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Mitochondrial Biogenesis - Ciconia boyciana (Oriental stork)
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AMitochondrial DNA transcription, translation, and replication factors
B Mitochondrial DNA-encoded proteins
C Mitochondrial respiratory chain complex I
D 809232 ND1; (RefSeq) NADH dehydrogenase subunit 1 K03878 ND1; NADH-ubiquinone oxidoreductase chain 1 [EC:7.1.1.2]
D 809231 ND2; (RefSeq) NADH dehydrogenase subunit 2 K03879 ND2; NADH-ubiquinone oxidoreductase chain 2 [EC:7.1.1.2]
D 809239 ND3; (RefSeq) NADH dehydrogenase subunit 3 K03880 ND3; NADH-ubiquinone oxidoreductase chain 3 [EC:7.1.1.2]
D 809236 ND4; (RefSeq) NADH dehydrogenase subunit 4 K03881 ND4; NADH-ubiquinone oxidoreductase chain 4 [EC:7.1.1.2]
D 809237 ND4L; (RefSeq) NADH dehydrogenase subunit 4L K03882 ND4L; NADH-ubiquinone oxidoreductase chain 4L [EC:7.1.1.2]
D 809235 ND5; (RefSeq) NADH dehydrogenase subunit 5 K03883 ND5; NADH-ubiquinone oxidoreductase chain 5 [EC:7.1.1.2]
D 809234 ND6; (RefSeq) NADH dehydrogenase subunit 6 K03884 ND6; NADH-ubiquinone oxidoreductase chain 6 [EC:7.1.1.2]
C Mitochondrial respiratory chain complex III
D 809230 CYTB; (RefSeq) cytochrome b K00412 CYTB; ubiquinol-cytochrome c reductase cytochrome b subunit
C Cytochrome c oxidase
D 809229 COX1; (RefSeq) cytochrome c oxidase subunit I K02256 COX1; cytochrome c oxidase subunit 1 [EC:7.1.1.9]
D 809233 COX2; (RefSeq) cytochrome c oxidase subunit II K02261 COX2; cytochrome c oxidase subunit 2
D 809241 COX3; (RefSeq) cytochrome c oxidase subunit III K02262 COX3; cytochrome c oxidase subunit 3
C F-type ATPase
D 809238 ATP6; (RefSeq) 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 140643727 POLRMT; (RefSeq) DNA-directed RNA polymerase, mitochondrial isoform X1 K10908 POLRMT; DNA-directed RNA polymerase, mitochondrial [EC:2.7.7.6]
C Mitochondrial transcription factors
D 140655246 TFAM; (RefSeq) transcription factor A, mitochondrial isoform X1 K11830 TFAM; transcription factor A, mitochondrial
D 140649213 TFB1M; (RefSeq) dimethyladenosine transferase 1, mitochondrial isoform X1 K15266 TFB1M; dimethyladenosine transferase 1, mitochondrial [EC:2.1.1.-]
D 140649392 TFB2M; (RefSeq) dimethyladenosine transferase 2, mitochondrial K17653 TFB2M; dimethyladenosine transferase 2, mitochondrial [EC:2.1.1.-]
C Mitochondrial translation factors
D 140649811 MTIF2; (RefSeq) translation initiation factor IF-2, mitochondrial isoform X1 K02519 infB; translation initiation factor IF-2
D 140646467 MTIF3; (RefSeq) translation initiation factor IF-3, mitochondrial K02520 infC; translation initiation factor IF-3
D 140644497 TSFM; (RefSeq) elongation factor Ts, mitochondrial isoform X1 K02357 tsf; elongation factor Ts
D 140645678 TUFM; (RefSeq) elongation factor Tu, mitochondrial K02358 tuf; elongation factor Tu
D 140650441 GFM2; (RefSeq) ribosome-releasing factor 2, mitochondrial isoform X1 K02355 fusA; elongation factor G
D 140654752 GFM1; (RefSeq) elongation factor G, mitochondrial K02355 fusA; elongation factor G
D 140647489 MTERF3; (RefSeq) transcription termination factor 3, mitochondrial isoform X1 K15031 MTERF; mitochondrial transcription termination factor family protein
D 140654902 MTERF4; (RefSeq) transcription termination factor 4, mitochondrial K15031 MTERF; mitochondrial transcription termination factor family protein
D 140655661 MTERF2; (RefSeq) transcription termination factor 2, mitochondrial isoform X1 K15031 MTERF; mitochondrial transcription termination factor family protein
D 140651670 GUF1; (RefSeq) translation factor GUF1, mitochondrial isoform X1 K21594 GUF1; translation factor GUF1, mitochondrial [EC:3.6.5.-]
C RNase MRP
D 140647481 POP1; (RefSeq) ribonucleases P/MRP protein subunit POP1 K01164 POP1; ribonuclease P/MRP protein subunit POP1 [EC:3.1.26.5]
D 140657004 POP4; (RefSeq) ribonuclease P protein subunit p29 K03538 POP4; ribonuclease P protein subunit POP4 [EC:3.1.26.5]
D 140660136 POP5; (RefSeq) ribonuclease P/MRP protein subunit POP5 isoform X1 K03537 POP5; ribonuclease P/MRP protein subunit POP5 [EC:3.1.26.5]
D 140655838 RPP30; (RefSeq) ribonuclease P protein subunit p30 isoform X1 K03539 RPP1; ribonuclease P/MRP protein subunit RPP1 [EC:3.1.26.5]
D 140645346 POP7; (RefSeq) LOW QUALITY PROTEIN: ribonuclease P protein subunit p20 K14527 RPP20; ribonuclease P/MRP protein subunit RPP20 [EC:3.1.26.5]
D 140651199 RPP25L; (RefSeq) ribonuclease P protein subunit p25-like protein isoform X1 K14525 RPP25; ribonucleases P/MRP protein subunit RPP25 [EC:3.1.26.5]
D 140655922 RPP25; (RefSeq) ribonuclease P protein subunit p25 K14525 RPP25; ribonucleases P/MRP protein subunit RPP25 [EC:3.1.26.5]
D 140648411 RPP38; (RefSeq) ribonuclease P protein subunit p38 isoform X1 K14523 RPP38; ribonucleases P/MRP protein subunit RPP38 [EC:3.1.26.5]
D 140647968 RPP40; (RefSeq) ribonuclease P protein subunit p40 K14530 RPP40; ribonucleases P/MRP protein subunit RPP40 [EC:3.1.26.5]
C Mitochondrial ribonuclease P
D 140661626 TRMT10C; (RefSeq) tRNA methyltransferase 10 homolog C K17654 TRMT10C; mitochondrial ribonuclease P protein 1 [EC:2.1.1.-]
D 140645553 HSD17B10; (RefSeq) 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 140652907 PRORP; (RefSeq) mitochondrial ribonuclease P catalytic subunit K17655 MRPP3; mitochondrial ribonuclease P protein 3
C Other mitochondrial DNA transcription and translation factors
D 140659232 MSS51; (RefSeq) putative protein MSS51 homolog, mitochondrial K17656 MSS51; mitochondrial splicing suppressor protein 51
D 140659132 PTCD1; (RefSeq) pentatricopeptide repeat-containing protein 1, mitochondrial isoform X1 K17710 PTCD1; pentatricopeptide repeat domain-containing protein 1
D 140650653 PTCD2; (RefSeq) pentatricopeptide repeat-containing protein 2, mitochondrial K17711 PTCD2; pentatricopeptide repeat domain-containing protein 2
D 140651611 PTCD3; (RefSeq) small ribosomal subunit protein mS39 isoform X1 K17659 PTCD3; pentatricopeptide repeat domain-containing protein 3 (small subunit ribosomal protein S39)
D 140648626 DHX30; (RefSeq) ATP-dependent RNA helicase DHX30 isoform X1 K13185 DHX30; ATP-dependent RNA helicase DHX30 [EC:5.6.2.6]
D 140659530 UQCC1; (RefSeq) ubiquinol-cytochrome c reductase complex assembly factor 1 isoform X1 K17662 CBP3; cytochrome b pre-mRNA-processing protein 3
D 140649020 AFG1L; (RefSeq) AFG1-like ATPase isoform X1 K18798 AFG1; peroxisome-assembly ATPase [EC:3.6.4.7]
D 140652166 ATP23; (RefSeq) mitochondrial inner membrane protease ATP23 homolog isoform X1 K18156 ATP23; mitochondrial inner membrane protease ATP23 [EC:3.4.24.-]
D 140656018 SUPV3L1; (RefSeq) ATP-dependent RNA helicase SUPV3L1, mitochondrial K17675 SUPV3L1; ATP-dependent RNA helicase SUPV3L1/SUV3 [EC:5.6.2.6]
D 140647585 LACTB2; (RefSeq) endoribonuclease LACTB2 isoform X1 K16639 LACTB2; endoribonuclease LACTB2 [EC:3.1.27.-]
D 140646129 SLC25A6; (RefSeq) ADP/ATP translocase 3 K05863 SLC25A4S; solute carrier family 25 (mitochondrial adenine nucleotide translocator), member 4/5/6/31
D 140651701 SLC25A4; (RefSeq) ADP/ATP translocase 1 K05863 SLC25A4S; solute carrier family 25 (mitochondrial adenine nucleotide translocator), member 4/5/6/31
D 140652527 SLC25A31; (RefSeq) LOW QUALITY PROTEIN: ADP/ATP translocase 4 K05863 SLC25A4S; solute carrier family 25 (mitochondrial adenine nucleotide translocator), member 4/5/6/31
D 140658558 SLC25A5; (RefSeq) ADP/ATP translocase 2 K05863 SLC25A4S; solute carrier family 25 (mitochondrial adenine nucleotide translocator), member 4/5/6/31
D 140643961 SLC25A37; (RefSeq) mitoferrin-1 isoform X1 K15113 SLC25A28_37; solute carrier family 25 (mitochondrial iron transporter), member 28/37
D 140656174 SLC25A28; (RefSeq) LOW QUALITY PROTEIN: mitoferrin-2 K15113 SLC25A28_37; solute carrier family 25 (mitochondrial iron transporter), member 28/37
D 140648319 MALSU1; (RefSeq) mitochondrial assembly of ribosomal large subunit protein 1 K25803 MALSU1; mitochondrial assembly of ribosomal large subunit protein 1
D 140661010 ERAL1; (RefSeq) GTPase Era, mitochondrial isoform X1 K03595 era; GTPase
D 140656541 YARS2; (RefSeq) tyrosine--tRNA ligase, mitochondrial K01866 YARS; tyrosyl-tRNA synthetase [EC:6.1.1.1]
D 140662061 YARS1; (RefSeq) tyrosine--tRNA ligase, cytoplasmic K01866 YARS; tyrosyl-tRNA synthetase [EC:6.1.1.1]
D 140648177 LARS2; (RefSeq) leucine--tRNA ligase, mitochondrial isoform X1 K01869 LARS; leucyl-tRNA synthetase [EC:6.1.1.4]
D 140657149 LARS1; (RefSeq) leucine--tRNA ligase, cytoplasmic isoform X1 K01869 LARS; leucyl-tRNA synthetase [EC:6.1.1.4]
D 140653813 DARS2; (RefSeq) aspartate--tRNA ligase, mitochondrial isoform X1 K01876 DARS2; aspartyl-tRNA synthetase [EC:6.1.1.12]
D 140648577 GARS1; (RefSeq) glycine--tRNA ligase K01880 GARS; glycyl-tRNA synthetase [EC:6.1.1.14]
D 140648936 RARS2; (RefSeq) probable arginine--tRNA ligase, mitochondrial isoform X1 K01887 RARS; arginyl-tRNA synthetase [EC:6.1.1.19]
D 140656379 RARS1; (RefSeq) arginine--tRNA ligase, cytoplasmic isoform X1 K01887 RARS; arginyl-tRNA synthetase [EC:6.1.1.19]
D 140649004 QRSL1; (RefSeq) glutamyl-tRNA(Gln) amidotransferase subunit A, mitochondrial K02433 gatA; aspartyl-tRNA(Asn)/glutamyl-tRNA(Gln) amidotransferase subunit A [EC:6.3.5.6 6.3.5.7]
D 140652200 GATB; (RefSeq) 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 140659825 GATC; (RefSeq) 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 140648484 MTPAP; (RefSeq) poly(A) RNA polymerase, mitochondrial K18060 MTPAP; poly(A) RNA polymerase, mitochondrial [EC:2.7.7.19]
D 140658152 PDE12; (RefSeq) 2',5'-phosphodiesterase 12 K19612 PDE12; 2',5'-phosphodiesterase [EC:3.1.13.4 3.1.4.-]
D 140655186 MTG1; (RefSeq) mitochondrial ribosome-associated GTPase 1 K19828 MTG1; mitochondrial GTPase 1
D 140651635 NOA1; (RefSeq) nitric oxide-associated protein 1 K19832 NOA1; nitric oxide-associated protein 1
D 140662105 NSUN3; (RefSeq) tRNA (cytosine(34)-C(5))-methyltransferase, mitochondrial isoform X1 K21969 NSUN3; tRNA (cytosine34-C5)-methyltransferase [EC:2.1.1.-]
D 140654121 NSUN4; (RefSeq) 5-cytosine rRNA methyltransferase NSUN4 isoform X1 K21970 NSUN4; 5-methylcytosine rRNA methyltransferase NSUN4 [EC:2.1.1.-]
D 140658139 RPUSD3; (RefSeq) mitochondrial mRNA pseudouridine synthase RPUSD3 K22537 RPUSD3; mitochondrial RNA pseudouridine synthase RPUSD3 [EC:5.4.99.-]
D 140661805 RPUSD4; (RefSeq) pseudouridylate synthase RPUSD4, mitochondrial isoform X1 K22538 RPUSD4; mitochondrial RNA pseudouridine synthase RPUSD4 [EC:5.4.99.-]
D 140660905 RCC1L; (RefSeq) RCC1-like G exchanging factor-like protein isoform X1 K23495 RCC1L; RCC1-like G exchanging factor-like protein
D 140661361 TRUB2; (RefSeq) pseudouridylate synthase TRUB2, mitochondrial K23497 TRUB2; mitochondrial mRNA pseudouridine synthase TRUB2 [EC:5.4.99.-]
D 140657799 FASTKD1; (RefSeq) FAST kinase domain-containing protein 1, mitochondrial isoform X1 K27754 FASTKD1; FAST kinase domain-containing protein 1
D 140657789 FASTKD2; (RefSeq) FAST kinase domain-containing protein 2, mitochondrial K18190 FASTKD2; FAST kinase domain-containing protein 2
D 140647890 FASTKD3; (RefSeq) FAST kinase domain-containing protein 3, mitochondrial K27755 FASTKD3; FAST kinase domain-containing protein 3
D 140648616 TBRG4; (RefSeq) FAST kinase domain-containing protein 4 K27756 TBRG4; FAST kinase domain-containing protein 4
D 140651647 FASTKD5; (RefSeq) FAST kinase domain-containing protein 5, mitochondrial K27757 FASTKD5; FAST kinase domain-containing protein 5
D 140659800 MTRFR; (RefSeq) mitochondrial translation release factor in rescue isoform X1 K23498 COXPD7; peptide chain release factor
D 140649225 RMND1; (RefSeq) required for meiotic nuclear division protein 1 homolog isoform X1 K23499 RMND1; required for meiotic nuclear division protein 1
D 140652570 GRSF1; (RefSeq) G-rich sequence factor 1 K24984 GRSF1; G-rich sequence factor 1
D 140653229 METTL15; (RefSeq) 12S rRNA N(4)-cytidine methyltransferase METTL15 K25876 METTL15; 12S rRNA N4-methylcytidine (m4C) methyltransferase [EC:2.1.1.-]
D 140644548 MYG1; (RefSeq) MYG1 exonuclease K25877 MYG1; MYG1 exonuclease [EC:3.1.-.-]
D 140649006 MTRES1; (RefSeq) mitochondrial transcription rescue factor 1 K26186 MTRES1; mitochondrial transcription rescue factor 1
B Mitochondrial DNA replication factors
C Mitochondrial DNA polymerase
D 140655322 POLG; (RefSeq) DNA polymerase subunit gamma-1 K02332 POLG; DNA polymerase gamma 1 [EC:2.7.7.7]
D 140660247 POLG2; (RefSeq) DNA polymerase subunit gamma-2 isoform X1 K02333 POLG2; DNA polymerase gamma 2
D 140651656 PRIMPOL; (RefSeq) 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 140661416 ENDOG; (RefSeq) endonuclease G, mitochondrial K01173 ENDOG; endonuclease G, mitochondrial
D 140649647 MGME1; (RefSeq) mitochondrial genome maintenance exonuclease 1 K19465 MGME1; mitochondrial genome maintenance exonuclease 1 [EC:3.1.-.-]
D 140648561 EXOG; (RefSeq) nuclease EXOG, mitochondrial K15050 EXOG; nuclease EXOG, mitochondrial
D 140655444 TWNK; (RefSeq) twinkle mtDNA helicase isoform X1 K17680 TWNK; twinkle mtDNA helicase [EC:5.6.2.3]
D 140654712 PIF1; (RefSeq) ATP-dependent DNA helicase PIF1 K15255 PIF1; ATP-dependent DNA helicase PIF1 [EC:5.6.2.3]
C Other Mitochondrial DNA replication factors
D 140656446 single-stranded DNA-binding protein, mitochondrial-like isoform X1 K03111 ssb; single-strand DNA-binding protein
D 140661476 ATPase family AAA domain-containing protein 3-like K17681 ATAD3A_B; ATPase family AAA domain-containing protein 3A/B
D 140643277 UQCC2; (RefSeq) ubiquinol-cytochrome c reductase complex assembly factor 2 K17682 MNF1; mitochondrial nucleoid factor 1
D 140660510 MTNAP1; (RefSeq) mitochondrial nucleoid-associated protein 1 K27759 MTNAP1; mitochondrial nucleoid-associated protein 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 140656301 DNAJA2; (RefSeq) dnaJ homolog subfamily A member 2 K09503 DNAJA2; DnaJ homolog subfamily A member 2
D 140659588 TOMM34; (RefSeq) mitochondrial import receptor subunit TOM34 K17766 TOM34; mitochondrial import receptor subunit TOM34
D 140660294 RHOT1; (RefSeq) mitochondrial Rho GTPase 1 isoform X1 K07870 RHOT1; mitochondrial Rho GTPase 1 [EC:3.6.5.-]
D 140661918 HSPA8; (RefSeq) heat shock cognate 71 kDa protein K03283 HSPA1_6_8; heat shock 70kDa protein 1/6/8
D 140653656 AIP; (RefSeq) AH receptor-interacting protein isoform X1 K17767 AIP; AH receptor-interacting protein
D 140658686 aryl-hydrocarbon-interacting protein-like 1 K17767 AIP; AH receptor-interacting protein
D 140658645 mitochondrial fission factor homolog A-like K28235 FATE1; fetal and adult testis-expressed transcript protein
B Outer membrane
C Transporter outer membrane (TOM) complex
D 140650711 TOMM5; (RefSeq) mitochondrial import receptor subunit TOM5 homolog K17773 TOM5; mitochondrial import receptor subunit TOM5
D 140643127 TOMM6; (RefSeq) mitochondrial import receptor subunit TOM6 homolog K17772 TOM6; mitochondrial import receptor subunit TOM6
D 140648325 TOMM7; (RefSeq) mitochondrial import receptor subunit TOM7 homolog K17771 TOM7; mitochondrial import receptor subunit TOM7
D 140649351 TOMM20; (RefSeq) mitochondrial import receptor subunit TOM20 homolog isoform X1 K17770 TOM20; mitochondrial import receptor subunit TOM20
D 140655155 TOMM22; (RefSeq) mitochondrial import receptor subunit TOM22 homolog K17769 TOM22; mitochondrial import receptor subunit TOM22
D 140644251 TOMM40L; (RefSeq) mitochondrial import receptor subunit TOM40B isoform X1 K11518 TOM40; mitochondrial import receptor subunit TOM40
D 140645005 TOMM40; (RefSeq) LOW QUALITY PROTEIN: mitochondrial import receptor subunit TOM40 homolog K11518 TOM40; mitochondrial import receptor subunit TOM40
D 140661494 TOMM70; (RefSeq) mitochondrial import receptor subunit TOM70 K17768 TOM70; mitochondrial import receptor subunit TOM70
C TOB/SAM complex
D 140658055 SAMM50; (RefSeq) sorting and assembly machinery component 50 homolog isoform X1 K07277 SAM50; outer membrane protein insertion porin family
C MIM complex
C Porin
D 140656652 VDAC1; (RefSeq) non-selective voltage-gated ion channel VDAC1 K05862 VDAC1; voltage-dependent anion channel protein 1
D 140655411 VDAC2; (RefSeq) non-selective voltage-gated ion channel VDAC2 isoform X1 K15040 VDAC2; voltage-dependent anion channel protein 2
D 140643855 VDAC3; (RefSeq) non-selective voltage-gated ion channel VDAC3 isoform X1 K15041 VDAC3; voltage-dependent anion channel protein 3
C Other outer membrane factor
D 140644275 MTX1; (RefSeq) metaxin-1 isoform X1 K17776 MTX; metaxin
D 140651026 MTX3; (RefSeq) metaxin-3 isoform X1 K17776 MTX; metaxin
D 140657494 MTX2; (RefSeq) metaxin-2 isoform X1 K17776 MTX; metaxin
D 140660985 MYO19; (RefSeq) unconventional myosin-XIX isoform X1 K25838 MYO19; myosin XIX
D 140649573 mitochondrial amidoxime reducing component 2-like K27318 MTARC; mitochondrial amidoxime-reducing component [EC:1.7.-.-]
B Inter membrane space
C Transporter inner membrane (TIM) 9/10 complex
D 140653738 TIMM9; (RefSeq) mitochondrial import inner membrane translocase subunit Tim9 K17777 TIM9; mitochondrial import inner membrane translocase subunit TIM9
D 140653585 TIMM10; (RefSeq) mitochondrial import inner membrane translocase subunit Tim10 K17778 TIM10; mitochondrial import inner membrane translocase subunit TIM10
D 140646716 TIMM10B; (RefSeq) mitochondrial import inner membrane translocase subunit Tim10 B K17779 TIM10B; mitochondrial import inner membrane translocase subunit TIM10B
C TIM8/13 complex
D 140658670 TIMM8A; (RefSeq) mitochondrial import inner membrane translocase subunit Tim8 A K17780 TIM8; mitochondrial import inner membrane translocase subunit TIM8
D 140643654 TIMM13; (RefSeq) mitochondrial import inner membrane translocase subunit Tim13 K17781 TIM13; mitochondrial import inner membrane translocase subunit TIM13
C Mitochondrial intermembrane space assembly (MIA) complex
D 140657963 CHCHD4; (RefSeq) mitochondrial intermembrane space import and assembly protein 40 K17782 MIA40; mitochondrial intermembrane space import and assembly protein 40
D 140659228 GFER; (RefSeq) FAD-linked sulfhydryl oxidase ALR K17783 ERV1; mitochondrial FAD-linked sulfhydryl oxidase [EC:1.8.3.2]
B Inner mambrane
C MICOS complex
D 140661710 MICOS10; (RefSeq) MICOS complex subunit MIC10 K17784 MICOS10; MICOS complex subunit MIC10
D 140651667 IMMT; (RefSeq) MICOS complex subunit MIC60 isoform X1 K17785 IMMT; MICOS complex subunit MIC60
D 140643615 MICOS13; (RefSeq) MICOS complex subunit MIC13 K24624 MICOS13; MICOS complex subunit MIC13
D 140649671 CHCHD3; (RefSeq) MICOS complex subunit MIC19 isoform X1 K17563 CHCHD3; MICOS complex subunit MIC19
D 140657986 CHCHD6; (RefSeq) MICOS complex subunit MIC25 isoform X1 K17564 CHCHD6; MICOS complex subunit MIC25
D 140645409 APOO; (RefSeq) MICOS complex subunit MIC26 isoform X1 K24625 APOO; MICOS complex subunit MIC26
D 140658502 APOOL; (RefSeq) MICOS complex subunit MIC27 K24626 APOOL; MICOS complex subunit MIC27
C MICOS complex (yeast)
D 140661710 MICOS10; (RefSeq) MICOS complex subunit MIC10 K17784 MICOS10; MICOS complex subunit MIC10
D 140651667 IMMT; (RefSeq) MICOS complex subunit MIC60 isoform X1 K17785 IMMT; MICOS complex subunit MIC60
C TIM22 complex
D 140660700 TIMM22; (RefSeq) mitochondrial import inner membrane translocase subunit Tim22 K17790 TIM22; mitochondrial import inner membrane translocase subunit TIM22
C TIM23 complex
D 140656032 mitochondrial import inner membrane translocase subunit Tim23 isoform X1 K17794 TIM23; mitochondrial import inner membrane translocase subunit TIM23
D 140643134 TIMM17A; (RefSeq) mitochondrial import inner membrane translocase subunit Tim17-A K17795 TIM17; mitochondrial import inner membrane translocase subunit TIM17
D 140647793 TIMM21; (RefSeq) mitochondrial import inner membrane translocase subunit Tim21 K17796 TIM21; mitochondrial import inner membrane translocase subunit TIM21
D 140645400 TIMM50; (RefSeq) mitochondrial import inner membrane translocase subunit TIM50 K17496 TIM50; mitochondrial import inner membrane translocase subunit TIM50
C Mitochondrial calcium uniporter complex
D 140655364 MCU; (RefSeq) calcium uniporter protein, mitochondrial isoform X1 K20858 MCU; calcium uniporter protein, mitochondrial
D 140655313 MICU1; (RefSeq) calcium uptake protein 1, mitochondrial isoform X1 K22827 MICU1; calcium uptake protein 1, mitochondrial
D 140646485 MICU2; (RefSeq) calcium uptake protein 2, mitochondrial K22828 MICU2; calcium uptake protein 2, mitochondrial
D 140652377 MCUB; (RefSeq) calcium uniporter regulatory subunit MCUb, mitochondrial K22829 MCUB; calcium uniporter regulatory subunit MCUb, mitochondrial
D 140654555 SMDT1; (RefSeq) essential MCU regulator, mitochondrial K22830 SMDT1; essential MCU regulator, mitochondrial
D 140648017 MCUR1; (RefSeq) mitochondrial calcium uniporter regulator 1 K22137 MCUR1; mitochondrial calcium uniporter regulator 1
C Other inner membrane factors
D 140651876 LETM1; (RefSeq) mitochondrial proton/calcium exchanger protein isoform X1 K17800 LETM1; mitochondrial proton/calcium exchanger protein
D 140652669 IMMP1L; (RefSeq) mitochondrial inner membrane protease subunit 1 K09647 IMP1; mitochondrial inner membrane protease subunit 1 [EC:3.4.21.-]
D 140651842 IMMP2L; (RefSeq) mitochondrial inner membrane protease subunit 2 isoform X1 K09648 IMP2; mitochondrial inner membrane protease subunit 2 [EC:3.4.21.-]
D 140646606 transmembrane protein 126A-like K18157 TMEM126A; transmembrane protein 126A
D 140649279 MPC1; (RefSeq) mitochondrial pyruvate carrier 1 K22138 MPC1; mitochondrial pyruvate carrier 1
D 140662234 MPC2; (RefSeq) mitochondrial pyruvate carrier 2 K22139 MPC2; mitochondrial pyruvate carrier 2
D 140652346 OCIAD1; (RefSeq) OCIA domain-containing protein 1 isoform X1 K28215 OCIAD1; OCIA domain-containing protein 1
B Matrix
C MMC/PAM proteins
D 140643603 TIMM44; (RefSeq) mitochondrial import inner membrane translocase subunit TIM44 isoform X1 K17804 TIM44; mitochondrial import inner membrane translocase subunit TIM44
D 140656460 HSPA9; (RefSeq) stress-70 protein, mitochondrial K04043 dnaK; molecular chaperone DnaK
D 140652504 GRPEL1; (RefSeq) grpE protein homolog 1, mitochondrial K03687 GRPE; molecular chaperone GrpE
D 140656671 GRPEL2; (RefSeq) grpE protein homolog 2, mitochondrial K03687 GRPE; molecular chaperone GrpE
D 140653977 DNAJC19; (RefSeq) mitochondrial import inner membrane translocase subunit TIM14 isoform X1 K09539 DNAJC19; DnaJ homolog subfamily C member 19
D 140658805 PAM16; (RefSeq) mitochondrial import inner membrane translocase subunit TIM16 K17805 PAM16; mitochondrial import inner membrane translocase subunit TIM16
C Other matrix factors
D 140661347 PMPCA; (RefSeq) mitochondrial-processing peptidase subunit alpha K01412 PMPCA; mitochondrial-processing peptidase subunit alpha [EC:3.4.24.64]
D 140650602 PMPCB; (RefSeq) mitochondrial-processing peptidase subunit beta isoform X1 K17732 PMPCB; mitochondrial-processing peptidase subunit beta [EC:3.4.24.64]
D 140646479 MIPEP; (RefSeq) mitochondrial intermediate peptidase isoform X1 K01410 MIPEP; mitochondrial intermediate peptidase [EC:3.4.24.59]
D 140657203 HSPD1; (RefSeq) 60 kDa heat shock protein, mitochondrial K04077 groEL; chaperonin GroEL [EC:5.6.1.7]
D 140659252 DNAJA3; (RefSeq) dnaJ homolog subfamily A member 3, mitochondrial isoform X1 K09504 DNAJA3; DnaJ homolog subfamily A member 3
D 140658156 TAMM41; (RefSeq) phosphatidate cytidylyltransferase, mitochondrial isoform X1 K17807 TAM41; mitochondrial translocator assembly and maintenance protein 41
D 140661188 DNLZ; (RefSeq) LOW QUALITY PROTEIN: DNL-type zinc finger protein K17808 ZIM17; mitochondrial protein import protein ZIM17
D 140645055 ETHE1; (RefSeq) LOW QUALITY PROTEIN: persulfide dioxygenase ETHE1, mitochondrial K17725 ETHE1; sulfur dioxygenase [EC:1.13.11.18]
D 140661006 POLDIP2; (RefSeq) polymerase delta-interacting protein 2 isoform X1 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 140657272 CREB1; (RefSeq) cyclic AMP-responsive element-binding protein 1 isoform X1 K05870 CREB1; cyclic AMP-responsive element-binding protein 1
D 140650441 GFM2; (RefSeq) ribosome-releasing factor 2, mitochondrial isoform X1 K02355 fusA; elongation factor G
D 140654752 GFM1; (RefSeq) elongation factor G, mitochondrial K02355 fusA; elongation factor G
D 140644535 SP1; (RefSeq) transcription factor Sp1 K04684 SP1; transcription factor Sp1
D 140652641 YY1; (RefSeq) transcriptional repressor protein YY1 K09201 YY; transcription factor YY
D 140644298 GABPB2; (RefSeq) GA-binding protein subunit beta-2 isoform X1 K09454 GABPB; GA-binding protein transcription factor, beta
D 140655696 GABPB1; (RefSeq) GA-binding protein subunit beta-1 isoform X1 K09454 GABPB; GA-binding protein transcription factor, beta
D 140655246 TFAM; (RefSeq) transcription factor A, mitochondrial isoform X1 K11830 TFAM; transcription factor A, mitochondrial
C Nuclear respiratory factors
D 140649457 NRF1; (RefSeq) nuclear respiratory factor 1 isoform X1 K11831 NRF1; nuclear respiratory factor 1
D 140657770 NFE2L2; (RefSeq) nuclear factor erythroid 2-related factor 2 isoform X1 K05638 NFE2L2; nuclear factor erythroid 2-related factor 2
D 140656646 metallothionein-2 K14739 MT1_2; metallothionein 1/2
D 140656767 metallothionein-1 K14739 MT1_2; metallothionein 1/2
C Coactivators
D 140651983 PPARGC1A; (RefSeq) peroxisome proliferator-activated receptor gamma coactivator 1-alpha K07202 PPARGC1A; alpha peroxisome proliferator-activated receptor gamma coactivator 1-alpha
D 140656909 PPARGC1B; (RefSeq) peroxisome proliferator-activated receptor gamma coactivator 1-beta K17962 PPARGC1B; peroxisome proliferator-activated receptor gamma coactivator 1-beta
D 140655594 PPRC1; (RefSeq) peroxisome proliferator-activated receptor gamma coactivator-related protein 1 isoform X1 K17963 PPRC1; peroxisome proliferator-activated receptor gamma coactivator-related protein 1
D 140643427 CRTC1; (RefSeq) CREB-regulated transcription coactivator 1 isoform X1 K15309 CRTC1; CREB-regulated transcription coactivator 1
D 140654734 EP300; (RefSeq) histone acetyltransferase p300 isoform X1 K04498 EP300; E1A/CREB-binding protein [EC:2.3.1.48]
D 140659152 CREBBP; (RefSeq) CREB-binding protein isoform X1 K04498 EP300; E1A/CREB-binding protein [EC:2.3.1.48]
C Proteins involved in Fe-S cluster biogenesis
D 140659398 NFS1; (RefSeq) cysteine desulfurase isoform X1 K04487 iscS; cysteine desulfurase [EC:2.8.1.7]
D 140660066 ISCU; (RefSeq) iron-sulfur cluster assembly enzyme ISCU K22068 ISCU; iron-sulfur cluster assembly enzyme ISCU, mitochondrial
D 140647973 LYRM4; (RefSeq) LYR motif-containing protein 4 isoform X1 K22069 LYRM4; LYR motif-containing protein 4
D 140650530 FXN; (RefSeq) frataxin, mitochondrial isoform X1 K19054 FXN; frataxin [EC:1.16.3.1]
D 140646510 FDX1; (RefSeq) adrenodoxin, mitochondrial K22070 FDX1; ferredoxin-1, mitochondrial
D 140645226 FDX2; (RefSeq) ferredoxin-2, mitochondrial K22071 FDX2; ferredoxin-2, mitochondrial
D 140658658 adrenodoxin-like K22071 FDX2; ferredoxin-2, mitochondrial
D 140660455 FDXR; (RefSeq) NADPH:adrenodoxin oxidoreductase, mitochondrial K18914 FDXR; adrenodoxin-NADP+ reductase [EC:1.18.1.6]
D 140656460 HSPA9; (RefSeq) stress-70 protein, mitochondrial K04043 dnaK; molecular chaperone DnaK
D 140659901 HSCB; (RefSeq) iron-sulfur cluster co-chaperone protein HscB isoform X1 K04082 hscB; molecular chaperone HscB
D 140656704 SIL1; (RefSeq) nucleotide exchange factor SIL1 isoform X1 K14001 SIL1; nucleotide exchange factor SIL1
D 140653207 GLRX5; (RefSeq) glutaredoxin-related protein 5, mitochondrial K07390 grxD; monothiol glutaredoxin
D 140650851 ISCA1; (RefSeq) iron-sulfur cluster assembly 1 homolog, mitochondrial K22063 ISCA1; iron-sulfur cluster assembly 1
D 140651142 iron-sulfur cluster assembly 1 homolog, mitochondrial-like K22063 ISCA1; iron-sulfur cluster assembly 1
D 140653468 ISCA2; (RefSeq) iron-sulfur cluster assembly 2 homolog, mitochondrial isoform X1 K22072 ISCA2; iron-sulfur cluster assembly 2
D 140648595 IBA57; (RefSeq) putative transferase CAF17, mitochondrial K22073 IBA57; transferase CAF17, mitochondrial [EC:2.1.-.-]
D 140643929 NFU1; (RefSeq) NFU1 iron-sulfur cluster scaffold homolog, mitochondrial isoform X1 K22074 NFU1; NFU1 iron-sulfur cluster scaffold homolog, mitochondrial
D 140644218 BOLA1; (RefSeq) bolA-like protein 1 K22066 BOLA1; BolA-like protein 1
D 140643943 BOLA3; (RefSeq) bolA-like protein 3 K22075 BOLA3; BolA-like protein 3
D 140655422 SFXN3; (RefSeq) sideroflexin-3 isoform X1 K23500 SFXN1_3; sideroflexin-1/3
D 140656946 SFXN1; (RefSeq) sideroflexin-1 isoform X1 K23500 SFXN1_3; sideroflexin-1/3
D 140655591 SFXN2; (RefSeq) sideroflexin-2 K23501 SFXN2; sideroflexin-2
D 140655482 SFXN4; (RefSeq) sideroflexin-4 K23502 SFXN4; sideroflexin-4
D 140652260 SFXN5; (RefSeq) sideroflexin-5 isoform X1 K23503 SFXN5; sideroflexin-5
C Other regulator of mitochondrial biogenesis
D 140644672 carnitine O-palmitoyltransferase 1, liver isoform-like K08765 CPT1A; carnitine O-palmitoyltransferase 1, liver isoform [EC:2.3.1.21]
D 140652589 LOW QUALITY PROTEIN: carnitine O-palmitoyltransferase 1, liver isoform-like K08765 CPT1A; carnitine O-palmitoyltransferase 1, liver isoform [EC:2.3.1.21]
D 140649243 CPT1B; (RefSeq) LOW QUALITY PROTEIN: carnitine O-palmitoyltransferase 1, muscle isoform K19523 CPT1B; carnitine O-palmitoyltransferase 1, muscle isoform [EC:2.3.1.21]
D 140649936 DHTKD1; (RefSeq) 2-oxoadipate dehydrogenase complex component E1 isoform X1 K15791 DHKTD1; 2-oxoadipate dehydrogenase E1 component [EC:1.2.4.-]
D 140648978 FBXL4; (RefSeq) F-box/LRR-repeat protein 4 isoform X1 K10270 FBXL4; F-box and leucine-rich repeat protein 4
D 140645498 HCFC1; (RefSeq) LOW QUALITY PROTEIN: host cell factor 1 K14966 HCFC1; host cell factor 1
D 140656053 HCFC2; (RefSeq) host cell factor 2 isoform X1 K27390 HCFC2; host cell factor 2
D 140651596 HTT; (RefSeq) huntingtin isoform X1 K04533 HD; huntingtin
D 140653556 ESRRB; (RefSeq) steroid hormone receptor ERR2 isoform X1 K08553 NR3B2; estrogen-related receptor beta
D 140649558 ESRRG; (RefSeq) estrogen-related receptor gamma isoform X1 K08554 NR3B3; estrogen-related receptor gamma
D 140662624 NRIP1; (RefSeq) nuclear receptor-interacting protein 1 K17965 NRIP1; nuclear receptor-interacting protein 1
D 140650590 PRKAA1; (RefSeq) 5'-AMP-activated protein kinase catalytic subunit alpha-1 K07198 PRKAA; 5'-AMP-activated protein kinase, catalytic alpha subunit [EC:2.7.11.31]
D 140654750 PRKAA2; (RefSeq) 5'-AMP-activated protein kinase catalytic subunit alpha-2 isoform X1 K07198 PRKAA; 5'-AMP-activated protein kinase, catalytic alpha subunit [EC:2.7.11.31]
D 140643452 LONP1; (RefSeq) lon protease homolog, mitochondrial isoform X1 K08675 LONP1; ATP-dependent Lon protease [EC:3.4.21.53]
D 140655295 SIRT1; (RefSeq) NAD-dependent protein deacetylase sirtuin-1 K11411 SIRT1; NAD-dependent protein deacetylase sirtuin 1 [EC:2.3.1.286]
D 140649202 SERAC1; (RefSeq) protein SERAC1 isoform X1 K23504 SERAC1; protein SERAC1
D 140645519 UXT; (RefSeq) protein UXT isoform X1 K26537 UXT; protein UXT
D 140661714 PERM1; (RefSeq) PGC-1 and ERR-induced regulator in muscle protein 1 K26738 PERM1; PGC-1 and ERR-induced regulator in muscle protein 1
D 140644605 GATD3; (RefSeq) glutamine amidotransferase-like class 1 domain-containing protein 3, mitochondrial K27835 GATD3; glutamine amidotransferase-like class 1 domain-containing protein 3, mitochondrial
D 140646319 glutamine amidotransferase-like class 1 domain-containing protein 3, mitochondrial K27835 GATD3; glutamine amidotransferase-like class 1 domain-containing protein 3, mitochondrial
D 140658584 glutamine amidotransferase-like class 1 domain-containing protein 3, mitochondrial K27835 GATD3; glutamine amidotransferase-like class 1 domain-containing protein 3, mitochondrial
D 140659732 TANGO2; (RefSeq) transport and Golgi organization protein 2 homolog isoform X1 K28098 TANGO2; transport and Golgi organization protein 2
D 140657705 C10H2orf69; (RefSeq) mitochondrial protein C2orf69 homolog K28271 C2ORF69; mitochondrial protein C2orf69
D 140650807 ATOSB; (RefSeq) atos homolog protein B isoform X1 K28528 ATOS; protein Atossa
D 140655891 ATOSA; (RefSeq) atos homolog protein A isoform X1 K28528 ATOS; protein Atossa
D 140654281 DDX47; (RefSeq) probable ATP-dependent RNA helicase DDX47 K14777 DDX47; ATP-dependent RNA helicase DDX47/RRP3 [EC:5.6.2.7]
D 140651239 PRR16; (RefSeq) LOW QUALITY PROTEIN: protein Largen K28918 PRR16; protein Largen
D 140649831 MTLN; (RefSeq) mitoregulin K29035 MTLN; mitoregulin
B Mitochondrial respiratory chain complex assembly factors
C Complex-I assembly factors
D 140652940 NDUFAF1; (RefSeq) complex I intermediate-associated protein 30, mitochondrial isoform X1 K18159 NDUFAF1; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 1
D 140651179 NDUFAF2; (RefSeq) NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 2 K18160 NDUFAF2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 2
D 140657877 NDUFAF3; (RefSeq) NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 3 isoform X1 K09008 NDUFAF3; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 3
D 140648974 NDUFAF4; (RefSeq) NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 4 K18161 NDUFAF4; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 4
D 140649761 NDUFAF5; (RefSeq) arginine-hydroxylase NDUFAF5, mitochondrial isoform X1 K18162 NDUFAF5; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 5 [EC:2.1.1.-]
D 140647500 NDUFAF6; (RefSeq) NADH dehydrogenase (ubiquinone) complex I, assembly factor 6 isoform X1 K18163 NDUFAF6; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 6
D 140649405 NDUFAF7; (RefSeq) protein arginine methyltransferase NDUFAF7, mitochondrial K18164 NDUFAF7; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 7
D 140660358 NDUFAF8; (RefSeq) NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 8 K24726 NDUFAF8; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 8
D 140645175 ECSIT; (RefSeq) evolutionarily conserved signaling intermediate in Toll pathway, mitochondrial isoform X1 K04405 ECSIT; evolutionarily conserved signaling intermediate in Toll pathway
D 140658104 ACAD9; (RefSeq) complex I assembly factor ACAD9, mitochondrial K15980 ACAD9; acyl-CoA dehydrogenase family member 9 [EC:1.3.99.-]
D 140652815 NUBPL; (RefSeq) iron-sulfur cluster transfer protein NUBPL isoform X1 K03593 mrp; ATP-binding protein involved in chromosome partitioning
D 140661943 FOXRED1; (RefSeq) LOW QUALITY PROTEIN: FAD-dependent oxidoreductase domain-containing protein 1 K18166 FOXRED1; FAD-dependent oxidoreductase domain-containing protein 1
D 140649005 RTN4IP1; (RefSeq) reticulon-4-interacting protein 1, mitochondrial K23164 RTN4IP1; reticulon-4-interacting protein 1, mitochondrial
D 140662365 TIMMDC1; (RefSeq) complex I assembly factor TIMMDC1, mitochondrial K23505 TIMMDC1; complex I assembly factor TIMMDC1
C Complex-II assembly factors
D 140653669 SDHAF2; (RefSeq) succinate dehydrogenase assembly factor 2, mitochondrial K18168 SDHAF2; succinate dehydrogenase assembly factor 2
C Complex-III assembly factors
D 140657298 BCS1L; (RefSeq) mitochondrial chaperone BCS1 isoform X1 K08900 BCS1; mitochondrial chaperone BCS1
D 140660719 TTC19; (RefSeq) tetratricopeptide repeat protein 19, mitochondrial isoform X1 K18169 TTC19; tetratricopeptide repeat protein 19, mitochondrial
D 140651172 LYRM7; (RefSeq) complex III assembly factor LYRM7 K18170 LYRM7; complex III assembly factor LYRM7
D 140659530 UQCC1; (RefSeq) ubiquinol-cytochrome c reductase complex assembly factor 1 isoform X1 K17662 CBP3; cytochrome b pre-mRNA-processing protein 3
D 140643277 UQCC2; (RefSeq) ubiquinol-cytochrome c reductase complex assembly factor 2 K17682 MNF1; mitochondrial nucleoid factor 1
D 140660176 UQCC3; (RefSeq) ubiquinol-cytochrome-c reductase complex assembly factor 3 K23165 UQCC3; ubiquinol-cytochrome-c reductase complex assembly factor 3
D 140659111 UQCC4; (RefSeq) ubiquinol-cytochrome c reductase complex assembly factor 4 K27339 UQCC4; ubiquinol-cytochrome c reductase complex assembly factor 4
D 140658017 UQCC5; (RefSeq) ubiquinol-cytochrome c reductase complex assembly factor 5 K27340 UQCC5; ubiquinol-cytochrome c reductase complex assembly factor 5
D 140656099 UQCC6; (RefSeq) ubiquinol-cytochrome c reductase complex assembly factor 6 K27341 UQCC6; ubiquinol-cytochrome c reductase complex assembly factor 6
D 140656507 STMP1; (RefSeq) short transmembrane mitochondrial protein 1 K27319 STMP1; short transmembrane mitochondrial protein 1
D 140652096 OCIAD2; (RefSeq) LOW QUALITY PROTEIN: OCIA domain-containing protein 2 K29036 OCIAD2; OCIA domain-containing protein 2
C Complex-IV assembly factors
D 140648241 CMC1; (RefSeq) COX assembly mitochondrial protein homolog K18171 CMC1; COX assembly mitochondrial protein 1
D 140656895 CMC2; (RefSeq) COX assembly mitochondrial protein 2 homolog K18172 CMC2; COX assembly mitochondrial protein 2
D 140648059 COA1; (RefSeq) cytochrome c oxidase assembly factor 1 homolog K18173 COA1; cytochrome c oxidase assembly factor 1
D 140646653 COA4; (RefSeq) cytochrome c oxidase assembly factor 4 homolog, mitochondrial K18177 COA4; cytochrome c oxidase assembly factor 4
D 140646159 COA5; (RefSeq) cytochrome c oxidase assembly factor 5 K18178 COA5; cytochrome c oxidase assembly factor 5
D 140650102 COA6; (RefSeq) cytochrome c oxidase assembly factor 6 homolog K18179 COA6; cytochrome c oxidase assembly factor 6
D 140654847 COA7; (RefSeq) cytochrome c oxidase assembly factor 7 K18180 COA7; cytochrome c oxidase assembly factor 7
D 140660348 COX10; (RefSeq) protoheme IX farnesyltransferase, mitochondrial K02257 COX10; heme o synthase [EC:2.5.1.141]
D 140660209 COX11; (RefSeq) cytochrome c oxidase assembly protein COX11, mitochondrial K02258 COX11; cytochrome c oxidase assembly protein subunit 11
D 140644526 COX14; (RefSeq) cytochrome c oxidase assembly protein COX14 K18181 COX14; cytochrome c oxidase assembly factor 14
D 140656175 COX15; (RefSeq) heme A synthase COX15 K02259 COX15; heme a synthase [EC:1.17.99.9]
D 140653234 COX16; (RefSeq) cytochrome c oxidase assembly protein COX16 homolog, mitochondrial K18182 COX16; cytochrome c oxidase assembly protein subunit 16
D 140660600 COX17; (RefSeq) cytochrome c oxidase copper chaperone isoform X1 K02260 COX17; cytochrome c oxidase assembly protein subunit 17
D 140651575 COX18; (RefSeq) cytochrome c oxidase assembly protein COX18, mitochondrial K17797 COX18; mitochondrial inner membrane protein COX18
D 140659145 COX19; (RefSeq) cytochrome c oxidase assembly protein COX19 K18183 COX19; cytochrome c oxidase assembly protein subunit 19
D 140649386 COX20; (RefSeq) cytochrome c oxidase assembly protein COX20, mitochondrial K18184 COX20; cytochrome c oxidase assembly protein subunit 20
D 140655852 C8H15orf48; (RefSeq) normal mucosa of esophagus-specific gene 1 protein K29037 COXFA4L3; cytochrome c oxidase associated subunit FA4L3
D 140659232 MSS51; (RefSeq) putative protein MSS51 homolog, mitochondrial K17656 MSS51; mitochondrial splicing suppressor protein 51
D 140645148 PET100; (RefSeq) LOW QUALITY PROTEIN: protein PET100 homolog, mitochondrial K18186 PET100; protein PET100, animal type
D 140649645 PET117; (RefSeq) protein PET117 homolog, mitochondrial isoform X1 K18188 PET117; protein PET117
D 140645759 OXA1L; (RefSeq) mitochondrial inner membrane protein OXA1L K03217 yidC; YidC/Oxa1 family membrane protein insertase
D 140661101 SURF1; (RefSeq) surfeit locus protein 1 K14998 SURF1; surfeit locus 1 family protein
D 140660596 SCO1; (RefSeq) LOW QUALITY PROTEIN: protein SCO1 homolog, mitochondrial K07152 SCO1; protein SCO1
D 140649301 SCO2; (RefSeq) protein SCO2 homolog, mitochondrial K23755 SCO2; protein SCO2
D 140645688 TACO1; (RefSeq) translational activator of cytochrome c oxidase 1 isoform X1 K18189 TACO1; translational activator of cytochrome c oxidase 1
D 140649499 LRPPRC; (RefSeq) leucine-rich PPR motif-containing protein, mitochondrial K17964 LRPPRC; leucine-rich PPR motif-containing protein, mitochondrial
D 140653190 COA8; (RefSeq) cytochrome c oxidase assembly factor 8 isoform X1 K23506 APOPT1; apoptogenic protein 1
D 140656507 STMP1; (RefSeq) short transmembrane mitochondrial protein 1 K27319 STMP1; short transmembrane mitochondrial protein 1
D 140658017 UQCC5; (RefSeq) ubiquinol-cytochrome c reductase complex assembly factor 5 K27340 UQCC5; ubiquinol-cytochrome c reductase complex assembly factor 5
D 140660172 TMEM223; (RefSeq) transmembrane protein 223 K27342 TMEM223; transmembrane protein 223
D 140648592 HIGD1A; (RefSeq) HIG1 domain family member 1A, mitochondrial isoform X1 K28216 HIGD1A_C; HIG1 domain family member 1A/C, mitochondrial
D 140656692 HIGD2A; (RefSeq) HIG1 domain family member 2A, mitochondrial K28217 HIGD2A; HIG1 domain family member 2A, mitochondrial
C Complex-V assembly factors
D 140654661 ATPAF1; (RefSeq) ATP synthase mitochondrial F1 complex assembly factor 1 K07555 ATPeAF1; ATP synthase mitochondrial F1 complex assembly factor 1
D 140658939 ATPAF2; (RefSeq) ATP synthase mitochondrial F1 complex assembly factor 2 isoform X1 K07556 ATPeAF2; ATP synthase mitochondrial F1 complex assembly factor 2
D 140647574 TMEM70; (RefSeq) transmembrane protein 70, mitochondrial isoform X1 K17966 TMEM70; transmembrane protein 70, mitochondrial
D 140652608 ATP5MJ; (RefSeq) ATP synthase subunit ATP5MJ, mitochondrial isoform X1 K18193 MP68; 6.8 kDa mitochondrial proteolipid
D 140655159 ATP5MK; (RefSeq) ATP synthase membrane subunit K, mitochondrial K18194 USMG5; up-regulated during skeletal muscle growth protein 5
B Mitochondrial dynamics
C Fission and Fusion factors
D 140653912 MFN1; (RefSeq) mitofusin-1 isoform X1 K21356 MFN1; mitofusin 1 [EC:3.6.5.-]
D 140661653 MFN2; (RefSeq) mitofusin-2 K06030 MFN2; mitofusin 2 [EC:3.6.5.-]
D 140653900 OPA1; (RefSeq) dynamin-like GTPase OPA1, mitochondrial K17079 OPA1; optic atrophy protein 1 [EC:3.6.5.5]
D 140645470 OPA3; (RefSeq) optic atrophy 3 protein K23166 OPA3; optic atrophy 3 protein
D 140659827 TRIAP1; (RefSeq) TP53-regulated inhibitor of apoptosis 1 isoform X1 K17968 TRIAP1; TRIAP1/MDM35 family protein
D 140653857 PARL; (RefSeq) presenilin-associated rhomboid-like protein, mitochondrial K09650 PARL; rhomboid-like protein [EC:3.4.21.105]
D 140643357 BAK1; (RefSeq) bcl-2 homologous antagonist/killer K14021 BAK; Bcl-2 homologous antagonist/killer
D 140644691 apoptosis regulator BAX-like isoform X1 K02159 BAX; apoptosis regulator BAX
D 140644692 BAX; (RefSeq) apoptosis regulator BAX isoform X1 K02159 BAX; apoptosis regulator BAX
D 140656517 DNM1L; (RefSeq) dynamin-1-like protein isoform X1 K17065 DNM1L; dynamin 1-like protein [EC:3.6.5.5]
D 140645243 FIS1; (RefSeq) mitochondrial fission 1 protein K17969 FIS1; mitochondrial fission 1 protein
D 140654960 small ubiquitin-related modifier 3 K12160 SUMO; small ubiquitin-related modifier
D 140657202 SUMO1; (RefSeq) small ubiquitin-related modifier 1 K12160 SUMO; small ubiquitin-related modifier
D 140660282 SUMO2; (RefSeq) small ubiquitin-related modifier 2 isoform X1 K12160 SUMO; small ubiquitin-related modifier
D 140654495 SH3GLB1; (RefSeq) endophilin-B1 isoform X1 K11248 SH3GLB1; endophilin-B1
D 140653659 MTCH2; (RefSeq) mitochondrial carrier homolog 2 K17885 MTCH; mitochondrial carrier
D 140662723 MTCH1; (RefSeq) mitochondrial carrier homolog 1 K17885 MTCH; mitochondrial carrier
D 140653935 MFF; (RefSeq) mitochondrial fission factor isoform X1 K22076 MFF; mitochondrial fission factor
D 140647573 GDAP1; (RefSeq) ganglioside-induced differentiation-associated protein 1 isoform X1 K22077 GDAP1; ganglioside-induced differentiation-associated protein 1
D 140659363 GDAP1L1; (RefSeq) ganglioside-induced differentiation-associated protein 1-like 1 K22077 GDAP1; ganglioside-induced differentiation-associated protein 1
D 140656915 VAT1L; (RefSeq) synaptic vesicle membrane protein VAT-1 homolog-like K23167 VAT1; synaptic vesicle membrane protein VAT-1 [EC:1.6.5.5]
D 140662304 VAT1; (RefSeq) synaptic vesicle membrane protein VAT-1 homolog K23167 VAT1; synaptic vesicle membrane protein VAT-1 [EC:1.6.5.5]
D 140654900 MIEF1; (RefSeq) mitochondrial dynamics protein MIEF1 isoform X1 K23507 MIEF1_2; mitochondrial dynamics protein MID51/49
D 140659140 MIEF2; (RefSeq) mitochondrial dynamics protein MID49 K23507 MIEF1_2; mitochondrial dynamics protein MID51/49
D 140659118 PLD6; (RefSeq) mitochondrial cardiolipin hydrolase K16862 PLD6; mitochondrial cardiolipin hydrolase [EC:3.1.4.-]
D 140653889 MIGA1; (RefSeq) mitoguardin 1 K27289 MIGA; mitoguardin
D 140661274 MIGA2; (RefSeq) mitoguardin 2 isoform X1 K27289 MIGA; mitoguardin
C Actin cytoskeleton organization
D 140655212 actin, cytoplasmic type 5 K05692 ACTB_G1; actin beta/gamma 1
D 140658857 ACTB; (RefSeq) actin, cytoplasmic 1 K05692 ACTB_G1; actin beta/gamma 1
D 140660266 ACTG1; (RefSeq) actin, cytoplasmic 2 K05692 ACTB_G1; actin beta/gamma 1
D 140654001 N-alpha-acetyltransferase 20 isoform X1 K17972 NAA20; N-terminal acetyltransferase B complex catalytic subunit [EC:2.3.1.254]
D 140659880 NAA25; (RefSeq) N-alpha-acetyltransferase 25, NatB auxiliary subunit K17973 NAA25; N-terminal acetyltransferase B complex non-catalytic subunit
C Mitochondrial movement
D 140662483 PHB1; (RefSeq) prohibitin 1 K17080 PHB1; prohibitin 1
D 140659683 PHB2; (RefSeq) prohibitin-2 K17081 PHB2; prohibitin 2
D 140652877 KTN1; (RefSeq) kinectin isoform X1 K17975 KTN1; kinectin
C Retention of mitochondria
D 140656517 DNM1L; (RefSeq) dynamin-1-like protein isoform X1 K17065 DNM1L; dynamin 1-like protein [EC:3.6.5.5]
D 140643708 unconventional myosin-Vb-like K10357 MYO5; myosin V
D 140650834 MYO5B; (RefSeq) LOW QUALITY PROTEIN: unconventional myosin-Vb K10357 MYO5; myosin V
D 140655196 MYO5A; (RefSeq) unconventional myosin-Va isoform X1 K10357 MYO5; myosin V
D 140655573 MYO5C; (RefSeq) unconventional myosin-Vc K10357 MYO5; myosin V
D 140658532 XIAP; (RefSeq) LOW QUALITY PROTEIN: E3 ubiquitin-protein ligase XIAP K04725 XIAP; E3 ubiquitin-protein ligase XIAP [EC:2.3.2.27]
D 140660028 DIABLO; (RefSeq) diablo IAP-binding mitochondrial protein isoform X1 K10522 DIABLO; diablo
C Morphogenesis factors
D 140652170 SNX25; (RefSeq) sorting nexin-25 K17887 SNX25; sorting nexin-25
D 140660294 RHOT1; (RefSeq) mitochondrial Rho GTPase 1 isoform X1 K07870 RHOT1; mitochondrial Rho GTPase 1 [EC:3.6.5.-]
D 140651667 IMMT; (RefSeq) MICOS complex subunit MIC60 isoform X1 K17785 IMMT; MICOS complex subunit MIC60
D 140660006 MTFP1; (RefSeq) mitochondrial fission process protein 1 K17981 MTFP1; mitochondrial fission process protein 1
D 140654647 C7H3orf33; (RefSeq) protein C3orf33 homolog isoform X1 K29121 MISO1; mitochondrial inner membrane subdomain organizer 1
D 140644307 DAP3; (RefSeq) small ribosomal subunit protein mS29 K17408 DAP3; small subunit ribosomal protein S29
D 140655792 NEDD4; (RefSeq) E3 ubiquitin-protein ligase NEDD4 isoform X1 K10591 NEDD4; E3 ubiquitin-protein ligase NEDD4 [EC:2.3.2.26]
D 140647437 OXR1; (RefSeq) oxidation resistance protein 1 isoform X1 K25437 OXR1; oxidation resistance protein 1
D 140644305 MSTO1; (RefSeq) protein misato homolog 1 isoform X1 K27320 MSTO1; protein misato
D 140656393 PRELID1; (RefSeq) PRELI domain-containing protein 1, mitochondrial K27964 PRELID1; PRELI domain-containing protein 1, mitochondrial
D 140656320 PRELID2; (RefSeq) PRELI domain-containing protein 2 K27965 PRELID2; PRELI domain-containing protein 2 mitochondrial
D 140647777 PRELID3A; (RefSeq) PRELI domain containing protein 3A isoform X1 K27966 PRELID3; PRELI domain-containing protein 3, mitochondrial
D 140659522 PRELID3B; (RefSeq) PRELI domain containing protein 3B K27966 PRELID3; PRELI domain-containing protein 3, mitochondrial
D 140655589 TM2D3; (RefSeq) TM2 domain-containing protein 3 isoform X1 K28688 TM2D3; TM2 domain-containing protein 3
B Mitophagy factors
C Autophagy-related proteins
D 140652739 ATG2B; (RefSeq) autophagy-related protein 2 homolog B isoform X1 K17906 ATG2; autophagy-related protein 2
D 140661044 ATG3; (RefSeq) ubiquitin-like-conjugating enzyme ATG3 isoform X1 K08343 ATG3; ubiquitin-like-conjugating enzyme ATG3
D 140645101 ATG4D; (RefSeq) cysteine protease ATG4D K08342 ATG4; cysteine protease ATG4 [EC:3.4.22.-]
D 140653809 ATG4B; (RefSeq) cysteine protease ATG4B isoform X1 K08342 ATG4; cysteine protease ATG4 [EC:3.4.22.-]
D 140654306 ATG4C; (RefSeq) cysteine protease ATG4C K08342 ATG4; cysteine protease ATG4 [EC:3.4.22.-]
D 140658372 ATG4A; (RefSeq) cysteine protease ATG4A isoform X1 K08342 ATG4; cysteine protease ATG4 [EC:3.4.22.-]
D 140649003 ATG5; (RefSeq) autophagy protein 5 isoform X1 K08339 ATG5; autophagy-related protein 5
D 140662614 BECN1; (RefSeq) beclin-1 K08334 BECN; beclin
D 140658155 ATG7; (RefSeq) ubiquitin-like modifier-activating enzyme ATG7 isoform X1 K08337 ATG7; ubiquitin-like modifier-activating enzyme ATG7
D 140645338 GABARAP; (RefSeq) gamma-aminobutyric acid receptor-associated protein K08341 GABARAP; GABA(A) receptor-associated protein
D 140657014 GABARAPL2; (RefSeq) gamma-aminobutyric acid receptor-associated protein-like 2 K08341 GABARAP; GABA(A) receptor-associated protein
D 140658959 GABARAPL1; (RefSeq) gamma-aminobutyric acid receptor-associated protein-like 1 K08341 GABARAP; GABA(A) receptor-associated protein
D 140648657 ATG9B; (RefSeq) autophagy-related protein 9B K17907 ATG9; autophagy-related protein 9
D 140657233 ATG9A; (RefSeq) autophagy-related protein 9A K17907 ATG9; autophagy-related protein 9
D 140651344 ATG10; (RefSeq) ubiquitin-like-conjugating enzyme ATG10 K17888 ATG10L; ubiquitin-like-conjugating enzyme ATG10
D 140651237 ATG12; (RefSeq) ubiquitin-like protein ATG12 K08336 ATG12; ubiquitin-like protein ATG12
D 140652647 ATG14; (RefSeq) beclin 1-associated autophagy-related key regulator K17889 ATG14L; beclin 1-associated autophagy-related key regulator
D 140654159 ATG16L1; (RefSeq) autophagy-related protein 16-1 isoform X1 K17890 ATG16L1; autophagy-related protein 16-1
D 140658999 WIPI2; (RefSeq) WD repeat domain phosphoinositide-interacting protein 2 isoform X1 K17908 WIPI1_2; autophagy-related protein 18
D 140660422 WIPI1; (RefSeq) WD repeat domain phosphoinositide-interacting protein 1 isoform X1 K17908 WIPI1_2; autophagy-related protein 18
D 140650071 uncharacterized protein K08332 VAC8; vacuolar protein 8
C Parkin-dependent mechanism factors
D 140650084 PRKN; (RefSeq) E3 ubiquitin-protein ligase parkin K04556 PARK2; parkin [EC:2.3.2.31]
D 140644579 RNF41; (RefSeq) E3 ubiquitin-protein ligase NRDP1 isoform X1 K11981 RNF41; E3 ubiquitin-protein ligase NRDP1 [EC:2.3.2.27]
D 140659207 CLEC16A; (RefSeq) protein CLEC16A isoform X1 K19513 CLEC16A; protein CLEC16A
D 140661634 PINK1; (RefSeq) serine/threonine-protein kinase PINK1, mitochondrial K05688 PINK1; PTEN induced putative kinase 1 [EC:2.7.11.1]
D 140653857 PARL; (RefSeq) presenilin-associated rhomboid-like protein, mitochondrial K09650 PARL; rhomboid-like protein [EC:3.4.21.105]
D 140656643 SQSTM1; (RefSeq) sequestosome-1 isoform X1 K14381 SQSTM1; sequestosome 1
D 140648288 TAX1BP1; (RefSeq) tax1-binding protein 1 isoform X1 K21347 TAX1BP1; Tax1-binding protein 1
D 140662570 CALCOCO2; (RefSeq) calcium-binding and coiled-coil domain-containing protein 2 K21348 CALCOCO2; calcium-binding and coiled-coil domain-containing protein 2
D 140653212 AMBRA1; (RefSeq) 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 140653213 ATG13; (RefSeq) autophagy-related protein 13 isoform X1 K08331 ATG13; autophagy-related protein 13
D 140659820 ULK1; (RefSeq) serine/threonine-protein kinase ULK1 isoform X1 K21357 ULK1; serine/threonine-protein kinase ULK1 [EC:2.7.11.1]
D 140660980 ULK2; (RefSeq) serine/threonine-protein kinase ULK2 K08269 ULK2; serine/threonine-protein kinase ULK2 [EC:2.7.11.1]
D 140647652 RB1CC1; (RefSeq) RB1-inducible coiled-coil protein 1 isoform X1 K17589 RB1CC1; RB1-inducible coiled-coil protein 1
D 140649450 HSP90AB1; (RefSeq) heat shock protein HSP 90-beta K04079 HSP90A; molecular chaperone HtpG
D 140652625 HSP90AA1; (RefSeq) heat shock protein HSP 90-alpha K04079 HSP90A; molecular chaperone HtpG
D 140645215 CDC37; (RefSeq) hsp90 co-chaperone Cdc37 K09554 CDC37; cell division cycle protein 37
D 140658871 SMURF1; (RefSeq) E3 ubiquitin-protein ligase SMURF1 isoform X1 K04678 SMURF; E3 ubiquitin ligase SMURF1/2 [EC:2.3.2.26]
D 140660615 SMURF2; (RefSeq) E3 ubiquitin-protein ligase SMURF2 isoform X1 K04678 SMURF; E3 ubiquitin ligase SMURF1/2 [EC:2.3.2.26]
D 140643820 BNIP3L; (RefSeq) 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 140644802 FUNDC1; (RefSeq) FUN14 domain-containing protein 1 isoform X1 K17986 FUNDC1; FUN14 domain-containing protein 1
C Other mitophagy factors
D 140650590 PRKAA1; (RefSeq) 5'-AMP-activated protein kinase catalytic subunit alpha-1 K07198 PRKAA; 5'-AMP-activated protein kinase, catalytic alpha subunit [EC:2.7.11.31]
D 140654750 PRKAA2; (RefSeq) 5'-AMP-activated protein kinase catalytic subunit alpha-2 isoform X1 K07198 PRKAA; 5'-AMP-activated protein kinase, catalytic alpha subunit [EC:2.7.11.31]
D 140645153 microtubule-associated protein 1 light chain 3 gamma-like K10435 MAP1LC; microtubule-associated protein 1 light chain
D 140649373 MAP1LC3C; (RefSeq) microtubule-associated protein 1 light chain 3 gamma K10435 MAP1LC; microtubule-associated protein 1 light chain
D 140656596 MAP1LC3B; (RefSeq) microtubule-associated protein 1 light chain 3 beta K10435 MAP1LC; microtubule-associated protein 1 light chain
D 140659521 MAP1LC3A; (RefSeq) microtubule-associated protein 1 light chain 3 alpha K10435 MAP1LC; microtubule-associated protein 1 light chain
D 140662379 NBR1; (RefSeq) next to BRCA1 gene 1 protein isoform X1 K17987 NBR1; next to BRCA1 gene 1 protein
D 140659000 TECPR1; (RefSeq) tectonin beta-propeller repeat-containing protein 1 K17988 TECPR1; tectonin beta-propeller repeat-containing protein 1
D 140652746 ZFYVE1; (RefSeq) zinc finger FYVE domain-containing protein 1 K17603 ZFYVE1; zinc finger FYVE domain-containing protein 1
D 140662337 COASY; (RefSeq) bifunctional coenzyme A synthase K02318 COASY; phosphopantetheine adenylyltransferase / dephospho-CoA kinase [EC:2.7.7.3 2.7.1.24]
D 140655183 PLA2G6; (RefSeq) 85/88 kDa calcium-independent phospholipase A2 isoform X1 K16343 PLA2G6; calcium-independent phospholipase A2 [EC:3.1.1.4]
D 140656553 C9H19orf12; (RefSeq) protein C19orf12 homolog K23168 MPAN; protein MPAN
D 140658732 FUNDC2; (RefSeq) FUN14 domain-containing protein 2 isoform X1 K24308 FUNDC2; FUN14 domain-containing protein 2
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#[ BRITE | KEGG2 | KEGG ]
#Last updated: July 24, 2026