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Mitochondrial Biogenesis - Canis lupus familiaris (dog)
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AMitochondrial DNA transcription, translation, and replication factors
B Mitochondrial DNA-encoded proteins
C Mitochondrial respiratory chain complex I
D 804476 ND1; NADH dehydrogenase subunit 1 K03878 ND1; NADH-ubiquinone oxidoreductase chain 1 [EC:7.1.1.2]
D 804477 ND2; NADH dehydrogenase subunit 2 K03879 ND2; NADH-ubiquinone oxidoreductase chain 2 [EC:7.1.1.2]
D 804481 ND3; NADH dehydrogenase subunit 3 K03880 ND3; NADH-ubiquinone oxidoreductase chain 3 [EC:7.1.1.2]
D 804483 ND4; NADH dehydrogenase subunit 4 K03881 ND4; NADH-ubiquinone oxidoreductase chain 4 [EC:7.1.1.2]
D 804482 ND4L; NADH dehydrogenase subunit 4L K03882 ND4L; NADH-ubiquinone oxidoreductase chain 4L [EC:7.1.1.2]
D 804484 ND5; NADH dehydrogenase subunit 5 K03883 ND5; NADH-ubiquinone oxidoreductase chain 5 [EC:7.1.1.2]
D 804485 ND6; NADH dehydrogenase subunit 6 K03884 ND6; NADH-ubiquinone oxidoreductase chain 6 [EC:7.1.1.2]
C Mitochondrial respiratory chain complex III
D 804486 CYTB; cytochrome b K00412 CYTB; ubiquinol-cytochrome c reductase cytochrome b subunit
C Cytochrome c oxidase
D 804478 COX1; cytochrome c oxidase subunit I K02256 COX1; cytochrome c oxidase subunit 1 [EC:7.1.1.9]
D 804479 COX2; cytochrome c oxidase subunit II K02261 COX2; cytochrome c oxidase subunit 2
D 804480 COX3; cytochrome c oxidase subunit III K02262 COX3; cytochrome c oxidase subunit 3
C F-type ATPase
D 804487 ATP8; ATP synthase F0 subunit 8 K02125 ATPeF08; F-type H+-transporting ATPase subunit 8
D 804488 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 485102 POLRMT; DNA-directed RNA polymerase, mitochondrial isoform X2 K10908 POLRMT; DNA-directed RNA polymerase, mitochondrial [EC:2.7.7.6]
C Mitochondrial transcription factors
D 488989 TFAM; transcription factor A, mitochondrial K11830 TFAM; transcription factor A, mitochondrial
D 476253 TFB1M; dimethyladenosine transferase 1, mitochondrial K15266 TFB1M; dimethyladenosine transferase 1, mitochondrial [EC:2.1.1.-]
D 480102 TFB2M; dimethyladenosine transferase 2, mitochondrial K17653 TFB2M; dimethyladenosine transferase 2, mitochondrial [EC:2.1.1.-]
C Mitochondrial translation factors
D 474600 MTIF2; translation initiation factor IF-2, mitochondrial isoform X2 K02519 infB; translation initiation factor IF-2
D 477328 MTIF3; translation initiation factor IF-3, mitochondrial K02520 infC; translation initiation factor IF-3
D 100682701 elongation factor Ts, mitochondrial K02357 tsf; elongation factor Ts
D 119873576 TSFM; elongation factor Ts, mitochondrial isoform X1 K02357 tsf; elongation factor Ts
D 479796 TUFM; elongation factor Tu, mitochondrial K02358 tuf; elongation factor Tu
D 478101 GFM2; ribosome-releasing factor 2, mitochondrial isoform X1 K02355 fusA; elongation factor G
D 477128 GFM1; elongation factor G, mitochondrial K02355 fusA; elongation factor G
D 100684053 MTERF1; transcription termination factor 1, mitochondrial isoform X2 K15031 MTERF; mitochondrial transcription termination factor family protein
D 106557455 MTERF2; transcription termination factor 2, mitochondrial K15031 MTERF; mitochondrial transcription termination factor family protein
D 100855859 MTERF4; transcription termination factor 4, mitochondrial isoform X1 K15031 MTERF; mitochondrial transcription termination factor family protein
D 482127 GUF1; translation factor GUF1, mitochondrial K21594 GUF1; translation factor GUF1, mitochondrial [EC:3.6.5.-]
C RNase MRP
D 481978 POP1; ribonucleases P/MRP protein subunit POP1 isoform X3 K01164 POP1; ribonuclease P/MRP protein subunit POP1 [EC:3.1.26.5]
D 477515 POP5; ribonuclease P/MRP protein subunit POP5 isoform X1 K03537 POP5; ribonuclease P/MRP protein subunit POP5 [EC:3.1.26.5]
D 477763 RPP30; ribonuclease P protein subunit p30 isoform X2 K03539 RPP1; ribonuclease P/MRP protein subunit RPP1 [EC:3.1.26.5]
D 489832 POP7; ribonuclease P protein subunit p20 K14527 RPP20; ribonuclease P/MRP protein subunit RPP20 [EC:3.1.26.5]
D 481586 RPP25L; ribonuclease P protein subunit p25-like protein K14525 RPP25; ribonucleases P/MRP protein subunit RPP25 [EC:3.1.26.5]
D 486757 ribonuclease P protein subunit p25-like protein K14525 RPP25; ribonucleases P/MRP protein subunit RPP25 [EC:3.1.26.5]
D 611587 RPP25; ribonuclease P protein subunit p25 K14525 RPP25; ribonucleases P/MRP protein subunit RPP25 [EC:3.1.26.5]
D 100683678 RPP38; ribonuclease P protein subunit p38 K14523 RPP38; ribonucleases P/MRP protein subunit RPP38 [EC:3.1.26.5]
D 100684788 LOW QUALITY PROTEIN: ribonuclease P protein subunit p38-like isoform X1 K14523 RPP38; ribonucleases P/MRP protein subunit RPP38 [EC:3.1.26.5]
D 478709 RPP40; ribonuclease P protein subunit p40 isoform X2 K14530 RPP40; ribonucleases P/MRP protein subunit RPP40 [EC:3.1.26.5]
C Mitochondrial ribonuclease P
D 478545 TRMT10C; tRNA methyltransferase 10 homolog C isoform X1 K17654 TRMT10C; mitochondrial ribonuclease P protein 1 [EC:2.1.1.-]
D 480930 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 490650 PRORP; mitochondrial ribonuclease P catalytic subunit isoform X2 K17655 MRPP3; mitochondrial ribonuclease P protein 3
C Other mitochondrial DNA transcription and translation factors
D 479247 MSS51; putative protein MSS51 homolog, mitochondrial K17656 MSS51; mitochondrial splicing suppressor protein 51
D 480615 TEFM; transcription elongation factor, mitochondrial isoform X1 K17658 TEFM; transcription elongation factor, mitochondrial
D 479742 ATP5J2; pentatricopeptide repeat-containing protein 1, mitochondrial isoform X11 K17710 PTCD1; pentatricopeptide repeat domain-containing protein 1
D 610392 PTCD2; pentatricopeptide repeat-containing protein 2, mitochondrial isoform X3 K17711 PTCD2; pentatricopeptide repeat domain-containing protein 2
D 475765 PTCD3; pentatricopeptide repeat domain-containing protein 3, mitochondrial isoform X2 K17659 PTCD3; pentatricopeptide repeat domain-containing protein 3
D 476639 DHX30; ATP-dependent RNA helicase DHX30 isoform X2 K13185 DHX30; ATP-dependent RNA helicase DHX30 [EC:5.6.2.6]
D 608882 UQCC1; ubiquinol-cytochrome-c reductase complex assembly factor 1 isoform X1 K17662 CBP3; cytochrome b pre-mRNA-processing protein 3
D 481952 AFG1L; AFG1-like ATPase K18798 AFG1; peroxisome-assembly ATPase [EC:3.6.4.7]
D 474422 ATP23; mitochondrial inner membrane protease ATP23 homolog isoform X2 K18156 ATP23; mitochondrial inner membrane protease ATP23 [EC:3.4.24.-]
D 489018 SUPV3L1; ATP-dependent RNA helicase SUPV3L1, mitochondrial isoform X2 K17675 SUPV3L1; ATP-dependent RNA helicase SUPV3L1/SUV3 [EC:5.6.2.6]
D 486990 LACTB2; endoribonuclease LACTB2 K16639 LACTB2; endoribonuclease LACTB2 [EC:3.1.27.-]
D 475630 SLC25A4; ADP/ATP translocase 1 K05863 SLC25A4S; solute carrier family 25 (mitochondrial adenine nucleotide translocator), member 4/5/6/31
D 480830 SLC25A6; ADP/ATP translocase 3 K05863 SLC25A4S; solute carrier family 25 (mitochondrial adenine nucleotide translocator), member 4/5/6/31
D 492093 SLC25A5; ADP/ATP translocase 2 K05863 SLC25A4S; solute carrier family 25 (mitochondrial adenine nucleotide translocator), member 4/5/6/31
D 483832 SLC25A31; ADP/ATP translocase 4 K05863 SLC25A4S; solute carrier family 25 (mitochondrial adenine nucleotide translocator), member 4/5/6/31
D 486837 SLC25A28; mitoferrin-2 isoform X2 K15113 SLC25A28_37; solute carrier family 25 (mitochondrial iron transporter), member 28/37
D 486115 SLC25A37; mitoferrin-1 isoform X2 K15113 SLC25A28_37; solute carrier family 25 (mitochondrial iron transporter), member 28/37
D 611116 MALSU1; mitochondrial assembly of ribosomal large subunit protein 1 K25803 MALSU1; mitochondrial assembly of ribosomal large subunit protein 1
D 480629 ERAL1; GTPase Era, mitochondrial K03595 era; GTPase
D 478148 YARS1; tyrosine--tRNA ligase, cytoplasmic isoform X2 K01866 YARS; tyrosyl-tRNA synthetase [EC:6.1.1.1]
D 486614 YARS2; tyrosine--tRNA ligase, mitochondrial K01866 YARS; tyrosyl-tRNA synthetase [EC:6.1.1.1]
D 476652 LARS2; probable leucine--tRNA ligase, mitochondrial isoform X1 K01869 LARS; leucyl-tRNA synthetase [EC:6.1.1.4]
D 478051 LARS1; leucine--tRNA ligase, cytoplasmic isoform X2 K01869 LARS; leucyl-tRNA synthetase [EC:6.1.1.4]
D 490337 DARS2; aspartate--tRNA ligase, mitochondrial isoform X1 K01876 DARS2; aspartyl-tRNA synthetase [EC:6.1.1.12]
D 100856659 GARS1; glycine--tRNA ligase K01880 GARS; glycyl-tRNA synthetase [EC:6.1.1.14]
D 609803 RARS1; arginine--tRNA ligase, cytoplasmic isoform X2 K01887 RARS; arginyl-tRNA synthetase [EC:6.1.1.19]
D 481911 RARS2; probable arginine--tRNA ligase, mitochondrial isoform X2 K01887 RARS; arginyl-tRNA synthetase [EC:6.1.1.19]
D 475014 QRSL1; glutamyl-tRNA(Gln) amidotransferase subunit A, mitochondrial isoform X11 K02433 gatA; aspartyl-tRNA(Asn)/glutamyl-tRNA(Gln) amidotransferase subunit A [EC:6.3.5.6 6.3.5.7]
D 475464 GATB; glutamyl-tRNA(Gln) amidotransferase subunit B, mitochondrial isoform X2 K02434 gatB; aspartyl-tRNA(Asn)/glutamyl-tRNA(Gln) amidotransferase subunit B [EC:6.3.5.6 6.3.5.7]
D 477509 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 477963 MTPAP; poly(A) RNA polymerase, mitochondrial K18060 MTPAP; poly(A) RNA polymerase, mitochondrial [EC:2.7.7.19]
D 484714 PDE12; 2',5'-phosphodiesterase 12 K19612 PDE12; 2',5'-phosphodiesterase [EC:3.1.13.4 3.1.4.-]
D 607676 MTG1; mitochondrial ribosome-associated GTPase 1 K19828 MTG1; mitochondrial GTPase 1
D 611447 NOA1; nitric oxide-associated protein 1 K19832 NOA1; nitric oxide-associated protein 1
D 607320 NSUN3; tRNA (cytosine(34)-C(5))-methyltransferase, mitochondrial isoform X2 K21969 NSUN3; tRNA (cytosine34-C5)-methyltransferase [EC:2.1.1.-]
D 610446 NSUN4; 5-methylcytosine rRNA methyltransferase NSUN4 isoform X5 K21970 NSUN4; 5-methylcytosine rRNA methyltransferase NSUN4 [EC:2.1.1.-]
D 484663 RPUSD3; mitochondrial mRNA pseudouridine synthase RPUSD3 isoform X2 K22537 RPUSD3; mitochondrial RNA pseudouridine synthase RPUSD3 [EC:5.4.99.-]
D 489295 RPUSD4; mitochondrial RNA pseudouridine synthase RPUSD4 K22538 RPUSD4; mitochondrial RNA pseudouridine synthase RPUSD4 [EC:5.4.99.-]
D 489793 RCC1L; RCC1-like G exchanging factor-like protein isoform X3 K23495 RCC1L; RCC1-like G exchanging factor-like protein
D 100684886 NGRN; neugrin K23496 NGRN; neugrin
D 608574 TRUB2; mitochondrial mRNA pseudouridine synthase TRUB2 K23497 TRUB2; mitochondrial mRNA pseudouridine synthase TRUB2 [EC:5.4.99.-]
D 478884 FASTKD2; FAST kinase domain-containing protein 2, mitochondrial isoform X1 K18190 FASTKD2; FAST kinase domain-containing protein 2
D 486249 C26H12orf65; probable peptide chain release factor C12orf65 homolog, mitochondrial isoform X1 K23498 COXPD7; peptide chain release factor
D 476247 RMND1; required for meiotic nuclear division protein 1 homolog isoform X1 K23499 RMND1; required for meiotic nuclear division protein 1
D 475170 GRSF1; LOW QUALITY PROTEIN: G-rich sequence factor 1 K24984 GRSF1; G-rich sequence factor 1
D 476897 METTL15; 12S rRNA N4-methylcytidine (m4C) methyltransferase isoform X6 K25876 METTL15; 12S rRNA N4-methylcytidine (m4C) methyltransferase [EC:2.1.1.-]
D 607875 MYG1; MYG1 exonuclease K25877 MYG1; MYG1 exonuclease [EC:3.1.-.-]
D 100687319 MTRES1; mitochondrial transcription rescue factor 1 isoform X3 K26186 MTRES1; mitochondrial transcription rescue factor 1
B Mitochondrial DNA replication factors
C Mitochondrial DNA polymerase
D 100856383 POLG; DNA polymerase subunit gamma-1 isoform X1 K02332 POLG; DNA polymerase gamma 1 [EC:2.7.7.7]
D 490910 POLG2; DNA polymerase subunit gamma-2, mitochondrial isoform X2 K02333 POLG2; DNA polymerase gamma 2
D 475632 PRIMPOL; DNA-directed primase/polymerase protein isoform X1 K22761 PRIMPOL; DNA-directed primase/polymerase protein [EC:2.7.7.102 2.7.7.7]
C Mitochondrial DNA maintenance factors
D 608916 ENDOG; endonuclease G, mitochondrial K01173 ENDOG; endonuclease G, mitochondrial
D 100855673 MGME1; mitochondrial genome maintenance exonuclease 1 K19465 MGME1; mitochondrial genome maintenance exonuclease 1 [EC:3.1.-.-]
D 485591 EXOG; nuclease EXOG, mitochondrial isoform X1 K15050 EXOG; nuclease EXOG, mitochondrial
D 100686111 LOW QUALITY PROTEIN: nuclease EXOG, mitochondrial-like K15050 EXOG; nuclease EXOG, mitochondrial
D 486845 TWNK; twinkle protein, mitochondrial isoform X4 K17680 PEO1; twinkle protein [EC:5.6.2.3]
D 100856387 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 475524 SSBP1; single-stranded DNA-binding protein, mitochondrial K03111 ssb; single-strand DNA-binding protein
D 479568 ATAD3A; ATPase family AAA domain-containing protein 3A isoform X2 K17681 ATAD3A_B; ATPase family AAA domain-containing protein 3A/B
D 100683221 UQCC2; ubiquinol-cytochrome-c reductase complex assembly factor 2 K17682 MNF1; mitochondrial nucleoid factor 1
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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 478143 DNAJA2; dnaJ homolog subfamily A member 2 K09503 DNAJA2; DnaJ homolog subfamily A member 2
D 477239 TOMM34; mitochondrial import receptor subunit TOM34 K17766 TOM34; mitochondrial import receptor subunit TOM34
D 480613 RHOT1; mitochondrial Rho GTPase 1 isoform X4 K07870 RHOT1; mitochondrial Rho GTPase 1 [EC:3.6.5.-]
D 474850 heat shock 70 kDa protein 1-like isoform X1 K03283 HSPA1_6_8; heat shock 70kDa protein 1/6/8
D 403612 HSP70; heat shock 70 kDa protein 1 K03283 HSPA1_6_8; heat shock 70kDa protein 1/6/8
D 479406 HSPA8; heat shock cognate 71 kDa protein K03283 HSPA1_6_8; heat shock 70kDa protein 1/6/8
D 119873859 LOW QUALITY PROTEIN: heat shock cognate 71 kDa protein-like isoform X2 K03283 HSPA1_6_8; heat shock 70kDa protein 1/6/8
D 607182 heat shock cognate 71 kDa protein-like K03283 HSPA1_6_8; heat shock 70kDa protein 1/6/8
D 489447 AIPL1; aryl-hydrocarbon-interacting protein-like 1 isoform X1 K17767 AIP; AH receptor-interacting protein
D 610467 AIP; AH receptor-interacting protein isoform X2 K17767 AIP; AH receptor-interacting protein
B Outer membrane
C Transporter outer membrane (TOM) complex
D 611055 TOMM40; mitochondrial import receptor subunit TOM40 homolog K11518 TOM40; mitochondrial import receptor subunit TOM40
D 609819 TOMM40L; mitochondrial import receptor subunit TOM40B isoform X2 K11518 TOM40; mitochondrial import receptor subunit TOM40
D 478541 TOMM70; mitochondrial import receptor subunit TOM70 isoform X2 K17768 TOM70; mitochondrial import receptor subunit TOM70
D 100855486 mitochondrial import receptor subunit TOM22 homolog K17769 TOM22; mitochondrial import receptor subunit TOM22
D 610117 TOMM22; mitochondrial import receptor subunit TOM22 homolog isoform X1 K17769 TOM22; mitochondrial import receptor subunit TOM22
D 100682999 TOMM20; mitochondrial import receptor subunit TOM20 homolog K17770 TOM20; mitochondrial import receptor subunit TOM20
D 611183 TOMM20L; TOMM20-like protein 1 K17770 TOM20; mitochondrial import receptor subunit TOM20
D 482351 TOMM7; mitochondrial import receptor subunit TOM7 homolog K17771 TOM7; mitochondrial import receptor subunit TOM7
D 609079 TOMM6; mitochondrial import receptor subunit TOM6 homolog K17772 TOM6; mitochondrial import receptor subunit TOM6
D 111097666 mitochondrial import receptor subunit TOM6 homolog K17772 TOM6; mitochondrial import receptor subunit TOM6
C TOB/SAM complex
D 474473 SAMM50; sorting and assembly machinery component 50 homolog K07277 SAM50; outer membrane protein insertion porin family
C Porin
D 474681 VDAC1; voltage-dependent anion-selective channel protein 1 K05862 VDAC1; voltage-dependent anion channel protein 1
D 102152873 LOW QUALITY PROTEIN: voltage-dependent anion-selective channel protein 1-like K05862 VDAC1; voltage-dependent anion channel protein 1
D 479255 VDAC2; voltage-dependent anion-selective channel protein 2 isoform X1 K15040 VDAC2; voltage-dependent anion channel protein 2
D 606963 VDAC3; voltage-dependent anion-selective channel protein 3 isoform 1 K15041 VDAC3; voltage-dependent anion channel protein 3
C ERMES complex
C Other outer membrane factor
D 480130 MTX1; metaxin-1 isoform X1 K17776 MTX; metaxin
D 488931 MTX3; metaxin-3 isoform X4 K17776 MTX; metaxin
D 478811 MTX2; metaxin-2 isoform X1 K17776 MTX; metaxin
D 100855669 MYO19; unconventional myosin-XIX isoform X1 K25838 MYO19; myosin XIX
D 478967 MTARC2; mitochondrial amidoxime reducing component 2 isoform X1 K27318 MTARC; mitochondrial amidoxime-reducing component [EC:1.7.-.-]
D 488601 MTARC1; 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 609619 TIMM9; mitochondrial import inner membrane translocase subunit Tim9 K17777 TIM9; mitochondrial import inner membrane translocase subunit TIM9
D 106559491 mitochondrial import inner membrane translocase subunit Tim9-like K17777 TIM9; mitochondrial import inner membrane translocase subunit TIM9
D 475968 TIMM10; mitochondrial import inner membrane translocase subunit Tim10 K17778 TIM10; mitochondrial import inner membrane translocase subunit TIM10
D 610986 TIMM10B; mitochondrial import inner membrane translocase subunit Tim10 B K17779 TIM10B; mitochondrial import inner membrane translocase subunit TIM10B
C TIM8/13 complex
D 611348 TIMM8B; mitochondrial import inner membrane translocase subunit Tim8 B K17780 TIM8; mitochondrial import inner membrane translocase subunit TIM8
D 119864597 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 476520 CHCHD4; mitochondrial intermembrane space import and assembly protein 40 K17782 MIA40; mitochondrial intermembrane space import and assembly protein 40
D 119874932 mitochondrial intermembrane space import and assembly protein 40-like K17782 MIA40; mitochondrial intermembrane space import and assembly protein 40
D 479885 GFER; FAD-linked sulfhydryl oxidase ALR K17783 ERV1; mitochondrial FAD-linked sulfhydryl oxidase [EC:1.8.3.2]
B Inner mambrane
C MICOS complex
D 612968 MICOS10; MICOS complex subunit MIC10 K17784 MICOS10; MICOS complex subunit MIC10
D 475764 IMMT; MICOS complex subunit MIC60 isoform X2 K17785 IMMT; MICOS complex subunit MIC60
D 476739 MICOS13; MICOS complex subunit MIC13 K24624 MICOS13; MICOS complex subunit MIC13
D 607574 CHCHD3; MICOS complex subunit MIC19 isoform X2 K17563 CHCHD3; MICOS complex subunit MIC19
D 476509 CHCHD6; MICOS complex subunit MIC25 isoform X2 K17564 CHCHD6; MICOS complex subunit MIC25
D 102152929 MICOS complex subunit MIC26-like K24625 APOO; MICOS complex subunit MIC26
D 119870656 MICOS complex subunit MIC26 K24625 APOO; MICOS complex subunit MIC26
D 608758 APOOL; MICOS complex subunit MIC27 isoform X1 K24626 APOOL; MICOS complex subunit MIC27
C MICOS complex (yeast)
D 612968 MICOS10; MICOS complex subunit MIC10 K17784 MICOS10; MICOS complex subunit MIC10
D 475764 IMMT; MICOS complex subunit MIC60 isoform X2 K17785 IMMT; MICOS complex subunit MIC60
C TIM22 complex
D 480639 TIMM22; mitochondrial import inner membrane translocase subunit Tim22 K17790 TIM22; mitochondrial import inner membrane translocase subunit TIM22
C TIM23 complex
D 486776 mitochondrial import inner membrane translocase subunit Tim23 K17794 TIM23; mitochondrial import inner membrane translocase subunit TIM23
D 100686615 mitochondrial import inner membrane translocase subunit Tim23-like K17794 TIM23; mitochondrial import inner membrane translocase subunit TIM23
D 480001 TIMM17A; mitochondrial import inner membrane translocase subunit Tim17-A isoform X2 K17795 TIM17; mitochondrial import inner membrane translocase subunit TIM17
D 480908 TIMM17B; mitochondrial import inner membrane translocase subunit Tim17-B isoform X2 K17795 TIM17; mitochondrial import inner membrane translocase subunit TIM17
D 476170 TIMM21; mitochondrial import inner membrane translocase subunit Tim21 K17796 TIM21; mitochondrial import inner membrane translocase subunit TIM21
D 476461 TIMM50; mitochondrial import inner membrane translocase subunit TIM50 isoform X1 K17496 TIM50; mitochondrial import inner membrane translocase subunit TIM50
C Mitochondrial calcium uniporter complex
D 489042 MCU; calcium uniporter protein, mitochondrial isoform X1 K20858 MCU; calcium uniporter protein, mitochondrial
D 479241 MICU1; calcium uptake protein 1, mitochondrial isoform X2 K22827 MICU1; calcium uptake protein 1, mitochondrial
D 486044 MICU2; calcium uptake protein 2, mitochondrial K22828 MICU2; calcium uptake protein 2, mitochondrial
D 478513 MCUB; calcium uniporter regulatory subunit MCUb, mitochondrial isoform X1 K22829 MCUB; calcium uniporter regulatory subunit MCUb, mitochondrial
D 609643 SMDT1; essential MCU regulator, mitochondrial K22830 SMDT1; essential MCU regulator, mitochondrial
D 610095 MCUR1; mitochondrial calcium uniporter regulator 1 K22137 MCUR1; mitochondrial calcium uniporter regulator 1
C Other inner membrane factors
D 488807 LETM1; mitochondrial proton/calcium exchanger protein isoform X1 K17800 LETM1; LETM1 and EF-hand domain-containing protein 1, mitochondrial
D 475954 IMMP1L; mitochondrial inner membrane protease subunit 1 isoform X1 K09647 IMP1; mitochondrial inner membrane protease subunit 1 [EC:3.4.21.-]
D 611911 IMMP2L; mitochondrial inner membrane protease subunit 2 isoform X1 K09648 IMP2; mitochondrial inner membrane protease subunit 2 [EC:3.4.21.-]
D 608427 TMEM126A; transmembrane protein 126A K18157 TMEM126A; transmembrane protein 126A
D 480882 MPC1L; mitochondrial pyruvate carrier 1-like protein K22138 MPC1; mitochondrial pyruvate carrier 1
D 609356 MPC1; mitochondrial pyruvate carrier 1 isoform X2 K22138 MPC1; mitochondrial pyruvate carrier 1
D 480086 MPC2; mitochondrial pyruvate carrier 2 K22139 MPC2; mitochondrial pyruvate carrier 2
D 477560 CHCHD10; LOW QUALITY PROTEIN: coiled-coil-helix-coiled-coil-helix domain-containing protein 10, mitochondrial K22759 CHCHD10; coiled-coil-helix-coiled-coil-helix domain-containing protein 10
B Matrix
C MMC/PAM proteins
D 485005 TIMM44; mitochondrial import inner membrane translocase subunit TIM44 K17804 TIM44; mitochondrial import inner membrane translocase subunit TIM44
D 474697 HSPA9; stress-70 protein, mitochondrial isoform X1 K04043 dnaK; molecular chaperone DnaK
D 488784 GRPEL1; grpE protein homolog 1, mitochondrial K03687 GRPE; molecular chaperone GrpE
D 489195 GRPEL2; grpE protein homolog 2, mitochondrial K03687 GRPE; molecular chaperone GrpE
D 100856039 DNAJC19; mitochondrial import inner membrane translocase subunit TIM14 K09539 DNAJC19; DnaJ homolog subfamily C member 19
D 100685102 LOW QUALITY PROTEIN: mitochondrial import inner membrane translocase subunit TIM14-like K09539 DNAJC19; DnaJ homolog subfamily C member 19
D 444856 PAM16; mitochondrial import inner membrane translocase subunit TIM16 isoform X1 K17805 PAM16; mitochondrial import inner membrane translocase subunit TIM16
C Other matrix factors
D 480677 PMPCA; mitochondrial-processing peptidase subunit alpha isoform X2 K01412 PMPCA; mitochondrial-processing peptidase subunit alpha [EC:3.4.24.64]
D 475897 PMPCB; mitochondrial-processing peptidase subunit beta K17732 PMPCB; mitochondrial-processing peptidase subunit beta [EC:3.4.24.64]
D 477338 MIPEP; mitochondrial intermediate peptidase isoform X1 K01410 MIPEP; mitochondrial intermediate peptidase [EC:3.4.24.59]
D 478854 HSPD1; 60 kDa heat shock protein, mitochondrial K04077 groEL; chaperonin GroEL [EC:5.6.1.7]
D 478855 HSPE1; 10 kDa heat shock protein, mitochondrial isoform X4 K04078 groES; chaperonin GroES
D 609748 10 kDa heat shock protein, mitochondrial K04078 groES; chaperonin GroES
D 100685171 10 kDa heat shock protein, mitochondrial-like K04078 groES; chaperonin GroES
D 100685272 10 kDa heat shock protein, mitochondrial-like K04078 groES; chaperonin GroES
D 100687092 10 kDa heat shock protein, mitochondrial-like K04078 groES; chaperonin GroES
D 479865 DNAJA3; dnaJ homolog subfamily A member 3, mitochondrial isoform X3 K09504 DNAJA3; DnaJ homolog subfamily A member 3
D 476532 TAMM41; phosphatidate cytidylyltransferase, mitochondrial K17807 TAM41; mitochondrial translocator assembly and maintenance protein 41
D 607502 DNLZ; DNL-type zinc finger protein K17808 ZIM17; mitochondrial protein import protein ZIM17
D 612416 ETHE1; persulfide dioxygenase ETHE1, mitochondrial K17725 ETHE1; sulfur dioxygenase [EC:1.13.11.18]
D 491162 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 607922 CREB1; cyclic AMP-responsive element-binding protein 1 isoform X1 K05870 CREB1; cyclic AMP-responsive element-binding protein 1
D 478101 GFM2; ribosome-releasing factor 2, mitochondrial isoform X1 K02355 fusA; elongation factor G
D 477128 GFM1; elongation factor G, mitochondrial K02355 fusA; elongation factor G
D 486507 SP1; transcription factor Sp1 isoform X2 K04684 SP1; transcription factor Sp1
D 611171 YY1; transcriptional repressor protein YY1 K09201 YY; transcription factor YY
D 608465 GABPB1; GA-binding protein subunit beta-1 isoform X2 K09454 GABPB; GA-binding protein transcription factor, beta
D 475841 GABPB2; GA-binding protein subunit beta-2 isoform X1 K09454 GABPB; GA-binding protein transcription factor, beta
D 488989 TFAM; transcription factor A, mitochondrial K11830 TFAM; transcription factor A, mitochondrial
C Nuclear respiratory factors
D 482260 NRF1; nuclear respiratory factor 1 isoform X2 K11831 NRF1; nuclear respiratory factor 1
D 478813 NFE2L2; nuclear factor erythroid 2-related factor 2 isoform X3 K05638 NFE2L2; nuclear factor erythroid 2-related factor 2
D 403768 MT2A; metallothionein-2 K14739 MT1_2; metallothionein 1/2
D 403800 MT1E; metallothionein-1 K14739 MT1_2; metallothionein 1/2
D 100686073 metallothionein-1 K14739 MT1_2; metallothionein 1/2
D 403769 MT4; metallothionein-4 K14741 MT4; metallothionein 4
C Coactivators
D 479127 PPARGC1A; peroxisome proliferator-activated receptor gamma coactivator 1-alpha isoform X3 K07202 PPARGC1A; alpha peroxisome proliferator-activated receptor gamma coactivator 1-alpha
D 489190 PPARGC1B; peroxisome proliferator-activated receptor gamma coactivator 1-beta isoform X1 K17962 PPARGC1B; peroxisome proliferator-activated receptor gamma coactivator 1-beta
D 477805 PPRC1; peroxisome proliferator-activated receptor gamma coactivator-related protein 1 isoform X5 K17963 PPRC1; peroxisome proliferator-activated receptor gamma coactivator-related protein 1
D 100684638 CRTC1; CREB-regulated transcription coactivator 1 isoform X1 K15309 CRTC1; CREB-regulated transcription coactivator 1
D 479866 CREBBP; CREB-binding protein isoform X8 K04498 EP300; E1A/CREB-binding protein [EC:2.3.1.48]
D 100684945 EP300; histone acetyltransferase p300 isoform X1 K04498 EP300; E1A/CREB-binding protein [EC:2.3.1.48]
C Proteins involved in Fe-S cluster biogenesis
D 477214 NFS1; cysteine desulfurase, mitochondrial isoform X2 K04487 iscS; cysteine desulfurase [EC:2.8.1.7]
D 477527 ISCU; iron-sulfur cluster assembly enzyme ISCU, mitochondrial K22068 ISCU; iron-sulfur cluster assembly enzyme ISCU, mitochondrial
D 102153000 LYRM4; LYR motif-containing protein 4 isoform X1 K22069 LYRM4; LYR motif-containing protein 4
D 609051 FXN; frataxin, mitochondrial K19054 FXN; frataxin [EC:1.16.3.1]
D 100686950 FDX1; adrenodoxin, mitochondrial isoform X1 K22070 FDX1; ferredoxin-1, mitochondrial
D 484957 FDX2; ferredoxin-2, mitochondrial K22071 FDX2; ferredoxin-2, mitochondrial
D 475910 FDXR; NADPH:adrenodoxin oxidoreductase, mitochondrial K18914 FDXR; adrenodoxin-NADP+ reductase [EC:1.18.1.6]
D 474697 HSPA9; stress-70 protein, mitochondrial isoform X1 K04043 dnaK; molecular chaperone DnaK
D 477531 HSCB; iron-sulfur cluster co-chaperone protein HscB isoform X2 K04082 hscB; molecular chaperone HscB
D 474699 SIL1; nucleotide exchange factor SIL1 isoform X1 K14001 SIL1; nucleotide exchange factor SIL1
D 612417 GLRX5; glutaredoxin-related protein 5, mitochondrial K07390 grxD; monothiol glutaredoxin
D 490783 ISCA2; iron-sulfur cluster assembly 2 homolog, mitochondrial K22072 ISCA2; iron-sulfur cluster assembly 2
D 482207 IBA57; putative transferase CAF17, mitochondrial isoform X2 K22073 IBA57; transferase CAF17, mitochondrial [EC:2.1.-.-]
D 612607 NFU1; NFU1 iron-sulfur cluster scaffold homolog, mitochondrial isoform X1 K22074 NFU1; NFU1 iron-sulfur cluster scaffold homolog, mitochondrial
D 100855629 BOLA1; bolA-like protein 1 K22066 BOLA1; BolA-like protein 1
D 612287 BOLA3; bolA-like protein 3 K22075 BOLA3; BolA-like protein 3
D 486848 SFXN3; sideroflexin-3 K23500 SFXN1_3; sideroflexin-1/3
D 489109 SFXN1; sideroflexin-1 isoform X3 K23500 SFXN1_3; sideroflexin-1/3
D 100855721 SFXN2; sideroflexin-2 isoform X2 K23501 SFXN2; sideroflexin-2
D 477838 SFXN4; sideroflexin-4 isoform X2 K23502 SFXN4; sideroflexin-4
D 612350 SFXN5; sideroflexin-5 isoform X1 K23503 SFXN5; sideroflexin-5
C Other regulator of mitochondrial biogenesis
D 403583 CPT1A; carnitine O-palmitoyltransferase 1, liver isoform K08765 CPT1A; carnitine O-palmitoyltransferase 1, liver isoform [EC:2.3.1.21]
D 481184 CPT1B; carnitine O-palmitoyltransferase 1, muscle isoform isoform X1 K19523 CPT1B; carnitine O-palmitoyltransferase 1, muscle isoform [EC:2.3.1.21]
D 100856778 CPT1C; carnitine O-palmitoyltransferase 1, brain isoform isoform X1 K19524 CPT1C; carnitine O-palmitoyltransferase 1, brain isoform [EC:2.3.1.21]
D 478008 DHTKD1; probable 2-oxoglutarate dehydrogenase E1 component DHKTD1, mitochondrial K15791 DHKTD1; 2-oxoadipate dehydrogenase E1 component [EC:1.2.4.-]
D 481932 FBXL4; F-box/LRR-repeat protein 4 K10270 FBXL4; F-box and leucine-rich repeat protein 4
D 492246 HCFC1; host cell factor 1 isoform X1 K14966 HCFC1; host cell factor 1
D 475449 HCFC2; host cell factor 2 isoform X2 K27390 HCFC2; host cell factor 2
D 479074 HTT; huntingtin isoform X2 K04533 HD; huntingtin
D 403169 ESRRA; steroid hormone receptor ERR1 K08552 NR3B1; estrogen-related receptor alpha
D 490798 ESRRB; steroid hormone receptor ERR2 isoform X3 K08553 NR3B2; estrogen-related receptor beta
D 478961 ESRRG; estrogen-related receptor gamma isoform X1 K08554 NR3B3; estrogen-related receptor gamma
D 478385 NRIP1; nuclear receptor-interacting protein 1 isoform X1 K17965 NRIP1; nuclear receptor-interacting protein 1
D 479351 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 489571 PRKAA2; 5'-AMP-activated protein kinase catalytic subunit alpha-2 isoform X2 K07198 PRKAA; 5'-AMP-activated protein kinase, catalytic alpha subunit [EC:2.7.11.31]
D 611425 LONP1; lon protease homolog, mitochondrial K08675 PRSS15; ATP-dependent Lon protease [EC:3.4.21.53]
D 489012 SIRT1; NAD-dependent protein deacetylase sirtuin-1 isoform X2 K11411 SIRT1; NAD-dependent protein deacetylase sirtuin 1 [EC:2.3.1.286]
D 476255 SERAC1; protein SERAC1 isoform X2 K23504 SERAC1; protein SERAC1
D 480900 UXT; protein UXT isoform X5 K26537 UXT; protein UXT
D 489605 PERM1; PGC-1 and ERR-induced regulator in muscle protein 1 isoform X5 K26738 PERM1; PGC-1 and ERR-induced regulator in muscle protein 1
B Mitochondrial respiratory chain complex assembly factors
C Complex-I assembly factors
D 487505 NDUFAF1; complex I intermediate-associated protein 30, mitochondrial K18159 NDUFAF1; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 1
D 478067 NDUFAF2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 2 K18160 NDUFAF2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 2
D 476631 NDUFAF3; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 3 K09008 NDUFAF3; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 3
D 475001 NDUFAF4; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 4 isoform X1 K18161 NDUFAF4; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 4
D 477150 NDUFAF5; arginine-hydroxylase NDUFAF5, mitochondrial isoform X2 K18162 NDUFAF5; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 5 [EC:2.1.1.-]
D 477943 NDUFAF6; NADH dehydrogenase (ubiquinone) complex I, assembly factor 6 isoform X2 K18163 NDUFAF6; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 6
D 475725 NDUFAF7; protein arginine methyltransferase NDUFAF7, mitochondrial isoform X1 K18164 NDUFAF7; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 7
D 100688960 NDUFAF8; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 8 isoform X2 K24726 NDUFAF8; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 8
D 485156 TMEM126B; complex I assembly factor TMEM126B, mitochondrial K18165 TMEM126B; complex I assembly factor TMEM126B
D 484938 ECSIT; evolutionarily conserved signaling intermediate in Toll pathway, mitochondrial K04405 ECSIT; evolutionarily conserved signaling intermediate in Toll pathway
D 476519 ACAD9; complex I assembly factor ACAD9, mitochondrial isoform X2 K15980 ACAD9; acyl-CoA dehydrogenase family member 9 [EC:1.3.99.-]
D 609340 NUBPL; iron-sulfur protein NUBPL isoform X1 K03593 mrp; ATP-binding protein involved in chromosome partitioning
D 100856312 FOXRED1; FAD-dependent oxidoreductase domain-containing protein 1 K18166 FOXRED1; FAD-dependent oxidoreductase domain-containing protein 1
D 475013 RTN4IP1; reticulon-4-interacting protein 1, mitochondrial isoform X1 K23164 RTN4IP1; reticulon-4-interacting protein 1, mitochondrial
D 487994 TIMMDC1; complex I assembly factor TIMMDC1, mitochondrial K23505 TIMMDC1; complex I assembly factor TIMMDC1
C Complex-II assembly factors
D 100684432 succinate dehydrogenase assembly factor 1, mitochondrial-like K18167 SDHAF1; succinate dehydrogenase assembly factor 1
D 484567 SDHAF1; succinate dehydrogenase assembly factor 1, mitochondrial K18167 SDHAF1; succinate dehydrogenase assembly factor 1
D 476065 SDHAF2; succinate dehydrogenase assembly factor 2, mitochondrial isoform X2 K18168 SDHAF2; succinate dehydrogenase assembly factor 2
C Complex-III assembly factors
D 478911 BCS1L; mitochondrial chaperone BCS1 K08900 BCS1; mitochondrial chaperone BCS1
D 479516 TTC19; tetratricopeptide repeat protein 19, mitochondrial K18169 TTC19; tetratricopeptide repeat protein 19, mitochondrial
D 609480 LYRM7; complex III assembly factor LYRM7 isoform X1 K18170 LYRM7; complex III assembly factor LYRM7
D 119870310 complex III assembly factor LYRM7-like K18170 LYRM7; complex III assembly factor LYRM7
D 608882 UQCC1; ubiquinol-cytochrome-c reductase complex assembly factor 1 isoform X1 K17662 CBP3; cytochrome b pre-mRNA-processing protein 3
D 100683221 UQCC2; ubiquinol-cytochrome-c reductase complex assembly factor 2 K17682 MNF1; mitochondrial nucleoid factor 1
D 612210 UQCC3; ubiquinol-cytochrome-c reductase complex assembly factor 3 K23165 UQCC3; ubiquinol-cytochrome-c reductase complex assembly factor 3
D 611188 C6H16orf91; protein CCSMST1 K27339 UQCC4; ubiquinol-cytochrome c reductase complex assembly factor 4
D 100856540 SMIM4; small integral membrane protein 4 K27340 UQCC5; ubiquinol-cytochrome c reductase complex assembly factor 5
D 100685280 C15H12orf73; protein BRAWNIN K27341 UQCC6; ubiquinol-cytochrome c reductase complex assembly factor 6
D 610249 STMP1; short transmembrane mitochondrial protein 1 isoform X5 K27319 STMP1; short transmembrane mitochondrial protein 1
D 102154691 short transmembrane mitochondrial protein 1 K27319 STMP1; short transmembrane mitochondrial protein 1
C Complex-IV assembly factors
D 485633 CMC1; COX assembly mitochondrial protein homolog isoform X5 K18171 CMC1; COX assembly mitochondrial protein 1
D 102155476 COX assembly mitochondrial protein 2 homolog K18172 CMC2; COX assembly mitochondrial protein 2
D 481143 CMC2; COX assembly mitochondrial protein 2 homolog K18172 CMC2; COX assembly mitochondrial protein 2
D 100685891 LOW QUALITY PROTEIN: COX assembly mitochondrial protein 2 homolog K18172 CMC2; COX assembly mitochondrial protein 2
D 106557454 COX assembly mitochondrial protein 2 homolog K18172 CMC2; COX assembly mitochondrial protein 2
D 608597 COA1; cytochrome c oxidase assembly factor 1 homolog K18173 COA1; cytochrome c oxidase assembly factor 1
D 480514 CCDC56; coiled-coil domain-containing protein 56 K18175 CCDC56; cytochrome c oxidase assembly factor 3, animal type
D 100685149 CHCHD8; cytochrome c oxidase assembly factor 4 homolog, mitochondrial isoform X2 K18177 COA4; cytochrome c oxidase assembly factor 4
D 611656 COA5; cytochrome c oxidase assembly factor 5 K18178 COA5; cytochrome c oxidase assembly factor 5
D 607627 COA6; cytochrome c oxidase assembly factor 6 homolog K18179 COA6; cytochrome c oxidase assembly factor 6
D 482496 COA7; cytochrome c oxidase assembly factor 7 K18180 COA7; cytochrome c oxidase assembly factor 7
D 489515 COX10; protoheme IX farnesyltransferase, mitochondrial K02257 COX10; heme o synthase [EC:2.5.1.141]
D 609555 COX11; cytochrome c oxidase assembly protein COX11, mitochondrial K02258 COX11; cytochrome c oxidase assembly protein subunit 11
D 100688544 COX14; cytochrome c oxidase assembly protein COX14 K18181 COX14; cytochrome c oxidase assembly factor 14
D 477792 COX15; cytochrome c oxidase assembly protein COX15 homolog isoform X2 K02259 COX15; heme a synthase [EC:1.17.99.9]
D 119863880 cytochrome c oxidase assembly protein COX16 homolog, mitochondrial K18182 COX16; cytochrome c oxidase assembly protein subunit 16
D 503668 COX17; cytochrome c oxidase copper chaperone K02260 COX17; cytochrome c oxidase assembly protein subunit 17
D 482193 COX18; cytochrome c oxidase assembly protein COX18, mitochondrial isoform X9 K17797 COX18; mitochondrial inner membrane protein COX18
D 100685945 COX19; cytochrome c oxidase assembly protein COX19 K18183 COX19; cytochrome c oxidase assembly protein subunit 19
D 480099 COX20; cytochrome c oxidase assembly protein COX20, mitochondrial isoform X6 K18184 COX20; cytochrome c oxidase assembly protein subunit 20
D 111091070 LOW QUALITY PROTEIN: cytochrome c oxidase assembly protein COX20, mitochondrial-like K18184 COX20; cytochrome c oxidase assembly protein subunit 20
D 479247 MSS51; putative protein MSS51 homolog, mitochondrial K17656 MSS51; mitochondrial splicing suppressor protein 51
D 100685574 protein PET100 homolog, mitochondrial-like K18186 PET100; protein PET100, animal type
D 611528 protein PET100 homolog, mitochondrial K18186 PET100; protein PET100, animal type
D 100688040 protein PET117 homolog, mitochondrial K18188 PET117; protein PET117
D 480239 OXA1L; mitochondrial inner membrane protein OXA1L K03217 yidC; YidC/Oxa1 family membrane protein insertase
D 480681 SURF1; surfeit locus protein 1 isoform X1 K14998 SURF1; surfeit locus 1 family protein
D 489505 SCO1; protein SCO1 homolog, mitochondrial K07152 SCO1; protein SCO1
D 481187 protein SCO2 homolog, mitochondrial K23755 SCO2; protein SCO2
D 100856685 TACO1; translational activator of cytochrome c oxidase 1 K18189 TACO1; translational activator of cytochrome c oxidase 1
D 474572 LRPPRC; leucine-rich PPR motif-containing protein, mitochondrial K17964 LRPPRC; leucine-rich PPR motif-containing protein, mitochondrial
D 100687117 COA8; cytochrome c oxidase assembly factor 8 isoform X1 K23506 APOPT1; apoptogenic protein 1
D 610249 STMP1; short transmembrane mitochondrial protein 1 isoform X5 K27319 STMP1; short transmembrane mitochondrial protein 1
D 102154691 short transmembrane mitochondrial protein 1 K27319 STMP1; short transmembrane mitochondrial protein 1
D 100856540 SMIM4; small integral membrane protein 4 K27340 UQCC5; ubiquinol-cytochrome c reductase complex assembly factor 5
D 612187 TMEM223; transmembrane protein 223 K27342 TMEM223; transmembrane protein 223
C Complex-V assembly factors
D 610408 ATPAF1; ATP synthase mitochondrial F1 complex assembly factor 1 isoform X1 K07555 ATPeAF1; ATP synthase mitochondrial F1 complex assembly factor 1
D 489539 ATPAF2; ATP synthase mitochondrial F1 complex assembly factor 2 K07556 ATPeAF2; ATP synthase mitochondrial F1 complex assembly factor 2
D 477915 TMEM70; transmembrane protein 70, mitochondrial isoform X1 K17966 TMEM70; transmembrane protein 70, mitochondrial
D 100683089 ATP5MJ; ATP synthase subunit ATP5MPL, mitochondrial K18193 MP68; 6.8 kDa mitochondrial proteolipid
D 111098095 ATP synthase subunit ATP5MPL, mitochondrial-like K18193 MP68; 6.8 kDa mitochondrial proteolipid
D 119867826 ATP synthase membrane subunit DAPIT, mitochondrial-like K18194 USMG5; up-regulated during skeletal muscle growth protein 5
D 100682634 ATP5MK; ATP synthase membrane subunit DAPIT, mitochondrial K18194 USMG5; up-regulated during skeletal muscle growth protein 5
D 100683064 LOW QUALITY PROTEIN: ATP synthase membrane subunit DAPIT, mitochondrial-like K18194 USMG5; up-regulated during skeletal muscle growth protein 5
D 102156136 ATP synthase membrane subunit DAPIT, mitochondrial-like K18194 USMG5; up-regulated during skeletal muscle growth protein 5
B Mitochondrial dynamics
C Fission and Fusion factors
D 488086 MFN1; mitofusin-1 K21356 MFN1; mitofusin 1 [EC:3.6.5.-]
D 487439 MFN2; mitofusin-2 K06030 MFN2; mitofusin 2 [EC:3.6.5.-]
D 477129 OPA1; dynamin-like 120 kDa protein, mitochondrial isoform X3 K17079 OPA1; optic atrophy protein 1 [EC:3.6.5.5]
D 119863922 OPA3; optic atrophy 3 protein isoform X2 K23166 OPA3; optic atrophy 3 protein
D 489217 TRIAP1; TP53-regulated inhibitor of apoptosis 1 K17968 TRIAP1; TRIAP1/MDM35 family protein
D 111094306 TP53-regulated inhibitor of apoptosis 1-like K17968 TRIAP1; TRIAP1/MDM35 family protein
D 488100 PARL; presenilins-associated rhomboid-like protein, mitochondrial isoform X2 K09650 PARL; rhomboid-like protein [EC:3.4.21.105]
D 481744 BAK1; bcl-2 homologous antagonist/killer K14021 BAK; Bcl-2 homologous antagonist/killer
D 403523 BAX; apoptosis regulator BAX K02159 BAX; apoptosis regulator BAX
D 477649 DNM1L; dynamin-1-like protein isoform X1 K17065 DNM1L; dynamin 1-like protein [EC:3.6.5.5]
D 479728 FIS1; mitochondrial fission 1 protein K17969 FIS1; mitochondrial fission 1 protein
D 100856530 SUMO3; small ubiquitin-related modifier 3 isoform X3 K12160 SUMO; small ubiquitin-related modifier
D 474963 SUMO2; small ubiquitin-related modifier 2 K12160 SUMO; small ubiquitin-related modifier
D 478874 SUMO1; small ubiquitin-related modifier 1 K12160 SUMO; small ubiquitin-related modifier
D 119871262 small ubiquitin-related modifier 2-like isoform X1 K12160 SUMO; small ubiquitin-related modifier
D 490297 small ubiquitin-related modifier 2-like K12160 SUMO; small ubiquitin-related modifier
D 612505 SH3GLB1; endophilin-B1 isoform X5 K11248 SH3GLB1; endophilin-B1
D 475977 MTCH2; mitochondrial carrier homolog 2 isoform X1 K17885 MTCH; mitochondrial carrier
D 608507 MTCH1; mitochondrial carrier homolog 1 isoform X1 K17885 MTCH; mitochondrial carrier
D 102151293 LOW QUALITY PROTEIN: mitochondrial carrier homolog 2-like isoform X4 K17885 MTCH; mitochondrial carrier
D 477395 MFF; mitochondrial fission factor isoform X1 K22076 MFF; mitochondrial fission factor
D 485881 GDAP1L1; ganglioside-induced differentiation-associated protein 1-like 1 isoform X2 K22077 GDAP1; ganglioside-induced differentiation-associated protein 1
D 487002 GDAP1; ganglioside-induced differentiation-associated protein 1 isoform X2 K22077 GDAP1; ganglioside-induced differentiation-associated protein 1
D 490953 VAT1; synaptic vesicle membrane protein VAT-1 homolog K23167 VAT1; synaptic vesicle membrane protein VAT-1
D 609352 MIEF2; mitochondrial dynamics protein MID49 isoform X3 K23507 MIEF1_2; mitochondrial dynamics protein MID51/49
D 610014 MIEF1; mitochondrial dynamics protein MID51 isoform X1 K23507 MIEF1_2; mitochondrial dynamics protein MID51/49
D 489545 PLD6; mitochondrial cardiolipin hydrolase K16862 PLD6; mitochondrial cardiolipin hydrolase [EC:3.1.4.-]
D 479983 MIGA1; mitoguardin 1 isoform X2 K27289 MIGA; mitoguardin
D 491305 MIGA2; mitoguardin 2 isoform X3 K27289 MIGA; mitoguardin
C Actin cytoskeleton organization
D 403580 ACTB; actin, cytoplasmic 1 K05692 ACTB_G1; actin beta/gamma 1
D 475923 ACTG1; actin, cytoplasmic 2 K05692 ACTB_G1; actin beta/gamma 1
D 477138 NAA20; N-alpha-acetyltransferase 20 isoform X4 K17972 NAA20; N-terminal acetyltransferase B complex catalytic subunit [EC:2.3.1.254]
D 486274 NAA25; N-alpha-acetyltransferase 25, NatB auxiliary subunit K17973 NAA25; N-terminal acetyltransferase B complex non-catalytic subunit
C Mitochondrial movement
D 480547 PHB; prohibitin K17080 PHB1; prohibitin 1
D 486716 PHB2; prohibitin-2 K17081 PHB2; prohibitin 2
D 480332 KTN1; kinectin isoform X1 K17975 KTN1; kinectin
C Retention of mitochondria
D 477649 DNM1L; dynamin-1-like protein isoform X1 K17065 DNM1L; dynamin 1-like protein [EC:3.6.5.5]
D 478312 MYO5A; unconventional myosin-Va isoform X1 K10357 MYO5; myosin V
D 480220 MYO5B; unconventional myosin-Vb K10357 MYO5; myosin V
D 487555 MYO5C; unconventional myosin-Vc K10357 MYO5; myosin V
D 442989 XIAP; E3 ubiquitin-protein ligase XIAP K04725 XIAP; E3 ubiquitin-protein ligase XIAP [EC:2.3.2.27]
D 477463 DIABLO; diablo homolog, mitochondrial K10522 DIABLO; diablo
C Morphogenesis factors
D 475629 SNX25; sorting nexin-25 isoform X2 K17887 SNX25; sorting nexin-25
D 480613 RHOT1; mitochondrial Rho GTPase 1 isoform X4 K07870 RHOT1; mitochondrial Rho GTPase 1 [EC:3.6.5.-]
D 475764 IMMT; MICOS complex subunit MIC60 isoform X2 K17785 IMMT; MICOS complex subunit MIC60
D 486352 MTFP1; mitochondrial fission process protein 1 K17981 MTFP1; mitochondrial fission process protein 1
D 480127 DAP3; 28S ribosomal protein S29, mitochondrial isoform X4 K17408 DAP3; small subunit ribosomal protein S29
D 608738 NEDD4; E3 ubiquitin-protein ligase NEDD4 isoform X1 K10591 NEDD4; E3 ubiquitin-protein ligase NEDD4 [EC:2.3.2.26]
D 481998 OXR1; oxidation resistance protein 1 isoform X1 K25437 OXR1; oxidation resistance protein 1
D 490423 MSTO1; protein misato homolog 1 isoform X2 K27320 MSTO1; protein misato
B Mitophagy factors
C Autophagy-related proteins
D 480428 ATG2B; autophagy-related protein 2 homolog B isoform X1 K17906 ATG2; autophagy-related protein 2
D 483756 ATG2A; autophagy-related protein 2 homolog A isoform X2 K17906 ATG2; autophagy-related protein 2
D 478564 ATG3; ubiquitin-like-conjugating enzyme ATG3 isoform X1 K08343 ATG3; ubiquitin-like-conjugating enzyme ATG3
D 481015 ATG4A; cysteine protease ATG4A K08342 ATG4; cysteine protease ATG4 [EC:3.4.22.-]
D 609599 ATG4B; cysteine protease ATG4B isoform X3 K08342 ATG4; cysteine protease ATG4 [EC:3.4.22.-]
D 484953 ATG4D; cysteine protease ATG4D isoform X1 K08342 ATG4; cysteine protease ATG4 [EC:3.4.22.-]
D 489561 ATG4C; cysteine protease ATG4C isoform X1 K08342 ATG4; cysteine protease ATG4 [EC:3.4.22.-]
D 610868 ATG5; autophagy protein 5 isoform X1 K08339 ATG5; autophagy-related protein 5
D 480513 BECN1; beclin-1 isoform X1 K08334 BECN; beclin
D 490371 BECN2; beclin-2 isoform X1 K08334 BECN; beclin
D 476533 ATG7; ubiquitin-like modifier-activating enzyme ATG7 isoform X2 K08337 ATG7; ubiquitin-like modifier-activating enzyme ATG7
D 479646 GABARAPL2; gamma-aminobutyric acid receptor-associated protein-like 2 isoform X1 K08341 GABARAP; GABA(A) receptor-associated protein
D 479479 GABARAP; gamma-aminobutyric acid receptor-associated protein K08341 GABARAP; GABA(A) receptor-associated protein
D 612919 GABARAPL1; gamma-aminobutyric acid receptor-associated protein-like 1 K08341 GABARAP; GABA(A) receptor-associated protein
D 482799 ATG9B; autophagy-related protein 9B K17907 ATG9; autophagy-related protein 9
D 102154798 ATG9A; autophagy-related protein 9A isoform X1 K17907 ATG9; autophagy-related protein 9
D 610103 ATG10; ubiquitin-like-conjugating enzyme ATG10 isoform X1 K17888 ATG10L; ubiquitin-like-conjugating enzyme ATG10
D 474636 ATG12; ubiquitin-like protein ATG12 isoform X2 K08336 ATG12; ubiquitin-like protein ATG12
D 490704 ATG14; beclin 1-associated autophagy-related key regulator K17889 ATG14L; beclin 1-associated autophagy-related key regulator
D 477412 ATG16L1; autophagy-related protein 16-1 isoform X1 K17890 ATG16L1; autophagy-related protein 16-1
D 485205 ATG16L2; protein Atg16l2 isoform X1 K20868 ATG16L2; autophagy-related protein 16-2
D 490899 WIPI1; WD repeat domain phosphoinositide-interacting protein 1 isoform X2 K17908 WIPI1_2; autophagy-related protein 18
D 608721 WIPI2; WD repeat domain phosphoinositide-interacting protein 2 isoform X8 K17908 WIPI1_2; autophagy-related protein 18
C Parkin-dependent mechanism factors
D 612316 PRKN; E3 ubiquitin-protein ligase parkin isoform X1 K04556 PARK2; parkin [EC:2.3.2.31]
D 607044 RNF41; E3 ubiquitin-protein ligase NRDP1 K11981 RNF41; E3 ubiquitin-protein ligase NRDP1 [EC:2.3.2.27]
D 100685190 CLEC16A; protein CLEC16A isoform X1 K19513 CLEC16A; protein CLEC16A
D 100688445 PINK1; serine/threonine-protein kinase PINK1, mitochondrial K05688 PINK1; PTEN induced putative kinase 1 [EC:2.7.11.1]
D 488100 PARL; presenilins-associated rhomboid-like protein, mitochondrial isoform X2 K09650 PARL; rhomboid-like protein [EC:3.4.21.105]
D 481459 SQSTM1; sequestosome-1 isoform X4 K14381 SQSTM1; sequestosome 1
D 475264 TAX1BP1; tax1-binding protein 1 isoform X1 K21347 TAX1BP1; Tax1-binding protein 1
D 480545 CALCOCO2; calcium-binding and coiled-coil domain-containing protein 2 isoform X6 K21348 CALCOCO2; calcium-binding and coiled-coil domain-containing protein 2
D 483634 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 483632 ATG13; autophagy-related protein 13 isoform X1 K08331 ATG13; autophagy-related protein 13
D 477437 ULK1; serine/threonine-protein kinase ULK1 isoform X3 K21357 ULK1; serine/threonine-protein kinase ULK1 [EC:2.7.11.1]
D 489525 ULK2; serine/threonine-protein kinase ULK2 isoform X8 K08269 ULK2; serine/threonine-protein kinase ULK2 [EC:2.7.11.1]
D 477876 RB1CC1; RB1-inducible coiled-coil protein 1 isoform X1 K17589 RB1CC1; RB1-inducible coiled-coil protein 1
D 480438 HSP90AA1; heat shock protein HSP 90-alpha K04079 HSP90A; molecular chaperone HtpG
D 474919 HSP90AB1; heat shock protein HSP 90-beta K04079 HSP90A; molecular chaperone HtpG
D 100687242 LOW QUALITY PROTEIN: heat shock protein HSP 90-beta-like K04079 HSP90A; molecular chaperone HtpG
D 484955 CDC37; hsp90 co-chaperone Cdc37 K09554 CDC37; cell division cycle protein 37
D 608821 SMURF1; E3 ubiquitin-protein ligase SMURF1 isoform X1 K04678 SMURF; E3 ubiquitin ligase SMURF1/2 [EC:2.3.2.26]
D 480470 SMURF2; E3 ubiquitin-protein ligase SMURF2 K04678 SMURF; E3 ubiquitin ligase SMURF1/2 [EC:2.3.2.26]
D 608552 BNIP3L; BCL2/adenovirus E1B 19 kDa protein-interacting protein 3-like isoform X2 K15465 BNIP3L; BCL2/adenovirus E1B 19 kDa protein-interacting protein 3-like
D 491842 FUNDC1; FUN14 domain-containing protein 1 K17986 FUNDC1; FUN14 domain-containing protein 1
C Other mitophagy factors
D 479351 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 489571 PRKAA2; 5'-AMP-activated protein kinase catalytic subunit alpha-2 isoform X2 K07198 PRKAA; 5'-AMP-activated protein kinase, catalytic alpha subunit [EC:2.7.11.31]
D 477201 MAP1LC3A; microtubule-associated proteins 1A/1B light chain 3A K10435 MAP1LC; microtubule-associated protein 1 light chain
D 479619 MAP1LC3B; microtubule-associated proteins 1A/1B light chain 3B isoform X1 K10435 MAP1LC; microtubule-associated protein 1 light chain
D 611659 MAP1LC3C; microtubule-associated proteins 1A/1B light chain 3C K10435 MAP1LC; microtubule-associated protein 1 light chain
D 480509 NBR1; next to BRCA1 gene 1 protein K17987 NBR1; next to BRCA1 gene 1 protein
D 489868 TECPR1; tectonin beta-propeller repeat-containing protein 1 K17988 TECPR1; tectonin beta-propeller repeat-containing protein 1
D 490768 ZFYVE1; zinc finger FYVE domain-containing protein 1 isoform X3 K17603 ZFYVE1; zinc finger FYVE domain-containing protein 1
D 480517 COASY; bifunctional coenzyme A synthase isoform X1 K02318 COASY; phosphopantetheine adenylyltransferase / dephospho-CoA kinase [EC:2.7.7.3 2.7.1.24]
D 481256 PLA2G6; 85/88 kDa calcium-independent phospholipase A2 isoform X2 K16343 PLA2G6; calcium-independent phospholipase A2 [EC:3.1.1.4]
D 111090009 protein C19orf12-like isoform X1 K23168 MPAN; protein MPAN
D 613014 FUNDC2; FUN14 domain-containing protein 2 K24308 FUNDC2; FUN14 domain-containing protein 2
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#[ BRITE | KEGG2 | KEGG ]
#Last updated: March 14, 2024