5.-]
D 105808416 OPA1; dynamin-like 120 kDa protein, mitochondrial K17079 OPA1; optic atrophy protein 1 [EC:3.6.5.5]
D 105817569 OPA3; optic atrophy 3 protein K23166 OPA3; optic atrophy 3 protein
D 105817571 optic atrophy 3 protein K23166 OPA3; optic atrophy 3 protein
D 105815073 TRIAP1; TP53-regulated inhibitor of apoptosis 1 K17968 TRIAP1; TRIAP1/MDM35 family protein
D 105806825 PARL; presenilins-associated rhomboid-like protein, mitochondrial K09650 PARL; rhomboid-like protein [EC:3.4.21.105]
D 105821684 BAK1; bcl-2 homologous antagonist/killer isoform X1 K14021 BAK; Bcl-2 homologous antagonist/killer
D 105809316 BAX; apoptosis regulator BAX K02159 BAX; apoptosis regulator BAX
D 105807585 DNM1L; dynamin-1-like protein isoform X1 K17065 DNM1L; dynamin 1-like protein [EC:3.6.5.5]
D 105826041 FIS1; mitochondrial fission 1 protein K17969 FIS1; mitochondrial fission 1 protein
D 105805363 LOW QUALITY PROTEIN: small ubiquitin-related modifier 2-like K12160 SUMO; small ubiquitin-related modifier
D 105817093 SUMO3; small ubiquitin-related modifier 3 isoform X1 K12160 SUMO; small ubiquitin-related modifier
D 105819188 SUMO1; small ubiquitin-related modifier 1 isoform X1 K12160 SUMO; small ubiquitin-related modifier
D 105824194 small ubiquitin-related modifier 2-like isoform X1 K12160 SUMO; small ubiquitin-related modifier
D 105824701 SUMO2; small ubiquitin-related modifier 2 K12160 SUMO; small ubiquitin-related modifier
D 105823877 SH3GLB1; endophilin-B1 K11248 SH3GLB1; endophilin-B1
D 105811025 MTCH2; mitochondrial carrier homolog 2 K17885 MTCH; mitochondrial carrier
D 105812224 MTCH1; mitochondrial carrier homolog 1 isoform X1 K17885 MTCH; mitochondrial carrier
D 105808742 MFF; mitochondrial fission factor K22076 MFF; mitochondrial fission factor
D 105821239 GDAP1L1; ganglioside-induced differentiation-associated protein 1-like 1 isoform X1 K22077 GDAP1; ganglioside-induced differentiation-associated protein 1
D 105825366 GDAP1; ganglioside-induced differentiation-associated protein 1 K22077 GDAP1; ganglioside-induced differentiation-associated protein 1
D 105820223 VAT1; synaptic vesicle membrane protein VAT-1 homolog isoform X1 K23167 VAT1; synaptic vesicle membrane protein VAT-1
D 105805233 MIEF1; mitochondrial dynamics protein MID51 isoform X1 K23507 MIEF1_2; mitochondrial dynamics protein MID51/49
D 105826955 MIEF2; mitochondrial dynamics protein MID49 K23507 MIEF1_2; mitochondrial dynamics protein MID51/49
D 105816083 PLD6; mitochondrial cardiolipin hydrolase K16862 PLD6; mitochondrial cardiolipin hydrolase [EC:3.1.4.-]
D 105806616 FAM73B; protein FAM73B K27289 MIGA; mitoguardin
D 105823787 FAM73A; LOW QUALITY PROTEIN: protein FAM73A K27289 MIGA; mitoguardin
C Actin cytoskeleton organization
D 105805953 ACTB; actin, cytoplasmic 1 K05692 ACTB_G1; actin beta/gamma 1
D 105819999 ACTG1; actin, cytoplasmic 2 K05692 ACTB_G1; actin beta/gamma 1
D 105820681 NAA20; N-alpha-acetyltransferase 20 K17972 NAA20; N-terminal acetyltransferase B complex catalytic subunit [EC:2.3.1.254]
D 105825581 NAA25; N-alpha-acetyltransferase 25, NatB auxiliary subunit K17973 NAA25; N-terminal acetyltransferase B complex non-catalytic subunit
C Mitochondrial movement
D 105824797 PHB; prohibitin isoform X1 K17080 PHB1; prohibitin 1
D 105805035 PHB2; prohibitin-2 K17081 PHB2; prohibitin 2
D 105825875 KTN1; kinectin isoform X1 K17975 KTN1; kinectin
C Retention of mitochondria
D 105807585 DNM1L; dynamin-1-like protein isoform X1 K17065 DNM1L; dynamin 1-like protein [EC:3.6.5.5]
D 105812790 MYO5B; unconventional myosin-Vb isoform X1 K10357 MYO5; myosin V
D 105821328 MYO5C; unconventional myosin-Vc K10357 MYO5; myosin V
D 105821383 MYO5A; unconventional myosin-Va K10357 MYO5; myosin V
D 105826835 XIAP; E3 ubiquitin-protein ligase XIAP K04725 XIAP; E3 ubiquitin-protein ligase XIAP [EC:2.3.2.27]
D 105825653 DIABLO; diablo homolog, mitochondrial isoform X1 K10522 DIABLO; diablo
C Morphogenesis factors
D 105810077 SNX25; sorting nexin-25 K17887 SNX25; sorting nexin-25
D 105813583 RHOT1; mitochondrial Rho GTPase 1 isoform X1 K07870 RHOT1; mitochondrial Rho GTPase 1 [EC:3.6.5.-]
D 105810282 IMMT; MICOS complex subunit MIC60 isoform X1 K17785 IMMT; MICOS complex subunit MIC60
D 105821306 MTFP1; mitochondrial fission process protein 1 isoform X1 K17981 MTFP1; mitochondrial fission process protein 1
D 105821866 DAP3; 28S ribosomal protein S29, mitochondrial K17408 DAP3; small subunit ribosomal protein S29
D 105821393 NEDD4; E3 ubiquitin-protein ligase NEDD4 isoform X1 K10591 NEDD4; E3 ubiquitin-protein ligase NEDD4 [EC:2.3.2.26]
D 105811183 OXR1; oxidation resistance protein 1 isoform X1 K25437 OXR1; oxidation resistance protein 1
D 105821865 MSTO1; protein misato homolog 1 isoform X1 K27320 MSTO1; protein misato
D 105823498 PRELID1; PRELI domain-containing protein 1, mitochondrial K27964 PRELID1; PRELI domain-containing protein 1, mitochondrial
D 105821555 PRELID2; PRELI domain-containing protein 2 K27965 PRELID2; PRELI domain-containing protein 2 mitochondrial
D 105808296 protein slowmo homolog 2-like isoform X1 K27966 PRELID3; PRELI domain-containing protein 3, mitochondrial
D 105814870 SLMO1; protein slowmo homolog 1 K27966 PRELID3; PRELI domain-containing protein 3, mitochondrial
D 105820087 SLMO2; protein slowmo homolog 2 K27966 PRELID3; PRELI domain-containing protein 3, mitochondrial
D 105821360 protein slowmo homolog 2-like K27966 PRELID3; PRELI domain-containing protein 3, mitochondrial
D 105823832 protein slowmo homolog 2-like isoform X1 K27966 PRELID3; PRELI domain-containing protein 3, mitochondrial
B Mitophagy factors
C Autophagy-related proteins
D 105814986 ATG2B; autophagy-related protein 2 homolog B K17906 ATG2; autophagy-related protein 2
D 105827410 ATG2A; autophagy-related protein 2 homolog A isoform X1 K17906 ATG2; autophagy-related protein 2
D 105816763 ATG3; ubiquitin-like-conjugating enzyme ATG3 K08343 ATG3; ubiquitin-like-conjugating enzyme ATG3
D 105806394 ATG4C; cysteine protease ATG4C K08342 ATG4; cysteine protease ATG4 [EC:3.4.22.-]
D 105807827 ATG4D; cysteine protease ATG4D isoform X1 K08342 ATG4; cysteine protease ATG4 [EC:3.4.22.-]
D 105811376 ATG4A; cysteine protease ATG4A isoform X1 K08342 ATG4; cysteine protease ATG4 [EC:3.4.22.-]
D 105824495 ATG4B; cysteine protease ATG4B K08342 ATG4; cysteine protease ATG4 [EC:3.4.22.-]
D 105815928 ATG5; autophagy protein 5 isoform X1 K08339 ATG5; autophagy-related protein 5
D 105820245 BECN1; beclin-1 isoform X1 K08334 BECN; beclin
D 105819008 ATG7; ubiquitin-like modifier-activating enzyme ATG7 isoform X1 K08337 ATG7; ubiquitin-like modifier-activating enzyme ATG7
D 105807995 GABARAPL2; gamma-aminobutyric acid receptor-associated protein-like 2 K08341 GABARAP; GABA(A) receptor-associated protein
D 105816656 GABARAPL1; gamma-aminobutyric acid receptor-associated protein-like 1 K08341 GABARAP; GABA(A) receptor-associated protein
D 105819431 GABARAP; gamma-aminobutyric acid receptor-associated protein K08341 GABARAP; GABA(A) receptor-associated protein
D 105808786 ATG9A; autophagy-related protein 9A K17907 ATG9; autophagy-related protein 9
D 105824575 ATG9B; LOW QUALITY PROTEIN: autophagy-related protein 9B K17907 ATG9; autophagy-related protein 9
D 105826590 ATG10; ubiquitin-like-conjugating enzyme ATG10 K17888 ATG10L; ubiquitin-like-conjugating enzyme ATG10
D 105822546 ATG12; ubiquitin-like protein ATG12 isoform X1 K08336 ATG12; ubiquitin-like protein ATG12
D 105825918 ATG14; beclin 1-associated autophagy-related key regulator K17889 ATG14L; beclin 1-associated autophagy-related key regulator
D 105822349 ATG16L1; autophagy-related protein 16-1 K17890 ATG16L1; autophagy-related protein 16-1
D 105811521 ATG16L2; autophagy-related protein 16-2 isoform X1 K20868 ATG16L2; autophagy-related protein 16-2
D 105806035 WIPI2; WD repeat domain phosphoinositide-interacting protein 2 isoform X1 K17908 WIPI1_2; autophagy-related protein 18
D 105824798 WIPI1; WD repeat domain phosphoinositide-interacting protein 1 K17908 WIPI1_2; autophagy-related protein 18
C Parkin-dependent mechanism factors
D 105812371 PARK2; E3 ubiquitin-protein ligase parkin K04556 PARK2; parkin [EC:2.3.2.31]
D 105816962 RNF41; E3 ubiquitin-protein ligase NRDP1 K11981 RNF41; E3 ubiquitin-protein ligase NRDP1 [EC:2.3.2.27]
D 105813162 CLEC16A; protein CLEC16A K19513 CLEC16A; protein CLEC16A
D 105822308 PINK1; serine/threonine-protein kinase PINK1, mitochondrial K05688 PINK1; PTEN induced putative kinase 1 [EC:2.7.11.1]
D 105806825 PARL; presenilins-associated rhomboid-like protein, mitochondrial K09650 PARL; rhomboid-like protein [EC:3.4.21.105]
D 105823270 SQSTM1; sequestosome-1 isoform X1 K14381 SQSTM1; sequestosome 1
D 105807350 TAX1BP1; tax1-binding protein 1 isoform X1 K21347 TAX1BP1; Tax1-binding protein 1
D 105824927 CALCOCO2; calcium-binding and coiled-coil domain-containing protein 2 K21348 CALCOCO2; calcium-binding and coiled-coil domain-containing protein 2
D 105811088 AMBRA1; activating molecule in BECN1-regulated autophagy protein 1 isoform X1 K17985 AMBRA1; activating molecule in BECN1-regulated autophagy protein 1
C Parkin-independent mechanism factors
D 105811079 ATG13; autophagy-related protein 13 isoform X1 K08331 ATG13; autophagy-related protein 13
D 105820727 ULK1; serine/threonine-protein kinase ULK1 isoform X1 K21357 ULK1; serine/threonine-protein kinase ULK1 [EC:2.7.11.1]
D 105816116 ULK2; serine/threonine-protein kinase ULK2 K08269 ULK2; serine/threonine-protein kinase ULK2 [EC:2.7.11.1]
D 105824092 RB1CC1; RB1-inducible coiled-coil protein 1 isoform X1 K17589 RB1CC1; RB1-inducible coiled-coil protein 1
D 105807468 HSP90AB1; heat shock protein HSP 90-beta isoform X1 K04079 HSP90A; molecular chaperone HtpG
D 105808869 heat shock protein HSP 90-beta-like K04079 HSP90A; molecular chaperone HtpG
D 105812585 HSP90AA1; heat shock protein HSP 90-alpha K04079 HSP90A; molecular chaperone HtpG
D 105807832 CDC37; hsp90 co-chaperone Cdc37 K09554 CDC37; cell division cycle protein 37
D 105812820 hsp90 co-chaperone Cdc37 K09554 CDC37; cell division cycle protein 37
D 105806029 SMURF1; E3 ubiquitin-protein ligase SMURF1 K04678 SMURF; E3 ubiquitin ligase SMURF1/2 [EC:2.3.2.26]
D 105822532 SMURF2; E3 ubiquitin-protein ligase SMURF2 K04678 SMURF; E3 ubiquitin ligase SMURF1/2 [EC:2.3.2.26]
D 105825257 BNIP3L; BCL2/adenovirus E1B 19 kDa protein-interacting protein 3-like K15465 BNIP3L; BCL2/adenovirus E1B 19 kDa protein-interacting protein 3-like
D 105809045 FUNDC1; FUN14 domain-containing protein 1 K17986 FUNDC1; FUN14 domain-containing protein 1
C Other mitophagy factors
D 105806302 PRKAA2; 5'-AMP-activated protein kinase catalytic subunit alpha-2 K07198 PRKAA; 5'-AMP-activated protein kinase, catalytic alpha subunit [EC:2.7.11.31]
D 105814830 PRKAA1; 5'-AMP-activated protein kinase catalytic subunit alpha-1 isoform X1 K07198 PRKAA; 5'-AMP-activated protein kinase, catalytic alpha subunit [EC:2.7.11.31]
D 105804954 MAP1LC3A; microtubule-associated proteins 1A/1B light chain 3A K10435 MAP1LC; microtubule-associated protein 1 light chain
D 105810391 microtubule-associated proteins 1A/1B light chain 3C K10435 MAP1LC; microtubule-associated protein 1 light chain
D 105821039 MAP1LC3B; microtubule-associated proteins 1A/1B light chain 3B K10435 MAP1LC; microtubule-associated protein 1 light chain
D 105820204 NBR1; next to BRCA1 gene 1 protein K17987 NBR1; next to BRCA1 gene 1 protein
D 105806054 TECPR1; tectonin beta-propeller repeat-containing protein 1 K17988 TECPR1; tectonin beta-propeller repeat-containing protein 1
D 105819915 ZFYVE1; zinc finger FYVE domain-containing protein 1 K17603 ZFYVE1; zinc finger FYVE domain-containing protein 1
D 105820235 COASY; bifunctional coenzyme A synthase isoform X1 K02318 COASY; phosphopantetheine adenylyltransferase / dephospho-CoA kinase [EC:2.7.7.3 2.7.1.24]
D 105805284 PLA2G6; 85/88 kDa calcium-independent phospholipase A2 isoform X1 K16343 PLA2G6; calcium-independent phospholipase A2 [EC:3.1.1.4]
D 105812531 protein C19orf12 homolog K23168 MPAN; protein MPAN
D 105824215 FUNDC2; FUN14 domain-containing protein 2 K24308 FUNDC2; FUN14 domain-containing protein 2
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#[ BRITE | KEGG2 | KEGG ]
#Last updated: September 5, 2025