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Mitochondrial Biogenesis - Sarcophilus harrisii (Tasmanian devil)
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AMitochondrial DNA transcription, translation, and replication factors
B Mitochondrial DNA-encoded proteins
C Mitochondrial respiratory chain complex I
D 13826446 ND1; NADH dehydrogenase subunit 1 K03878 ND1; NADH-ubiquinone oxidoreductase chain 1 [EC:7.1.1.2]
D 13826439 ND2; NADH dehydrogenase subunit 2 K03879 ND2; NADH-ubiquinone oxidoreductase chain 2 [EC:7.1.1.2]
D 13826438 ND3; NADH dehydrogenase subunit 3 K03880 ND3; NADH-ubiquinone oxidoreductase chain 3 [EC:7.1.1.2]
D 13826443 ND4; NADH dehydrogenase subunit 4 K03881 ND4; NADH-ubiquinone oxidoreductase chain 4 [EC:7.1.1.2]
D 13826441 ND4L; NADH dehydrogenase subunit 4L K03882 ND4L; NADH-ubiquinone oxidoreductase chain 4L [EC:7.1.1.2]
D 13826442 ND5; NADH dehydrogenase subunit 5 K03883 ND5; NADH-ubiquinone oxidoreductase chain 5 [EC:7.1.1.2]
D 13826444 ND6; NADH dehydrogenase subunit 6 K03884 ND6; NADH-ubiquinone oxidoreductase chain 6 [EC:7.1.1.2]
C Mitochondrial respiratory chain complex III
D 13826445 CYTB; cytochrome b K00412 CYTB; ubiquinol-cytochrome c reductase cytochrome b subunit
C Cytochrome c oxidase
D 13826440 COX1; cytochrome c oxidase subunit I K02256 COX1; cytochrome c oxidase subunit 1 [EC:7.1.1.9]
D 13826435 COX2; cytochrome c oxidase subunit II K02261 COX2; cytochrome c oxidase subunit 2
D 13826436 COX3; cytochrome c oxidase subunit III K02262 COX3; cytochrome c oxidase subunit 3
C F-type ATPase
D 13826437 ATP8; ATP synthase F0 subunit 8 K02125 ATPeF08; F-type H+-transporting ATPase subunit 8
D 13826434 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 100915916 POLRMT; DNA-directed RNA polymerase, mitochondrial K10908 POLRMT; DNA-directed RNA polymerase, mitochondrial [EC:2.7.7.6]
C Mitochondrial transcription factors
D 100932137 TFAM; transcription factor A, mitochondrial K11830 TFAM; transcription factor A, mitochondrial
D 100919867 TFB1M; dimethyladenosine transferase 1, mitochondrial K15266 TFB1M; dimethyladenosine transferase 1, mitochondrial [EC:2.1.1.-]
D 100932622 TFB2M; dimethyladenosine transferase 2, mitochondrial K17653 TFB2M; dimethyladenosine transferase 2, mitochondrial [EC:2.1.1.-]
C Mitochondrial translation factors
D 100934927 MTIF2; translation initiation factor IF-2, mitochondrial isoform X1 K02519 infB; translation initiation factor IF-2
D 100928881 MTIF3; translation initiation factor IF-3, mitochondrial K02520 infC; translation initiation factor IF-3
D 100933164 TSFM; elongation factor Ts, mitochondrial K02357 tsf; elongation factor Ts
D 100918707 TUFM; elongation factor Tu, mitochondrial K02358 tuf; elongation factor Tu
D 100915680 GFM1; elongation factor G, mitochondrial K02355 fusA; elongation factor G
D 100920093 GFM2; ribosome-releasing factor 2, mitochondrial K02355 fusA; elongation factor G
D 100923794 MTERF2; transcription termination factor 2, mitochondrial K15031 MTERF; mitochondrial transcription termination factor family protein
D 100926042 MTERF4; transcription termination factor 4, mitochondrial K15031 MTERF; mitochondrial transcription termination factor family protein
D 100929036 MTERF3; transcription termination factor 3, mitochondrial K15031 MTERF; mitochondrial transcription termination factor family protein
D 100913446 GUF1; translation factor GUF1, mitochondrial K21594 GUF1; translation factor GUF1, mitochondrial [EC:3.6.5.-]
C RNase MRP
D 100932424 POP1; ribonucleases P/MRP protein subunit POP1 K01164 POP1; ribonuclease P/MRP protein subunit POP1 [EC:3.1.26.5]
D 100921211 POP4; ribonuclease P protein subunit p29 K03538 POP4; ribonuclease P protein subunit POP4 [EC:3.1.26.5]
D 111719271 POP5; ribonuclease P/MRP protein subunit POP5 K03537 POP5; ribonuclease P/MRP protein subunit POP5 [EC:3.1.26.5]
D 100923024 RPP30; ribonuclease P protein subunit p30 isoform X1 K03539 RPP1; ribonuclease P/MRP protein subunit RPP1 [EC:3.1.26.5]
D 100913960 POP7; ribonuclease P protein subunit p20 K14527 RPP20; ribonuclease P/MRP protein subunit RPP20 [EC:3.1.26.5]
D 100914271 RPP25L; ribonuclease P protein subunit p25-like protein K14525 RPP25; ribonucleases P/MRP protein subunit RPP25 [EC:3.1.26.5]
D 116421954 RPP25; ribonuclease P protein subunit p25 K14525 RPP25; ribonucleases P/MRP protein subunit RPP25 [EC:3.1.26.5]
D 100933940 RPP38; ribonuclease P protein subunit p38 K14523 RPP38; ribonucleases P/MRP protein subunit RPP38 [EC:3.1.26.5]
D 100915491 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 100917929 TRMT10C; tRNA methyltransferase 10 homolog C K17654 TRMT10C; mitochondrial ribonuclease P protein 1 [EC:2.1.1.-]
D 100918332 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 100916946 PRORP; mitochondrial ribonuclease P catalytic subunit isoform X1 K17655 MRPP3; mitochondrial ribonuclease P protein 3
C Other mitochondrial DNA transcription and translation factors
D 100914408 putative protein MSS51 homolog, mitochondrial isoform X3 K17656 MSS51; mitochondrial splicing suppressor protein 51
D 100922061 TEFM; transcription elongation factor, mitochondrial K17658 TEFM; transcription elongation factor, mitochondrial
D 100925326 PTCD1; pentatricopeptide repeat-containing protein 1, mitochondrial K17710 PTCD1; pentatricopeptide repeat domain-containing protein 1
D 100933294 PTCD2; pentatricopeptide repeat-containing protein 2, mitochondrial isoform X1 K17711 PTCD2; pentatricopeptide repeat domain-containing protein 2
D 100918514 PTCD3; pentatricopeptide repeat domain-containing protein 3, mitochondrial isoform X1 K17659 PTCD3; pentatricopeptide repeat domain-containing protein 3
D 100928007 DHX30; ATP-dependent RNA helicase DHX30 isoform X1 K13185 DHX30; ATP-dependent RNA helicase DHX30 [EC:5.6.2.6]
D 100930308 ubiquinol-cytochrome-c reductase complex assembly factor 1 K17662 CBP3; cytochrome b pre-mRNA-processing protein 3
D 100916820 AFG1L; AFG1-like ATPase isoform X1 K18798 AFG1; peroxisome-assembly ATPase [EC:3.6.4.7]
D 100915012 ATP23; mitochondrial inner membrane protease ATP23 homolog K18156 ATP23; mitochondrial inner membrane protease ATP23 [EC:3.4.24.-]
D 100917714 SUPV3L1; ATP-dependent RNA helicase SUPV3L1, mitochondrial isoform X1 K17675 SUPV3L1; ATP-dependent RNA helicase SUPV3L1/SUV3 [EC:5.6.2.6]
D 100931808 LACTB2; endoribonuclease LACTB2 K16639 LACTB2; endoribonuclease LACTB2 [EC:3.1.27.-]
D 100920060 SLC25A4; ADP/ATP translocase 1 K05863 SLC25A4S; solute carrier family 25 (mitochondrial adenine nucleotide translocator), member 4/5/6/31
D 100929945 SLC25A31; ADP/ATP translocase 4 K05863 SLC25A4S; solute carrier family 25 (mitochondrial adenine nucleotide translocator), member 4/5/6/31
D 116422319 SLC25A6; ADP/ATP translocase 3 K05863 SLC25A4S; solute carrier family 25 (mitochondrial adenine nucleotide translocator), member 4/5/6/31
D 100914002 SLC25A37; mitoferrin-1 isoform X2 K15113 SLC25A28_37; solute carrier family 25 (mitochondrial iron transporter), member 28/37
D 100926078 SLC25A28; mitoferrin-2 isoform X1 K15113 SLC25A28_37; solute carrier family 25 (mitochondrial iron transporter), member 28/37
D 100929166 MALSU1; mitochondrial assembly of ribosomal large subunit protein 1 K25803 MALSU1; mitochondrial assembly of ribosomal large subunit protein 1
D 100932968 ERAL1; GTPase Era, mitochondrial K03595 era; GTPase
D 100924215 YARS1; tyrosine--tRNA ligase, cytoplasmic K01866 YARS; tyrosyl-tRNA synthetase [EC:6.1.1.1]
D 100928476 YARS2; tyrosine--tRNA ligase, mitochondrial K01866 YARS; tyrosyl-tRNA synthetase [EC:6.1.1.1]
D 100914088 LARS1; leucine--tRNA ligase, cytoplasmic K01869 LARS; leucyl-tRNA synthetase [EC:6.1.1.4]
D 100928555 DARS2; aspartate--tRNA ligase, mitochondrial K01876 DARS2; aspartyl-tRNA synthetase [EC:6.1.1.12]
D 100932085 GARS1; glycine--tRNA ligase K01880 GARS; glycyl-tRNA synthetase [EC:6.1.1.14]
D 100916563 RARS2; probable arginine--tRNA ligase, mitochondrial isoform X2 K01887 RARS; arginyl-tRNA synthetase [EC:6.1.1.19]
D 100918593 RARS1; arginine--tRNA ligase, cytoplasmic isoform X1 K01887 RARS; arginyl-tRNA synthetase [EC:6.1.1.19]
D 100935237 QRSL1; glutamyl-tRNA(Gln) amidotransferase subunit A, mitochondrial isoform X1 K02433 gatA; aspartyl-tRNA(Asn)/glutamyl-tRNA(Gln) amidotransferase subunit A [EC:6.3.5.6 6.3.5.7]
D 100914495 GATB; glutamyl-tRNA(Gln) amidotransferase subunit B, mitochondrial isoform X1 K02434 gatB; aspartyl-tRNA(Asn)/glutamyl-tRNA(Gln) amidotransferase subunit B [EC:6.3.5.6 6.3.5.7]
D 100920268 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 100921011 MTPAP; poly(A) RNA polymerase, mitochondrial isoform X1 K18060 MTPAP; poly(A) RNA polymerase, mitochondrial [EC:2.7.7.19]
D 111719426 PDE12; 2',5'-phosphodiesterase 12 K19612 PDE12; 2',5'-phosphodiesterase [EC:3.1.13.4 3.1.4.-]
D 100935332 MTG1; mitochondrial ribosome-associated GTPase 1 isoform X2 K19828 MTG1; mitochondrial GTPase 1
D 100921546 NOA1; nitric oxide-associated protein 1 K19832 NOA1; nitric oxide-associated protein 1
D 100914202 NSUN3; tRNA (cytosine(34)-C(5))-methyltransferase, mitochondrial isoform X1 K21969 NSUN3; tRNA (cytosine34-C5)-methyltransferase [EC:2.1.1.-]
D 100934109 NSUN4; 5-methylcytosine rRNA methyltransferase NSUN4 isoform X1 K21970 NSUN4; 5-methylcytosine rRNA methyltransferase NSUN4 [EC:2.1.1.-]
D 100929986 RPUSD3; mitochondrial mRNA pseudouridine synthase RPUSD3 K22537 RPUSD3; mitochondrial RNA pseudouridine synthase RPUSD3 [EC:5.4.99.-]
D 100924995 RPUSD4; mitochondrial RNA pseudouridine synthase RPUSD4 K22538 RPUSD4; mitochondrial RNA pseudouridine synthase RPUSD4 [EC:5.4.99.-]
D 100920132 RCC1L; RCC1-like G exchanging factor-like protein K23495 RCC1L; RCC1-like G exchanging factor-like protein
D 116421865 NGRN; neugrin K23496 NGRN; neugrin
D 105749234 TRUB2; mitochondrial mRNA pseudouridine synthase TRUB2 isoform X1 K23497 TRUB2; mitochondrial mRNA pseudouridine synthase TRUB2 [EC:5.4.99.-]
D 100926473 FASTKD1; FAST kinase domain-containing protein 1, mitochondrial K27754 FASTKD1; FAST kinase domain-containing protein 1
D 100917680 FASTKD2; FAST kinase domain-containing protein 2, mitochondrial isoform X1 K18190 FASTKD2; FAST kinase domain-containing protein 2
D 100922959 FASTKD3; FAST kinase domain-containing protein 3, mitochondrial K27755 FASTKD3; FAST kinase domain-containing protein 3
D 100923757 TBRG4; FAST kinase domain-containing protein 4 K27756 TBRG4; FAST kinase domain-containing protein 4
D 105750932 FASTKD5; FAST kinase domain-containing protein 5, mitochondrial K27757 FASTKD5; FAST kinase domain-containing protein 5
D 100932764 C1H12orf65; probable peptide chain release factor C12orf65 homolog, mitochondrial K23498 COXPD7; peptide chain release factor
D 116419136 RMND1; required for meiotic nuclear division protein 1 homolog isoform X1 K23499 RMND1; required for meiotic nuclear division protein 1
D 100931254 GRSF1; G-rich sequence factor 1 isoform X2 K24984 GRSF1; G-rich sequence factor 1
D 100932985 METTL15; probable methyltransferase-like protein 15 K25876 METTL15; 12S rRNA N4-methylcytidine (m4C) methyltransferase [EC:2.1.1.-]
D 100921542 C5H12orf10; UPF0160 protein MYG1, mitochondrial K25877 MYG1; MYG1 exonuclease [EC:3.1.-.-]
D 100913267 MTRES1; mitochondrial transcription rescue factor 1 K26186 MTRES1; mitochondrial transcription rescue factor 1
B Mitochondrial DNA replication factors
C Mitochondrial DNA polymerase
D 100932218 POLG; DNA polymerase subunit gamma-1 K02332 POLG; DNA polymerase gamma 1 [EC:2.7.7.7]
D 100922241 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 111718546 ENDOG; endonuclease G, mitochondrial K01173 ENDOG; endonuclease G, mitochondrial
D 100920086 MGME1; mitochondrial genome maintenance exonuclease 1 K19465 MGME1; mitochondrial genome maintenance exonuclease 1 [EC:3.1.-.-]
D 111719580 EXOG; nuclease EXOG, mitochondrial K15050 EXOG; nuclease EXOG, mitochondrial
D 100931263 TWNK; twinkle protein, mitochondrial K17680 PEO1; twinkle protein [EC:5.6.2.3]
D 100915973 PIF1; ATP-dependent DNA helicase PIF1 isoform X2 K15255 PIF1; ATP-dependent DNA helicase PIF1 [EC:5.6.2.3]
C Other Mitochondrial DNA replication factors
D 100931778 SSBP1; single-stranded DNA-binding protein, mitochondrial K03111 ssb; single-strand DNA-binding protein
D 100934163 ATAD3A; ATPase family AAA domain-containing protein 3A K17681 ATAD3A_B; ATPase family AAA domain-containing protein 3A/B
D 100917257 ubiquinol-cytochrome-c reductase complex assembly factor 2 K17682 MNF1; mitochondrial nucleoid factor 1
D 100928461 C4H17orf80; uncharacterized protein C17orf80 homolog isoform X1 K27759 MTNAP1; mitochondrial nucleoid-associated protein 1
D 100915014 GTPase IMAP family member 5 K27758 GIMAP1_3_5; GTPase IMAP family member 1/3/5
D 100934456 GTPase IMAP family member 1 isoform X2 K27758 GIMAP1_3_5; GTPase IMAP family member 1/3/5
D 100934723 GTPase IMAP family member 1 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 100925918 DNAJA2; dnaJ homolog subfamily A member 2 isoform X1 K09503 DNAJA2; DnaJ homolog subfamily A member 2
D 100915553 TOMM34; mitochondrial import receptor subunit TOM34 K17766 TOM34; mitochondrial import receptor subunit TOM34
D 100921534 RHOT1; mitochondrial Rho GTPase 1 isoform X1 K07870 RHOT1; mitochondrial Rho GTPase 1 [EC:3.6.5.-]
D 100923174 heat shock 70 kDa protein 1-like K03283 HSPA1_6_8; heat shock 70kDa protein 1/6/8
D 100932179 HSPA8; heat shock cognate 71 kDa protein K03283 HSPA1_6_8; heat shock 70kDa protein 1/6/8
D 116419051 heat shock 70 kDa protein 6 K03283 HSPA1_6_8; heat shock 70kDa protein 1/6/8
D 116423214 heat shock 70 kDa protein 1 K03283 HSPA1_6_8; heat shock 70kDa protein 1/6/8
D 100919416 AIPL1; aryl-hydrocarbon-interacting protein-like 1 K17767 AIP; AH receptor-interacting protein
D 100928585 AIP; AH receptor-interacting protein isoform X1 K17767 AIP; AH receptor-interacting protein
D 116420361 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 116419076 TOMM5; mitochondrial import receptor subunit TOM5 homolog K17773 TOM5; mitochondrial import receptor subunit TOM5
D 100917775 TOMM6; mitochondrial import receptor subunit TOM6 homolog K17772 TOM6; mitochondrial import receptor subunit TOM6
D 116419613 TOMM7; mitochondrial import receptor subunit TOM7 homolog K17771 TOM7; mitochondrial import receptor subunit TOM7
D 100918893 TOMM20; mitochondrial import receptor subunit TOM20 homolog K17770 TOM20; mitochondrial import receptor subunit TOM20
D 100926087 TOMM20L; TOMM20-like protein 1 isoform X1 K17770 TOM20; mitochondrial import receptor subunit TOM20
D 100919178 TOMM22; mitochondrial import receptor subunit TOM22 homolog K17769 TOM22; mitochondrial import receptor subunit TOM22
D 100917600 TOMM40L; mitochondrial import receptor subunit TOM40B isoform X1 K11518 TOM40; mitochondrial import receptor subunit TOM40
D 100920379 TOMM40; LOW QUALITY PROTEIN: mitochondrial import receptor subunit TOM40 homolog K11518 TOM40; mitochondrial import receptor subunit TOM40
D 100921680 TOMM70; mitochondrial import receptor subunit TOM70 K17768 TOM70; mitochondrial import receptor subunit TOM70
C TOB/SAM complex
D 100934014 SAMM50; sorting and assembly machinery component 50 homolog isoform X2 K07277 SAM50; outer membrane protein insertion porin family
C Porin
D 100927813 VDAC1; voltage-dependent anion-selective channel protein 1 K05862 VDAC1; voltage-dependent anion channel protein 1
D 100920408 VDAC2; voltage-dependent anion-selective channel protein 2 K15040 VDAC2; voltage-dependent anion channel protein 2
D 100923123 VDAC3; voltage-dependent anion-selective channel protein 3 isoform X2 K15041 VDAC3; voltage-dependent anion channel protein 3
C ERMES complex
C Other outer membrane factor
D 100918622 MTX2; metaxin-2 isoform X2 K17776 MTX; metaxin
D 100919421 MTX1; metaxin-1 K17776 MTX; metaxin
D 100927134 MTX3; metaxin-3 isoform X1 K17776 MTX; metaxin
D 100932797 MYO19; unconventional myosin-XIX isoform X1 K25838 MYO19; myosin XIX
D 100917770 MTARC2; mitochondrial amidoxime reducing component 2 K27318 MTARC; mitochondrial amidoxime-reducing component [EC:1.7.-.-]
D 116423382 mitochondrial amidoxime-reducing component 1-like isoform X1 K27318 MTARC; mitochondrial amidoxime-reducing component [EC:1.7.-.-]
B Inter membrane space
C Transporter inner membrane (TIM) 9/10 complex
D 100926342 TIMM9; mitochondrial import inner membrane translocase subunit Tim9 isoform X3 K17777 TIM9; mitochondrial import inner membrane translocase subunit TIM9
D 100930730 TIMM10; mitochondrial import inner membrane translocase subunit Tim10 K17778 TIM10; mitochondrial import inner membrane translocase subunit TIM10
D 100917418 TIMM10B; mitochondrial import inner membrane translocase subunit Tim10 B K17779 TIM10B; mitochondrial import inner membrane translocase subunit TIM10B
C TIM8/13 complex
D 100916891 TIMM8B; mitochondrial import inner membrane translocase subunit Tim8 B K17780 TIM8; mitochondrial import inner membrane translocase subunit TIM8
D 100929441 TIMM8A; mitochondrial import inner membrane translocase subunit Tim8 A K17780 TIM8; mitochondrial import inner membrane translocase subunit TIM8
D 100923837 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 100920275 CHCHD4; LOW QUALITY PROTEIN: mitochondrial intermembrane space import and assembly protein 40 K17782 MIA40; mitochondrial intermembrane space import and assembly protein 40
D 116420494 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 100931479 MICOS10; MICOS complex subunit MIC10 K17784 MICOS10; MICOS complex subunit MIC10
D 100918254 IMMT; MICOS complex subunit MIC60 isoform X1 K17785 IMMT; MICOS complex subunit MIC60
D 100922702 MICOS13; MICOS complex subunit MIC13 K24624 MICOS13; MICOS complex subunit MIC13
D 100915527 CHCHD3; MICOS complex subunit MIC19 isoform X2 K17563 CHCHD3; MICOS complex subunit MIC19
D 100919228 CHCHD6; MICOS complex subunit MIC25 isoform X1 K17564 CHCHD6; MICOS complex subunit MIC25
D 100927330 APOO; MICOS complex subunit MIC26 isoform X1 K24625 APOO; MICOS complex subunit MIC26
D 100934119 LOW QUALITY PROTEIN: MICOS complex subunit Mic26-like K24625 APOO; MICOS complex subunit MIC26
C MICOS complex (yeast)
D 100931479 MICOS10; MICOS complex subunit MIC10 K17784 MICOS10; MICOS complex subunit MIC10
D 100918254 IMMT; MICOS complex subunit MIC60 isoform X1 K17785 IMMT; MICOS complex subunit MIC60
C TIM22 complex
D 100925275 TIMM22; mitochondrial import inner membrane translocase subunit Tim22 K17790 TIM22; mitochondrial import inner membrane translocase subunit TIM22
C TIM23 complex
D 100918060 TIMM23B; putative mitochondrial import inner membrane translocase subunit Tim23B K17794 TIM23; mitochondrial import inner membrane translocase subunit TIM23
D 100917372 TIMM17B; mitochondrial import inner membrane translocase subunit Tim17-B K17795 TIM17; mitochondrial import inner membrane translocase subunit TIM17
D 100919778 TIMM17A; mitochondrial import inner membrane translocase subunit Tim17-A K17795 TIM17; mitochondrial import inner membrane translocase subunit TIM17
D 100920359 TIMM21; mitochondrial import inner membrane translocase subunit Tim21 K17796 TIM21; mitochondrial import inner membrane translocase subunit TIM21
D 100921420 TIMM50; mitochondrial import inner membrane translocase subunit TIM50 K17496 TIM50; mitochondrial import inner membrane translocase subunit TIM50
C Mitochondrial calcium uniporter complex
D 100913360 MCU; calcium uniporter protein, mitochondrial K20858 MCU; calcium uniporter protein, mitochondrial
D 100934813 MICU1; calcium uptake protein 1, mitochondrial isoform X1 K22827 MICU1; calcium uptake protein 1, mitochondrial
D 100917168 MICU2; calcium uptake protein 2, mitochondrial K22828 MICU2; calcium uptake protein 2, mitochondrial
D 100930810 MCUB; calcium uniporter regulatory subunit MCUb, mitochondrial K22829 MCUB; calcium uniporter regulatory subunit MCUb, mitochondrial
D 100927292 essential MCU regulator, mitochondrial K22830 SMDT1; essential MCU regulator, mitochondrial
D 100918179 MCUR1; mitochondrial calcium uniporter regulator 1 K22137 MCUR1; mitochondrial calcium uniporter regulator 1
C Other inner membrane factors
D 100913712 LETM1; mitochondrial proton/calcium exchanger protein isoform X2 K17800 LETM1; LETM1 and EF-hand domain-containing protein 1, mitochondrial
D 100914329 IMMP1L; mitochondrial inner membrane protease subunit 1 isoform X1 K09647 IMP1; mitochondrial inner membrane protease subunit 1 [EC:3.4.21.-]
D 100913481 mitochondrial inner membrane protease subunit 2 K09648 IMP2; mitochondrial inner membrane protease subunit 2 [EC:3.4.21.-]
D 111721112 IMMP2L; mitochondrial inner membrane protease subunit 2 K09648 IMP2; mitochondrial inner membrane protease subunit 2 [EC:3.4.21.-]
D 100921592 TMEM126A; transmembrane protein 126A K18157 TMEM126A; transmembrane protein 126A
D 100913850 MPC1L; mitochondrial pyruvate carrier 1-like protein K22138 MPC1; mitochondrial pyruvate carrier 1
D 100931590 MPC1; mitochondrial pyruvate carrier 1 isoform X1 K22138 MPC1; mitochondrial pyruvate carrier 1
D 100913681 MPC2; mitochondrial pyruvate carrier 2 K22139 MPC2; mitochondrial pyruvate carrier 2
D 111719535 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 100927196 OCIAD1; OCIA domain-containing protein 1 K28215 OCIAD1; OCIA domain-containing protein 1
B Matrix
C MMC/PAM proteins
D 100922704 TIMM44; mitochondrial import inner membrane translocase subunit TIM44 isoform X3 K17804 TIM44; mitochondrial import inner membrane translocase subunit TIM44
D 100922082 HSPA9; stress-70 protein, mitochondrial K04043 dnaK; molecular chaperone DnaK
D 100924509 GRPEL1; grpE protein homolog 1, mitochondrial K03687 GRPE; molecular chaperone GrpE
D 100930144 GRPEL2; grpE protein homolog 2, mitochondrial K03687 GRPE; molecular chaperone GrpE
D 100913859 DNAJC19; mitochondrial import inner membrane translocase subunit TIM14 K09539 DNAJC19; DnaJ homolog subfamily C member 19
D 116420475 PAM16; mitochondrial import inner membrane translocase subunit TIM16 K17805 PAM16; mitochondrial import inner membrane translocase subunit TIM16
C Other matrix factors
D 100928506 PMPCA; mitochondrial-processing peptidase subunit alpha isoform X1 K01412 PMPCA; mitochondrial-processing peptidase subunit alpha [EC:3.4.24.64]
D 100920317 PMPCB; mitochondrial-processing peptidase subunit beta K17732 PMPCB; mitochondrial-processing peptidase subunit beta [EC:3.4.24.64]
D 100923419 MIPEP; mitochondrial intermediate peptidase K01410 MIPEP; mitochondrial intermediate peptidase [EC:3.4.24.59]
D 100917239 HSPD1; 60 kDa heat shock protein, mitochondrial isoform X2 K04077 groEL; chaperonin GroEL [EC:5.6.1.7]
D 100920453 10 kDa heat shock protein, mitochondrial isoform X2 K04078 groES; chaperonin GroES
D 100930587 DNAJA3; dnaJ homolog subfamily A member 3, mitochondrial isoform X1 K09504 DNAJA3; DnaJ homolog subfamily A member 3
D 100933634 TAMM41; phosphatidate cytidylyltransferase, mitochondrial isoform X1 K17807 TAM41; mitochondrial translocator assembly and maintenance protein 41
D 100931271 DNLZ; DNL-type zinc finger protein K17808 ZIM17; mitochondrial protein import protein ZIM17
D 100919768 ETHE1; persulfide dioxygenase ETHE1, mitochondrial K17725 ETHE1; sulfur dioxygenase [EC:1.13.11.18]
D 100928825 POLDIP2; LOW QUALITY PROTEIN: 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 100913327 CREB1; cyclic AMP-responsive element-binding protein 1 isoform X1 K05870 CREB1; cyclic AMP-responsive element-binding protein 1
D 100915680 GFM1; elongation factor G, mitochondrial K02355 fusA; elongation factor G
D 100920093 GFM2; ribosome-releasing factor 2, mitochondrial K02355 fusA; elongation factor G
D 100930024 SP1; transcription factor Sp1 isoform X1 K04684 SP1; transcription factor Sp1
D 100930391 LOW QUALITY PROTEIN: transcription factor YY2-like K09201 YY; transcription factor YY
D 100934651 YY1; transcriptional repressor protein YY1 isoform X1 K09201 YY; transcription factor YY
D 100915981 GABPB1; GA-binding protein subunit beta-1 isoform X1 K09454 GABPB; GA-binding protein transcription factor, beta
D 100919525 GABPB2; GA-binding protein subunit beta-2 isoform X1 K09454 GABPB; GA-binding protein transcription factor, beta
D 100932137 TFAM; transcription factor A, mitochondrial K11830 TFAM; transcription factor A, mitochondrial
C Nuclear respiratory factors
D 100933595 NRF1; nuclear respiratory factor 1 K11831 NRF1; nuclear respiratory factor 1
D 100915496 NFE2L2; nuclear factor erythroid 2-related factor 2 isoform X2 K05638 NFE2L2; nuclear factor erythroid 2-related factor 2
D 100920166 metallothionein-2B K14739 MT1_2; metallothionein 1/2
D 105751160 metallothionein-1D K14739 MT1_2; metallothionein 1/2
D 105751161 metallothionein-1E K14739 MT1_2; metallothionein 1/2
D 100916162 metallothionein-4 K14741 MT4; metallothionein 4
C Coactivators
D 100931691 PPARGC1A; peroxisome proliferator-activated receptor gamma coactivator 1-alpha isoform X5 K07202 PPARGC1A; alpha peroxisome proliferator-activated receptor gamma coactivator 1-alpha
D 100915901 PPARGC1B; peroxisome proliferator-activated receptor gamma coactivator 1-beta K17962 PPARGC1B; peroxisome proliferator-activated receptor gamma coactivator 1-beta
D 105749347 PPRC1; peroxisome proliferator-activated receptor gamma coactivator-related protein 1 isoform X1 K17963 PPRC1; peroxisome proliferator-activated receptor gamma coactivator-related protein 1
D 100934324 CRTC1; CREB-regulated transcription coactivator 1 isoform X1 K15309 CRTC1; CREB-regulated transcription coactivator 1
D 100921187 EP300; histone acetyltransferase p300 isoform X1 K04498 EP300; E1A/CREB-binding protein [EC:2.3.1.48]
D 100926283 CREBBP; CREB-binding protein isoform X2 K04498 EP300; E1A/CREB-binding protein [EC:2.3.1.48]
C Proteins involved in Fe-S cluster biogenesis
D 100931095 NFS1; cysteine desulfurase, mitochondrial K04487 iscS; cysteine desulfurase [EC:2.8.1.7]
D 100915139 ISCU; iron-sulfur cluster assembly enzyme ISCU, mitochondrial isoform X1 K22068 ISCU; iron-sulfur cluster assembly enzyme ISCU, mitochondrial
D 100932069 LYRM4; LYR motif-containing protein 4 isoform X2 K22069 LYRM4; LYR motif-containing protein 4
D 100925413 FXN; frataxin, mitochondrial K19054 FXN; frataxin [EC:1.16.3.1]
D 100913253 FDX1; adrenodoxin, mitochondrial K22070 FDX1; ferredoxin-1, mitochondrial
D 100933020 FDX2; ferredoxin-2, mitochondrial K22071 FDX2; ferredoxin-2, mitochondrial
D 100922569 FDXR; NADPH:adrenodoxin oxidoreductase, mitochondrial isoform X1 K18914 FDXR; adrenodoxin-NADP+ reductase [EC:1.18.1.6]
D 100922082 HSPA9; stress-70 protein, mitochondrial K04043 dnaK; molecular chaperone DnaK
D 100932586 HSCB; iron-sulfur cluster co-chaperone protein HscB K04082 hscB; molecular chaperone HscB
D 100925743 SIL1; nucleotide exchange factor SIL1 K14001 SIL1; nucleotide exchange factor SIL1
D 100918420 GLRX5; glutaredoxin-related protein 5, mitochondrial K07390 grxD; monothiol glutaredoxin
D 100931026 ISCA1; iron-sulfur cluster assembly 1 homolog, mitochondrial isoform X1 K22063 ISCA1; iron-sulfur cluster assembly 1
D 100932480 ISCA2; iron-sulfur cluster assembly 2 homolog, mitochondrial isoform X1 K22072 ISCA2; iron-sulfur cluster assembly 2
D 100915665 IBA57; putative transferase CAF17, mitochondrial K22073 IBA57; transferase CAF17, mitochondrial [EC:2.1.-.-]
D 100935097 NFU1; NFU1 iron-sulfur cluster scaffold homolog, mitochondrial isoform X1 K22074 NFU1; NFU1 iron-sulfur cluster scaffold homolog, mitochondrial
D 100925020 BOLA1; bolA-like protein 1 K22066 BOLA1; BolA-like protein 1
D 100916867 BOLA3; bolA-like protein 3 K22075 BOLA3; BolA-like protein 3
D 100928593 SFXN1; sideroflexin-1 K23500 SFXN1_3; sideroflexin-1/3
D 116421881 SFXN3; sideroflexin-3 K23500 SFXN1_3; sideroflexin-1/3
D 100916672 SFXN2; sideroflexin-2 K23501 SFXN2; sideroflexin-2
D 100915541 SFXN4; sideroflexin-4 K23502 SFXN4; sideroflexin-4
D 100929630 SFXN5; sideroflexin-5 K23503 SFXN5; sideroflexin-5
C Other regulator of mitochondrial biogenesis
D 100931262 CPT1A; carnitine O-palmitoyltransferase 1, liver isoform K08765 CPT1A; carnitine O-palmitoyltransferase 1, liver isoform [EC:2.3.1.21]
D 100930017 CPT1B; carnitine O-palmitoyltransferase 1, muscle isoform K19523 CPT1B; carnitine O-palmitoyltransferase 1, muscle isoform [EC:2.3.1.21]
D 105749969 CPT1C; carnitine O-palmitoyltransferase 1, brain isoform K19524 CPT1C; carnitine O-palmitoyltransferase 1, brain isoform [EC:2.3.1.21]
D 100931159 DHTKD1; probable 2-oxoglutarate dehydrogenase E1 component DHKTD1, mitochondrial K15791 DHKTD1; 2-oxoadipate dehydrogenase E1 component [EC:1.2.4.-]
D 100928047 FBXL4; F-box/LRR-repeat protein 4 K10270 FBXL4; F-box and leucine-rich repeat protein 4
D 100923376 HCFC1; host cell factor 1 isoform X1 K14966 HCFC1; host cell factor 1
D 116420403 host cell factor 1-like isoform X1 K14966 HCFC1; host cell factor 1
D 100920915 HCFC2; host cell factor 2 K27390 HCFC2; host cell factor 2
D 100930812 HTT; huntingtin isoform X1 K04533 HD; huntingtin
D 100923988 ESRRA; steroid hormone receptor ERR1 K08552 NR3B1; estrogen-related receptor alpha
D 100929361 ESRRB; steroid hormone receptor ERR2 K08553 NR3B2; estrogen-related receptor beta
D 100913786 ESRRG; estrogen-related receptor gamma isoform X2 K08554 NR3B3; estrogen-related receptor gamma
D 100926750 NRIP1; nuclear receptor-interacting protein 1 K17965 NRIP1; nuclear receptor-interacting protein 1
D 100926139 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 100928944 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 100926021 LONP1; lon protease homolog, mitochondrial K08675 PRSS15; ATP-dependent Lon protease [EC:3.4.21.53]
D 100920847 SIRT1; NAD-dependent protein deacetylase sirtuin-1 isoform X1 K11411 SIRT1; NAD-dependent protein deacetylase sirtuin 1 [EC:2.3.1.286]
D 100921170 SERAC1; protein SERAC1 isoform X1 K23504 SERAC1; protein SERAC1
D 100914941 UXT; protein UXT K26537 UXT; protein UXT
D 100924398 PERM1; PGC-1 and ERR-induced regulator in muscle protein 1 K26738 PERM1; PGC-1 and ERR-induced regulator in muscle protein 1
D 100929393 glutamine amidotransferase-like class 1 domain-containing protein 3B, mitochondrial K27835 GATD3; glutamine amidotransferase-like class 1 domain-containing protein 3, mitochondrial
D 100930609 glutamine amidotransferase-like class 1 domain-containing protein 3A, mitochondrial K27835 GATD3; glutamine amidotransferase-like class 1 domain-containing protein 3, mitochondrial
D 100934683 TANGO2; transport and Golgi organization protein 2 homolog K28098 TANGO2; transport and Golgi organization protein 2
D 100922296 C3H2orf69; UPF0565 protein C2orf69 homolog K28271 C2ORF69; mitochondrial protein C2orf69
D 100914415 FAM214A; protein FAM214A isoform X2 K28528 ATOS; protein Atossa
D 100934941 FAM214B; protein FAM214B isoform X1 K28528 ATOS; protein Atossa
D 100927620 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 100926437 NDUFAF1; complex I intermediate-associated protein 30, mitochondrial K18159 NDUFAF1; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 1
D 100917302 NDUFAF2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 2 K18160 NDUFAF2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 2
D 100921145 NDUFAF3; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 3 K09008 NDUFAF3; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 3
D 100926498 NDUFAF4; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 4 K18161 NDUFAF4; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 4
D 105749897 NDUFAF5; arginine-hydroxylase NDUFAF5, mitochondrial isoform X1 K18162 NDUFAF5; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 5 [EC:2.1.1.-]
D 100919927 NDUFAF6; NADH dehydrogenase (ubiquinone) complex I, assembly factor 6 isoform X1 K18163 NDUFAF6; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 6
D 100914168 NDUFAF7; protein arginine methyltransferase NDUFAF7, mitochondrial K18164 NDUFAF7; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 7
D 111720561 NDUFAF8; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 8 K24726 NDUFAF8; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 8
D 100923243 TMEM126B; complex I assembly factor TMEM126B, mitochondrial K18165 TMEM126B; complex I assembly factor TMEM126B
D 100921137 ECSIT; evolutionarily conserved signaling intermediate in Toll pathway, mitochondrial isoform X1 K04405 ECSIT; evolutionarily conserved signaling intermediate in Toll pathway
D 100930590 ACAD9; complex I assembly factor ACAD9, mitochondrial isoform X1 K15980 ACAD9; acyl-CoA dehydrogenase family member 9 [EC:1.3.99.-]
D 100916243 NUBPL; iron-sulfur protein NUBPL isoform X1 K03593 mrp; ATP-binding protein involved in chromosome partitioning
D 100924209 FOXRED1; FAD-dependent oxidoreductase domain-containing protein 1 K18166 FOXRED1; FAD-dependent oxidoreductase domain-containing protein 1
D 100934977 RTN4IP1; reticulon-4-interacting protein 1, mitochondrial isoform X1 K23164 RTN4IP1; reticulon-4-interacting protein 1, mitochondrial
D 100917577 TIMMDC1; complex I assembly factor TIMMDC1, mitochondrial K23505 TIMMDC1; complex I assembly factor TIMMDC1
C Complex-II assembly factors
D 100933684 SDHAF2; succinate dehydrogenase assembly factor 2, mitochondrial K18168 SDHAF2; succinate dehydrogenase assembly factor 2
C Complex-III assembly factors
D 100919063 mitochondrial chaperone BCS1 isoform X1 K08900 BCS1; mitochondrial chaperone BCS1
D 100914060 TTC19; tetratricopeptide repeat protein 19, mitochondrial K18169 TTC19; tetratricopeptide repeat protein 19, mitochondrial
D 100918356 LYRM7; complex III assembly factor LYRM7 K18170 LYRM7; complex III assembly factor LYRM7
D 100930308 ubiquinol-cytochrome-c reductase complex assembly factor 1 K17662 CBP3; cytochrome b pre-mRNA-processing protein 3
D 100917257 ubiquinol-cytochrome-c reductase complex assembly factor 2 K17682 MNF1; mitochondrial nucleoid factor 1
D 100916591 ubiquinol-cytochrome-c reductase complex assembly factor 3 K23165 UQCC3; ubiquinol-cytochrome-c reductase complex assembly factor 3
D 100922024 C1H16orf91; protein CCSMST1 K27339 UQCC4; ubiquinol-cytochrome c reductase complex assembly factor 4
D 100928009 SMIM4; small integral membrane protein 4 K27340 UQCC5; ubiquinol-cytochrome c reductase complex assembly factor 5
D 100931605 C5H12orf73; uncharacterized protein C12orf73 homolog K27341 UQCC6; ubiquinol-cytochrome c reductase complex assembly factor 6
D 100922233 STMP1; short transmembrane mitochondrial protein 1 K27319 STMP1; short transmembrane mitochondrial protein 1
C Complex-IV assembly factors
D 100929600 CMC1; COX assembly mitochondrial protein homolog isoform X1 K18171 CMC1; COX assembly mitochondrial protein 1
D 100917904 CMC2; COX assembly mitochondrial protein 2 homolog K18172 CMC2; COX assembly mitochondrial protein 2
D 100932276 cytochrome c oxidase assembly factor 3 homolog, mitochondrial isoform X1 K18175 CCDC56; cytochrome c oxidase assembly factor 3, animal type
D 100922553 cytochrome c oxidase assembly factor 4 homolog, mitochondrial isoform X2 K18177 COA4; cytochrome c oxidase assembly factor 4
D 100918982 cytochrome c oxidase assembly factor 5 K18178 COA5; cytochrome c oxidase assembly factor 5
D 100923779 cytochrome c oxidase assembly factor 6 homolog K18179 COA6; cytochrome c oxidase assembly factor 6
D 100915694 cytochrome c oxidase assembly factor 7 K18180 COA7; cytochrome c oxidase assembly factor 7
D 100930365 protoheme IX farnesyltransferase, mitochondrial K02257 COX10; heme o synthase [EC:2.5.1.141]
D 100924752 cytochrome c oxidase assembly protein COX11, mitochondrial K02258 COX11; cytochrome c oxidase assembly protein subunit 11
D 100929248 cytochrome c oxidase assembly protein COX14 K18181 COX14; cytochrome c oxidase assembly factor 14
D 100925550 cytochrome c oxidase assembly protein COX15 homolog K02259 COX15; heme a synthase [EC:1.17.99.9]
D 105750377 cytochrome c oxidase assembly protein COX16 homolog, mitochondrial K18182 COX16; cytochrome c oxidase assembly protein subunit 16
D 100919403 cytochrome c oxidase copper chaperone K02260 COX17; cytochrome c oxidase assembly protein subunit 17
D 100913621 cytochrome c oxidase assembly protein COX18, mitochondrial isoform X1 K17797 COX18; mitochondrial inner membrane protein COX18
D 100925762 cytochrome c oxidase assembly protein COX19 K18183 COX19; cytochrome c oxidase assembly protein subunit 19
D 100931584 cytochrome c oxidase assembly protein COX20, mitochondrial isoform X1 K18184 COX20; cytochrome c oxidase assembly protein subunit 20
D 100914408 putative protein MSS51 homolog, mitochondrial isoform X3 K17656 MSS51; mitochondrial splicing suppressor protein 51
D 100930332 protein PET100 homolog, mitochondrial K18186 PET100; protein PET100, animal type
D 100919822 protein PET117 homolog, mitochondrial K18188 PET117; protein PET117
D 100931617 OXA1L; mitochondrial inner membrane protein OXA1L K03217 yidC; YidC/Oxa1 family membrane protein insertase
D 100923826 surfeit locus protein 1 isoform X1 K14998 SURF1; surfeit locus 1 family protein
D 100918395 protein SCO1 homolog, mitochondrial K07152 SCO1; protein SCO1
D 111721020 protein SCO2 homolog, mitochondrial K23755 SCO2; protein SCO2
D 100933582 translational activator of cytochrome c oxidase 1 K18189 TACO1; translational activator of cytochrome c oxidase 1
D 100926353 LRPPRC; leucine-rich PPR motif-containing protein, mitochondrial K17964 LRPPRC; leucine-rich PPR motif-containing protein, mitochondrial
D 100924867 cytochrome c oxidase assembly factor 8 isoform X1 K23506 APOPT1; apoptogenic protein 1
D 100922233 STMP1; short transmembrane mitochondrial protein 1 K27319 STMP1; short transmembrane mitochondrial protein 1
D 100928009 SMIM4; small integral membrane protein 4 K27340 UQCC5; ubiquinol-cytochrome c reductase complex assembly factor 5
D 116419713 TMEM223; transmembrane protein 223 K27342 TMEM223; transmembrane protein 223
D 100918448 HIGD1A; HIG1 domain family member 1A, mitochondrial K28216 HIGD1A_C; HIG1 domain family member 1A/C, mitochondrial
D 100918250 HIGD2A; LOW QUALITY PROTEIN: HIG1 domain family member 2A, mitochondrial K28217 HIGD2A; HIG1 domain family member 2A, mitochondrial
C Complex-V assembly factors
D 105750280 ATPAF1; LOW QUALITY PROTEIN: ATP synthase mitochondrial F1 complex assembly factor 1 K07555 ATPeAF1; ATP synthase mitochondrial F1 complex assembly factor 1
D 100926461 ATPAF2; ATP synthase mitochondrial F1 complex assembly factor 2 K07556 ATPeAF2; ATP synthase mitochondrial F1 complex assembly factor 2
D 100928171 TMEM70; transmembrane protein 70, mitochondrial K17966 TMEM70; transmembrane protein 70, mitochondrial
D 100920341 ATP5MPL; ATP synthase subunit ATP5MPL, mitochondrial isoform X2 K18193 MP68; 6.8 kDa mitochondrial proteolipid
D 100914932 ATP5MD; ATP synthase membrane subunit DAPIT, mitochondrial K18194 USMG5; up-regulated during skeletal muscle growth protein 5
B Mitochondrial dynamics
C Fission and Fusion factors
D 100932357 MFN1; mitofusin-1 K21356 MFN1; mitofusin 1 [EC:3.6.5.-]
D 100915154 MFN2; mitofusin-2 K06030 MFN2; mitofusin 2 [EC:3.6.5.-]
D 100930263 OPA1; dynamin-like 120 kDa protein, mitochondrial K17079 OPA1; optic atrophy protein 1 [EC:3.6.5.5]
D 100931395 OPA3; optic atrophy 3 protein K23166 OPA3; optic atrophy 3 protein
D 100918786 TRIAP1; TP53-regulated inhibitor of apoptosis 1 K17968 TRIAP1; TRIAP1/MDM35 family protein
D 100926501 PARL; presenilins-associated rhomboid-like protein, mitochondrial K09650 PARL; rhomboid-like protein [EC:3.4.21.105]
D 100916731 BAK1; bcl-2 homologous antagonist/killer isoform X2 K14021 BAK; Bcl-2 homologous antagonist/killer
D 100928220 DNM1L; dynamin-1-like protein isoform X1 K17065 DNM1L; dynamin 1-like protein [EC:3.6.5.5]
D 100921615 FIS1; mitochondrial fission 1 protein K17969 FIS1; mitochondrial fission 1 protein
D 100920473 SUMO2; small ubiquitin-related modifier 2 K12160 SUMO; small ubiquitin-related modifier
D 100925194 SUMO3; small ubiquitin-related modifier 3 K12160 SUMO; small ubiquitin-related modifier
D 100934141 small ubiquitin-related modifier 2-A-like K12160 SUMO; small ubiquitin-related modifier
D 105750041 SUMO1; small ubiquitin-related modifier 1 K12160 SUMO; small ubiquitin-related modifier
D 100934970 SH3GLB1; endophilin-B1 isoform X1 K11248 SH3GLB1; endophilin-B1
D 100930530 MTCH1; mitochondrial carrier homolog 1 K17885 MTCH; mitochondrial carrier
D 100933764 MTCH2; mitochondrial carrier homolog 2 isoform X1 K17885 MTCH; mitochondrial carrier
D 100914721 MFF; mitochondrial fission factor isoform X1 K22076 MFF; mitochondrial fission factor
D 100927653 GDAP1; ganglioside-induced differentiation-associated protein 1 K22077 GDAP1; ganglioside-induced differentiation-associated protein 1
D 100927821 GDAP1L1; ganglioside-induced differentiation-associated protein 1-like 1 isoform X2 K22077 GDAP1; ganglioside-induced differentiation-associated protein 1
D 100914835 VAT1; synaptic vesicle membrane protein VAT-1 homolog K23167 VAT1; synaptic vesicle membrane protein VAT-1
D 100913963 MIEF1; mitochondrial dynamics protein MID51 isoform X1 K23507 MIEF1_2; mitochondrial dynamics protein MID51/49
D 100919752 MIEF2; mitochondrial dynamics protein MID49 K23507 MIEF1_2; mitochondrial dynamics protein MID51/49
D 100934329 PLD6; LOW QUALITY PROTEIN: mitochondrial cardiolipin hydrolase K16862 PLD6; mitochondrial cardiolipin hydrolase [EC:3.1.4.-]
D 100918393 MIGA1; mitoguardin 1 isoform X1 K27289 MIGA; mitoguardin
D 100926445 MIGA2; mitoguardin 2 K27289 MIGA; mitoguardin
C Actin cytoskeleton organization
D 100916712 LOW QUALITY PROTEIN: actin, cytoplasmic 2 K05692 ACTB_G1; actin beta/gamma 1
D 100919051 ACTB; actin, cytoplasmic 1 K05692 ACTB_G1; actin beta/gamma 1
D 100923807 actin, cytoplasmic type 5 K05692 ACTB_G1; actin beta/gamma 1
D 100928302 ACTG1; actin, cytoplasmic 2 K05692 ACTB_G1; actin beta/gamma 1
D 100921304 N-alpha-acetyltransferase 20 K17972 NAA20; N-terminal acetyltransferase B complex catalytic subunit [EC:2.3.1.254]
D 116421451 N-alpha-acetyltransferase 20 K17972 NAA20; N-terminal acetyltransferase B complex catalytic subunit [EC:2.3.1.254]
D 100931113 NAA25; N-alpha-acetyltransferase 25, NatB auxiliary subunit K17973 NAA25; N-terminal acetyltransferase B complex non-catalytic subunit
C Mitochondrial movement
D 100926833 PHB; prohibitin K17080 PHB1; prohibitin 1
D 100924504 PHB2; prohibitin-2 K17081 PHB2; prohibitin 2
D 100928589 KTN1; kinectin isoform X1 K17975 KTN1; kinectin
C Retention of mitochondria
D 100928220 DNM1L; dynamin-1-like protein isoform X1 K17065 DNM1L; dynamin 1-like protein [EC:3.6.5.5]
D 100913898 MYO5C; unconventional myosin-Vc isoform X1 K10357 MYO5; myosin V
D 100914159 MYO5A; unconventional myosin-Va isoform X1 K10357 MYO5; myosin V
D 100920694 MYO5B; unconventional myosin-Vb isoform X1 K10357 MYO5; myosin V
D 100928240 XIAP; E3 ubiquitin-protein ligase XIAP K04725 XIAP; E3 ubiquitin-protein ligase XIAP [EC:2.3.2.27]
D 100925410 DIABLO; diablo homolog, mitochondrial K10522 DIABLO; diablo
C Morphogenesis factors
D 100919795 SNX25; sorting nexin-25 isoform X1 K17887 SNX25; sorting nexin-25
D 100921534 RHOT1; mitochondrial Rho GTPase 1 isoform X1 K07870 RHOT1; mitochondrial Rho GTPase 1 [EC:3.6.5.-]
D 100918254 IMMT; MICOS complex subunit MIC60 isoform X1 K17785 IMMT; MICOS complex subunit MIC60
D 100932081 MTFP1; mitochondrial fission process protein 1 K17981 MTFP1; mitochondrial fission process protein 1
D 100932019 DAP3; 28S ribosomal protein S29, mitochondrial isoform X2 K17408 DAP3; small subunit ribosomal protein S29
D 100916239 NEDD4; E3 ubiquitin-protein ligase NEDD4 isoform X1 K10591 NEDD4; E3 ubiquitin-protein ligase NEDD4 [EC:2.3.2.26]
D 100922877 OXR1; oxidation resistance protein 1 isoform X1 K25437 OXR1; oxidation resistance protein 1
D 100920991 MSTO1; protein misato homolog 1 isoform X1 K27320 MSTO1; protein misato
D 100913725 PRELID1; PRELI domain-containing protein 1, mitochondrial K27964 PRELID1; PRELI domain-containing protein 1, mitochondrial
D 100932051 PRELID2; PRELI domain-containing protein 2 K27965 PRELID2; PRELI domain-containing protein 2 mitochondrial
D 100926874 PRELID3B; PRELI domain containing protein 3B isoform X1 K27966 PRELID3; PRELI domain-containing protein 3, mitochondrial
D 100934238 PRELID3A; PRELI domain containing protein 3A isoform X1 K27966 PRELID3; PRELI domain-containing protein 3, mitochondrial
B Mitophagy factors
C Autophagy-related proteins
D 100914858 ATG2A; autophagy-related protein 2 homolog A K17906 ATG2; autophagy-related protein 2
D 100917633 ATG2B; autophagy-related protein 2 homolog B isoform X1 K17906 ATG2; autophagy-related protein 2
D 100932255 ATG3; ubiquitin-like-conjugating enzyme ATG3 isoform X4 K08343 ATG3; ubiquitin-like-conjugating enzyme ATG3
D 100914909 ATG4C; cysteine protease ATG4C isoform X1 K08342 ATG4; cysteine protease ATG4 [EC:3.4.22.-]
D 100930091 ATG4B; cysteine protease ATG4B isoform X1 K08342 ATG4; cysteine protease ATG4 [EC:3.4.22.-]
D 100931463 ATG4D; cysteine protease ATG4D isoform X1 K08342 ATG4; cysteine protease ATG4 [EC:3.4.22.-]
D 100931882 ATG4A; cysteine protease ATG4A isoform X1 K08342 ATG4; cysteine protease ATG4 [EC:3.4.22.-]
D 100934445 ATG5; autophagy protein 5 isoform X1 K08339 ATG5; autophagy-related protein 5
D 100931761 BECN1; beclin-1 isoform X1 K08334 BECN; beclin
D 100933380 ATG7; ubiquitin-like modifier-activating enzyme ATG7 K08337 ATG7; ubiquitin-like modifier-activating enzyme ATG7
D 100930358 GABARAP; gamma-aminobutyric acid receptor-associated protein isoform X1 K08341 GABARAP; GABA(A) receptor-associated protein
D 100931278 GABARAPL2; gamma-aminobutyric acid receptor-associated protein-like 2 K08341 GABARAP; GABA(A) receptor-associated protein
D 100931419 GABARAPL1; gamma-aminobutyric acid receptor-associated protein-like 1 K08341 GABARAP; GABA(A) receptor-associated protein
D 100915884 ATG9B; autophagy-related protein 9B isoform X1 K17907 ATG9; autophagy-related protein 9
D 100933049 ATG9A; autophagy-related protein 9A K17907 ATG9; autophagy-related protein 9
D 100918781 ATG10; ubiquitin-like-conjugating enzyme ATG10 isoform X1 K17888 ATG10L; ubiquitin-like-conjugating enzyme ATG10
D 100923575 ATG12; ubiquitin-like protein ATG12 K08336 ATG12; ubiquitin-like protein ATG12
D 100924525 ATG14; beclin 1-associated autophagy-related key regulator K17889 ATG14L; beclin 1-associated autophagy-related key regulator
D 100915878 ATG16L1; autophagy-related protein 16-1 isoform X1 K17890 ATG16L1; autophagy-related protein 16-1
D 100926478 ATG16L2; autophagy-related protein 16-2 isoform X1 K20868 ATG16L2; autophagy-related protein 16-2
D 100920269 WIPI2; WD repeat domain phosphoinositide-interacting protein 2 isoform X1 K17908 WIPI1_2; autophagy-related protein 18
D 100931931 WIPI1; WD repeat domain phosphoinositide-interacting protein 1 isoform X1 K17908 WIPI1_2; autophagy-related protein 18
C Parkin-dependent mechanism factors
D 100928463 PRKN; E3 ubiquitin-protein ligase parkin isoform X1 K04556 PARK2; parkin [EC:2.3.2.31]
D 100918310 RNF41; E3 ubiquitin-protein ligase NRDP1 isoform X1 K11981 RNF41; E3 ubiquitin-protein ligase NRDP1 [EC:2.3.2.27]
D 100918962 CLEC16A; protein CLEC16A isoform X1 K19513 CLEC16A; protein CLEC16A
D 100928885 PINK1; serine/threonine-protein kinase PINK1, mitochondrial K05688 PINK1; PTEN induced putative kinase 1 [EC:2.7.11.1]
D 100926501 PARL; presenilins-associated rhomboid-like protein, mitochondrial K09650 PARL; rhomboid-like protein [EC:3.4.21.105]
D 100924705 SQSTM1; sequestosome-1 isoform X1 K14381 SQSTM1; sequestosome 1
D 100918309 TAX1BP1; tax1-binding protein 1 isoform X1 K21347 TAX1BP1; Tax1-binding protein 1
D 100915090 CALCOCO2; calcium-binding and coiled-coil domain-containing protein 2 isoform X1 K21348 CALCOCO2; calcium-binding and coiled-coil domain-containing protein 2
D 100922467 LOW QUALITY PROTEIN: calcium-binding and coiled-coil domain-containing protein 2-like K21348 CALCOCO2; calcium-binding and coiled-coil domain-containing protein 2
D 100914667 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 100930382 ATG13; autophagy-related protein 13 isoform X1 K08331 ATG13; autophagy-related protein 13
D 100934149 ULK1; serine/threonine-protein kinase ULK1 isoform X1 K21357 ULK1; serine/threonine-protein kinase ULK1 [EC:2.7.11.1]
D 100934448 ULK2; serine/threonine-protein kinase ULK2 K08269 ULK2; serine/threonine-protein kinase ULK2 [EC:2.7.11.1]
D 100933709 RB1CC1; RB1-inducible coiled-coil protein 1 isoform X1 K17589 RB1CC1; RB1-inducible coiled-coil protein 1
D 100928900 HSP90AB1; heat shock protein HSP 90-beta K04079 HSP90A; molecular chaperone HtpG
D 100929957 HSP90AA1; heat shock protein HSP 90-alpha K04079 HSP90A; molecular chaperone HtpG
D 100932241 CDC37; hsp90 co-chaperone Cdc37 K09554 CDC37; cell division cycle protein 37
D 100924021 SMURF1; E3 ubiquitin-protein ligase SMURF1 isoform X1 K04678 SMURF; E3 ubiquitin ligase SMURF1/2 [EC:2.3.2.26]
D 100932418 BNIP3L; BCL2/adenovirus E1B 19 kDa protein-interacting protein 3-like isoform X1 K15465 BNIP3L; BCL2/adenovirus E1B 19 kDa protein-interacting protein 3-like
D 100931227 FUNDC1; FUN14 domain-containing protein 1 K17986 FUNDC1; FUN14 domain-containing protein 1
C Other mitophagy factors
D 100926139 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 100928944 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 100914051 MAP1LC3C; microtubule-associated proteins 1A/1B light chain 3C K10435 MAP1LC; microtubule-associated protein 1 light chain
D 100927558 MAP1LC3A; microtubule-associated proteins 1A/1B light chain 3A K10435 MAP1LC; microtubule-associated protein 1 light chain
D 100928511 MAP1LC3B; microtubule-associated proteins 1A/1B light chain 3B K10435 MAP1LC; microtubule-associated protein 1 light chain
D 100930527 NBR1; next to BRCA1 gene 1 protein isoform X1 K17987 NBR1; next to BRCA1 gene 1 protein
D 100922021 TECPR1; tectonin beta-propeller repeat-containing protein 1 isoform X1 K17988 TECPR1; tectonin beta-propeller repeat-containing protein 1
D 100915120 ZFYVE1; zinc finger FYVE domain-containing protein 1 isoform X1 K17603 ZFYVE1; zinc finger FYVE domain-containing protein 1
D 100933060 COASY; bifunctional coenzyme A synthase K02318 COASY; phosphopantetheine adenylyltransferase / dephospho-CoA kinase [EC:2.7.7.3 2.7.1.24]
D 100916580 PLA2G6; 85/88 kDa calcium-independent phospholipase A2 isoform X1 K16343 PLA2G6; calcium-independent phospholipase A2 [EC:3.1.1.4]
D 100919461 C2H19orf12; protein C19orf12 homolog K23168 MPAN; protein MPAN
D 100916767 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