+D
#
Mitochondrial Biogenesis - Haliaeetus albicilla (white-tailed eagle)
%
!
AMitochondrial DNA transcription, translation, and replication factors
B Mitochondrial DNA-encoded proteins
C Mitochondrial respiratory chain complex I
C Mitochondrial respiratory chain complex III
C Cytochrome c oxidase
C F-type ATPase
B Mitochondrial transcription and translation factors
C Mitochondrial RNA polymerase
D 104318079 POLRMT; LOW QUALITY PROTEIN: DNA-directed RNA polymerase, mitochondrial K10908 POLRMT; DNA-directed RNA polymerase, mitochondrial [EC:2.7.7.6]
C Mitochondrial transcription factors
D 104311469 TFAM; transcription factor A, mitochondrial K11830 TFAM; transcription factor A, mitochondrial
D 104312114 dimethyladenosine transferase 1, mitochondrial-like isoform X1 K15266 TFB1M; dimethyladenosine transferase 1, mitochondrial [EC:2.1.1.-]
D 104311581 TFB2M; dimethyladenosine transferase 2, mitochondrial K17653 TFB2M; dimethyladenosine transferase 2, mitochondrial [EC:2.1.1.-]
C Mitochondrial translation factors
D 104311325 MTIF2; translation initiation factor IF-2, mitochondrial K02519 infB; translation initiation factor IF-2
D 104311856 MTIF3; translation initiation factor IF-3, mitochondrial K02520 infC; translation initiation factor IF-3
D 104321304 GFM1; elongation factor G, mitochondrial K02355 fusA; elongation factor G
D 104321412 GFM2; ribosome-releasing factor 2, mitochondrial isoform X1 K02355 fusA; elongation factor G
D 104324034 MTERF3; transcription termination factor 3, mitochondrial K15031 MTERF; mitochondrial transcription termination factor family protein
D 104325170 MTERF1; transcription termination factor 1, mitochondrial K15031 MTERF; mitochondrial transcription termination factor family protein
D 104310745 GUF1; translation factor GUF1, mitochondrial K21594 GUF1; translation factor GUF1, mitochondrial [EC:3.6.5.-]
C RNase MRP
D 104320237 POP1; LOW QUALITY PROTEIN: ribonucleases P/MRP protein subunit POP1 K01164 POP1; ribonuclease P/MRP protein subunit POP1 [EC:3.1.26.5]
D 104323629 POP4; ribonuclease P protein subunit p29 K03538 POP4; ribonuclease P protein subunit POP4 [EC:3.1.26.5]
D 104311992 POP5; ribonuclease P/MRP protein subunit POP5 K03537 POP5; ribonuclease P/MRP protein subunit POP5 [EC:3.1.26.5]
D 104313671 RPP30; ribonuclease P protein subunit p30 K03539 RPP1; ribonuclease P/MRP protein subunit RPP1 [EC:3.1.26.5]
D 104313079 ribonuclease P protein subunit p25-like protein K14525 RPP25; ribonucleases P/MRP protein subunit RPP25 [EC:3.1.26.5]
D 104314086 RPP38; ribonuclease P protein subunit p38 K14523 RPP38; ribonucleases P/MRP protein subunit RPP38 [EC:3.1.26.5]
D 104315113 RPP40; ribonuclease P protein subunit p40 K14530 RPP40; ribonucleases P/MRP protein subunit RPP40 [EC:3.1.26.5]
C Mitochondrial ribonuclease P
D 104312957 TRMT10C; mitochondrial ribonuclease P protein 1 K17654 TRMT10C; mitochondrial ribonuclease P protein 1 [EC:2.1.1.-]
D 104314222 KIAA0391; mitochondrial ribonuclease P protein 3 isoform X1 K17655 MRPP3; mitochondrial ribonuclease P protein 3
C Other mitochondrial DNA transcription and translation factors
D 104311026 PTCD1; pentatricopeptide repeat-containing protein 1, mitochondrial K17710 PTCD1; pentatricopeptide repeat domain-containing protein 1
D 104310591 PTCD2; pentatricopeptide repeat-containing protein 2, mitochondrial K17711 PTCD2; pentatricopeptide repeat domain-containing protein 2
D 104323723 PTCD3; LOW QUALITY PROTEIN: pentatricopeptide repeat domain-containing protein 3, mitochondrial K17659 PTCD3; pentatricopeptide repeat domain-containing protein 3
D 104318758 DHX30; LOW QUALITY PROTEIN: putative ATP-dependent RNA helicase DHX30 K13185 DHX30; ATP-dependent RNA helicase DHX30 [EC:5.6.2.6]
D 104314735 ubiquinol-cytochrome-c reductase complex assembly factor 1-like K17662 CBP3; cytochrome b pre-mRNA-processing protein 3
D 104318108 LACE1; lactation elevated protein 1 K18798 AFG1; peroxisome-assembly ATPase [EC:3.6.4.7]
D 104312270 XRCC6BP1; mitochondrial inner membrane protease ATP23 homolog K18156 ATP23; mitochondrial inner membrane protease ATP23 [EC:3.4.24.-]
D 104319947 SUPV3L1; LOW QUALITY PROTEIN: ATP-dependent RNA helicase SUPV3L1, mitochondrial K17675 SUPV3L1; ATP-dependent RNA helicase SUPV3L1/SUV3 [EC:5.6.2.6]
D 104314748 LACTB2; beta-lactamase-like protein 2 K16639 LACTB2; endoribonuclease LACTB2 [EC:3.1.27.-]
D 104314269 SLC25A31; LOW QUALITY PROTEIN: ADP/ATP translocase 4 K05863 SLC25A4S; solute carrier family 25 (mitochondrial adenine nucleotide translocator), member 4/5/6/31
D 104325314 ADP/ATP translocase 3 K05863 SLC25A4S; solute carrier family 25 (mitochondrial adenine nucleotide translocator), member 4/5/6/31
D 104320174 mitoferrin-2 K15113 SLC25A28_37; solute carrier family 25 (mitochondrial iron transporter), member 28/37
D 104325880 mitoferrin-1-like K15113 SLC25A28_37; solute carrier family 25 (mitochondrial iron transporter), member 28/37
D 104314439 MALSU1; mitochondrial assembly of ribosomal large subunit protein 1 K25803 MALSU1; mitochondrial assembly of ribosomal large subunit protein 1
D 104324142 ERAL1; LOW QUALITY PROTEIN: GTPase Era, mitochondrial K03595 era; GTPase
D 104310541 YARS; tyrosine--tRNA ligase, cytoplasmic K01866 YARS; tyrosyl-tRNA synthetase [EC:6.1.1.1]
D 104310466 LARS; leucine--tRNA ligase, cytoplasmic K01869 LARS; leucyl-tRNA synthetase [EC:6.1.1.4]
D 104320118 LARS2; probable leucine--tRNA ligase, mitochondrial K01869 LARS; leucyl-tRNA synthetase [EC:6.1.1.4]
D 104313653 DARS2; aspartate--tRNA ligase, mitochondrial K01876 DARS2; aspartyl-tRNA synthetase [EC:6.1.1.12]
D 104324297 GARS; glycine--tRNA ligase K01880 GARS; glycyl-tRNA synthetase [EC:6.1.1.14]
D 104322625 RARS2; probable arginine--tRNA ligase, mitochondrial K01887 RARS; arginyl-tRNA synthetase [EC:6.1.1.19]
D 104323023 RARS; arginine--tRNA ligase, cytoplasmic K01887 RARS; arginyl-tRNA synthetase [EC:6.1.1.19]
D 104318269 QRSL1; glutamyl-tRNA(Gln) amidotransferase subunit A, mitochondrial K02433 gatA; aspartyl-tRNA(Asn)/glutamyl-tRNA(Gln) amidotransferase subunit A [EC:6.3.5.6 6.3.5.7]
D 104314615 glutamyl-tRNA(Gln) amidotransferase subunit B, mitochondrial-like K02434 gatB; aspartyl-tRNA(Asn)/glutamyl-tRNA(Gln) amidotransferase subunit B [EC:6.3.5.6 6.3.5.7]
D 104312465 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 104316912 MTPAP; poly(A) RNA polymerase, mitochondrial K18060 MTPAP; poly(A) RNA polymerase, mitochondrial [EC:2.7.7.19]
D 104321823 PDE12; LOW QUALITY PROTEIN: 2',5'-phosphodiesterase 12 K19612 PDE12; 2',5'-phosphodiesterase [EC:3.1.13.4 3.1.4.-]
D 104322218 NSUN3; putative methyltransferase NSUN3 K21969 NSUN3; tRNA (cytosine34-C5)-methyltransferase [EC:2.1.1.-]
D 104321449 NSUN4; 5-methylcytosine rRNA methyltransferase NSUN4 K21970 NSUN4; 5-methylcytosine rRNA methyltransferase NSUN4 [EC:2.1.1.-]
D 104320802 RNA pseudouridylate synthase domain-containing protein 4-like K22538 RPUSD4; mitochondrial RNA pseudouridine synthase RPUSD4 [EC:5.4.99.-]
D 104318999 WBSCR16; Williams-Beuren syndrome chromosomal region 16 protein isoform X1 K23495 RCC1L; RCC1-like G exchanging factor-like protein
D 104311215 TRUB2; probable tRNA pseudouridine synthase 2 K23497 TRUB2; mitochondrial mRNA pseudouridine synthase TRUB2 [EC:5.4.99.-]
D 104310869 FASTKD1; FAST kinase domain-containing protein 1 K27754 FASTKD1; FAST kinase domain-containing protein 1
D 104323680 FASTKD2; FAST kinase domain-containing protein 2 K18190 FASTKD2; FAST kinase domain-containing protein 2
D 104319858 FASTKD3; FAST kinase domain-containing protein 3 K27755 FASTKD3; FAST kinase domain-containing protein 3
D 104312795 TBRG4; LOW QUALITY PROTEIN: protein TBRG4 K27756 TBRG4; FAST kinase domain-containing protein 4
D 104313803 FASTKD5; FAST kinase domain-containing protein 5 isoform X1 K27757 FASTKD5; FAST kinase domain-containing protein 5
D 104312750 probable peptide chain release factor C12orf65 homolog, mitochondrial K23498 COXPD7; peptide chain release factor
D 104314213 RMND1; required for meiotic nuclear division protein 1 homolog K23499 RMND1; required for meiotic nuclear division protein 1
D 104325353 GRSF1; G-rich sequence factor 1 K24984 GRSF1; G-rich sequence factor 1
D 104321664 METTL15; probable methyltransferase-like protein 15 K25876 METTL15; 12S rRNA N4-methylcytidine (m4C) methyltransferase [EC:2.1.1.-]
D 104320954 uncharacterized protein C6orf203 homolog K26186 MTRES1; mitochondrial transcription rescue factor 1
B Mitochondrial DNA replication factors
C Mitochondrial DNA polymerase
D 104323941 POLG2; DNA polymerase subunit gamma-2, mitochondrial K02333 POLG2; DNA polymerase gamma 2
D 104316492 PRIMPOL; DNA-directed primase/polymerase protein K22761 PRIMPOL; DNA-directed primase/polymerase protein [EC:2.7.7.102 2.7.7.7]
C Mitochondrial DNA maintenance factors
D 104315341 MGME1; mitochondrial genome maintenance exonuclease 1 isoform X1 K19465 MGME1; mitochondrial genome maintenance exonuclease 1 [EC:3.1.-.-]
D 104322554 EXOG; nuclease EXOG, mitochondrial K15050 EXOG; nuclease EXOG, mitochondrial
D 104323800 LOW QUALITY PROTEIN: twinkle protein, mitochondrial K17680 PEO1; twinkle protein [EC:5.6.2.3]
D 104324766 PIF1; ATP-dependent DNA helicase PIF1 K15255 PIF1; ATP-dependent DNA helicase PIF1 [EC:5.6.2.3]
C Other Mitochondrial DNA replication factors
D 104312553 SSBP1; single-stranded DNA-binding protein, mitochondrial isoform X1 K03111 ssb; single-strand DNA-binding protein
D 104325362 ATAD3A; ATPase family AAA domain-containing protein 3A isoform X1 K17681 ATAD3A_B; ATPase family AAA domain-containing protein 3A/B
D 104313839 ubiquinol-cytochrome-c reductase complex assembly factor 2 K17682 MNF1; mitochondrial nucleoid factor 1
#
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 104311531 DNAJA2; dnaJ homolog subfamily A member 2 K09503 DNAJA2; DnaJ homolog subfamily A member 2
D 104323489 TOMM34; mitochondrial import receptor subunit TOM34 K17766 TOM34; mitochondrial import receptor subunit TOM34
D 104311511 RHOT1; mitochondrial Rho GTPase 1 isoform X1 K07870 RHOT1; mitochondrial Rho GTPase 1 [EC:3.6.5.-]
D 104319819 HSPA6; heat shock 70 kDa protein 6 K03283 HSPA1_6_8; heat shock 70kDa protein 1/6/8
D 104316580 aryl-hydrocarbon-interacting protein-like 1 K17767 AIP; AH receptor-interacting protein
D 104326028 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 104319835 TOMM20; mitochondrial import receptor subunit TOM20 homolog K17770 TOM20; mitochondrial import receptor subunit TOM20
D 104323001 TOMM70A; mitochondrial import receptor subunit TOM70 K17768 TOM70; mitochondrial import receptor subunit TOM70
C TOB/SAM complex
D 104311297 SAMM50; sorting and assembly machinery component 50 homolog isoform X1 K07277 SAM50; outer membrane protein insertion porin family
C Porin
D 104317395 VDAC1; voltage-dependent anion-selective channel protein 1 K05862 VDAC1; voltage-dependent anion channel protein 1
D 104315983 VDAC2; voltage-dependent anion-selective channel protein 2 K15040 VDAC2; voltage-dependent anion channel protein 2
D 104312360 VDAC3; voltage-dependent anion-selective channel protein 3 isoform X1 K15041 VDAC3; voltage-dependent anion channel protein 3
C ERMES complex
C Other outer membrane factor
D 104313170 MTX3; metaxin-3 K17776 MTX; metaxin
D 104315968 MTX1; LOW QUALITY PROTEIN: metaxin-1 K17776 MTX; metaxin
D 104316453 MTX2; metaxin-2 K17776 MTX; metaxin
D 104310643 MYO19; unconventional myosin-XIX K25838 MYO19; myosin XIX
D 104314518 LOW QUALITY PROTEIN: 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 104314551 TIMM9; mitochondrial import inner membrane translocase subunit Tim9 K17777 TIM9; mitochondrial import inner membrane translocase subunit TIM9
C TIM8/13 complex
C Mitochondrial intermembrane space assembly (MIA) complex
D 104312431 CHCHD4; mitochondrial intermembrane space import and assembly protein 40 K17782 MIA40; mitochondrial intermembrane space import and assembly protein 40
B Inner mambrane
C MICOS complex
D 104312243 MINOS1; mitochondrial inner membrane organizing system protein 1 K17784 MICOS10; MICOS complex subunit MIC10
D 104323721 IMMT; mitochondrial inner membrane protein isoform X1 K17785 IMMT; MICOS complex subunit MIC60
D 104312906 protein QIL1 isoform X1 K24624 MICOS13; MICOS complex subunit MIC13
D 104316330 CHCHD3; coiled-coil-helix-coiled-coil-helix domain-containing protein 3, mitochondrial K17563 CHCHD3; MICOS complex subunit MIC19
D 104323801 CHCHD6; coiled-coil-helix-coiled-coil-helix domain-containing protein 6, mitochondrial K17564 CHCHD6; MICOS complex subunit MIC25
D 104312733 APOO; apolipoprotein O K24625 APOO; MICOS complex subunit MIC26
D 104311662 APOOL; apolipoprotein O-like K24626 APOOL; MICOS complex subunit MIC27
C MICOS complex (yeast)
D 104312243 MINOS1; mitochondrial inner membrane organizing system protein 1 K17784 MICOS10; MICOS complex subunit MIC10
D 104323721 IMMT; mitochondrial inner membrane protein isoform X1 K17785 IMMT; MICOS complex subunit MIC60
C TIM22 complex
D 104312181 TIMM22; mitochondrial import inner membrane translocase subunit Tim22 K17790 TIM22; mitochondrial import inner membrane translocase subunit TIM22
C TIM23 complex
D 104321128 TIMM23; mitochondrial import inner membrane translocase subunit Tim23 K17794 TIM23; mitochondrial import inner membrane translocase subunit TIM23
D 104319409 TIMM17A; mitochondrial import inner membrane translocase subunit Tim17-A K17795 TIM17; mitochondrial import inner membrane translocase subunit TIM17
D 104311679 TIMM21; mitochondrial import inner membrane translocase subunit Tim21 K17796 TIM21; mitochondrial import inner membrane translocase subunit TIM21
C Mitochondrial calcium uniporter complex
D 104325129 MCU; calcium uniporter protein, mitochondrial K20858 MCU; calcium uniporter protein, mitochondrial
D 104322722 MICU1; calcium uptake protein 1, mitochondrial isoform X1 K22827 MICU1; calcium uptake protein 1, mitochondrial
D 104324904 MICU2; calcium uptake protein 2, mitochondrial K22828 MICU2; calcium uptake protein 2, mitochondrial
D 104322652 CCDC109B; mitochondrial calcium uniporter regulatory subunit MCUb K22829 MCUB; calcium uniporter regulatory subunit MCUb, mitochondrial
D 104315012 MCUR1; mitochondrial calcium uniporter regulator 1 K22137 MCUR1; mitochondrial calcium uniporter regulator 1
C Other inner membrane factors
D 104317605 LETM1; LETM1 and EF-hand domain-containing protein 1, mitochondrial isoform X1 K17800 LETM1; LETM1 and EF-hand domain-containing protein 1, mitochondrial
D 104317824 IMMP1L; mitochondrial inner membrane protease subunit 1 K09647 IMP1; mitochondrial inner membrane protease subunit 1 [EC:3.4.21.-]
D 104313596 IMMP2L; mitochondrial inner membrane protease subunit 2 K09648 IMP2; mitochondrial inner membrane protease subunit 2 [EC:3.4.21.-]
D 104311164 TMEM126A; transmembrane protein 126A K18157 TMEM126A; transmembrane protein 126A
D 104317646 MPC1; mitochondrial pyruvate carrier 1 K22138 MPC1; mitochondrial pyruvate carrier 1
D 104312313 MPC2; mitochondrial pyruvate carrier 2 K22139 MPC2; mitochondrial pyruvate carrier 2
D 104324644 OCIAD1; OCIA domain-containing protein 1 K28215 OCIAD1; OCIA domain-containing protein 1
B Matrix
C MMC/PAM proteins
D 104317543 TIMM44; mitochondrial import inner membrane translocase subunit TIM44 isoform X1 K17804 TIM44; mitochondrial import inner membrane translocase subunit TIM44
D 104320204 HSPA9; stress-70 protein, mitochondrial K04043 dnaK; molecular chaperone DnaK
D 104312370 GRPEL1; grpE protein homolog 1, mitochondrial K03687 GRPE; molecular chaperone GrpE
D 104313002 grpE protein homolog 2, mitochondrial-like K03687 GRPE; molecular chaperone GrpE
D 104318626 grpE protein homolog 2, mitochondrial-like K03687 GRPE; molecular chaperone GrpE
D 104321476 DNAJC19; mitochondrial import inner membrane translocase subunit TIM14 K09539 DNAJC19; DnaJ homolog subfamily C member 19
C Other matrix factors
D 104311065 PMPCA; mitochondrial-processing peptidase subunit alpha K01412 PMPCA; mitochondrial-processing peptidase subunit alpha [EC:3.4.24.64]
D 104315331 PMPCB; mitochondrial-processing peptidase subunit beta K17732 PMPCB; mitochondrial-processing peptidase subunit beta [EC:3.4.24.64]
D 104311653 HSPD1; 60 kDa heat shock protein, mitochondrial isoform X1 K04077 groEL; chaperonin GroEL [EC:5.6.1.7]
D 104312486 HSPE1; 10 kDa heat shock protein, mitochondrial isoform X1 K04078 groES; chaperonin GroES
D 104316473 DNAJA3; dnaJ homolog subfamily A member 3, mitochondrial K09504 DNAJA3; DnaJ homolog subfamily A member 3
D 104314066 TAMM41; mitochondrial translocator assembly and maintenance protein 41 homolog isoform X1 K17807 TAM41; mitochondrial translocator assembly and maintenance protein 41
D 104314071 DNLZ; DNL-type zinc finger protein K17808 ZIM17; mitochondrial protein import protein ZIM17
D 104311062 POLDIP2; polymerase delta-interacting protein 2 K17809 POLDIP2; polymerase delta-interacting protein 2
#
AMitochondrial quality control factors
B Regulator of mitochondrial biogenesis
C Ubiquitas transcription factors
D 104325151 CREB1; cyclic AMP-responsive element-binding protein 1 K05870 CREB1; cyclic AMP-responsive element-binding protein 1
D 104321304 GFM1; elongation factor G, mitochondrial K02355 fusA; elongation factor G
D 104321412 GFM2; ribosome-releasing factor 2, mitochondrial isoform X1 K02355 fusA; elongation factor G
D 104320978 SP1; transcription factor Sp1 K04684 SP1; transcription factor Sp1
D 104316367 GABPB2; GA-binding protein subunit beta-2 isoform X1 K09454 GABPB; GA-binding protein transcription factor, beta
D 104320295 GABPB1; GA-binding protein subunit beta-1 isoform X1 K09454 GABPB; GA-binding protein transcription factor, beta
D 104311469 TFAM; transcription factor A, mitochondrial K11830 TFAM; transcription factor A, mitochondrial
C Nuclear respiratory factors
D 104314465 nuclear respiratory factor 1-like K11831 NRF1; nuclear respiratory factor 1
D 104319839 NFE2L2; nuclear factor erythroid 2-related factor 2 K05638 NFE2L2; nuclear factor erythroid 2-related factor 2
C Coactivators
D 104310684 PPARGC1A; peroxisome proliferator-activated receptor gamma coactivator 1-alpha K07202 PPARGC1A; alpha peroxisome proliferator-activated receptor gamma coactivator 1-alpha
D 104325823 CRTC1; CREB-regulated transcription coactivator 1 K15309 CRTC1; CREB-regulated transcription coactivator 1
D 104321861 LOW QUALITY PROTEIN: CREB-binding protein-like K04498 EP300; E1A/CREB-binding protein [EC:2.3.1.48]
C Proteins involved in Fe-S cluster biogenesis
D 104314930 NFS1; LOW QUALITY PROTEIN: cysteine desulfurase, mitochondrial K04487 iscS; cysteine desulfurase [EC:2.8.1.7]
D 104323771 ISCU; iron-sulfur cluster assembly enzyme ISCU, mitochondrial K22068 ISCU; iron-sulfur cluster assembly enzyme ISCU, mitochondrial
D 104312639 FXN; frataxin, mitochondrial K19054 FXN; frataxin [EC:1.16.3.1]
D 104325805 FDX1; adrenodoxin, mitochondrial isoform X1 K22070 FDX1; ferredoxin-1, mitochondrial
D 104323946 adrenodoxin-like K22071 FDX2; ferredoxin-2, mitochondrial
D 104323368 FDXR; NADPH:adrenodoxin oxidoreductase, mitochondrial K18914 FDXR; adrenodoxin-NADP+ reductase [EC:1.18.1.6]
D 104320204 HSPA9; stress-70 protein, mitochondrial K04043 dnaK; molecular chaperone DnaK
D 104313191 HSCB; iron-sulfur cluster co-chaperone protein HscB, mitochondrial K04082 hscB; molecular chaperone HscB
D 104316556 SIL1; nucleotide exchange factor SIL1 K14001 SIL1; nucleotide exchange factor SIL1
D 104315096 ISCA1; iron-sulfur cluster assembly 1 homolog, mitochondrial K22063 ISCA1; iron-sulfur cluster assembly 1
D 104324012 ISCA2; iron-sulfur cluster assembly 2 homolog, mitochondrial K22072 ISCA2; iron-sulfur cluster assembly 2
D 104312576 IBA57; putative transferase CAF17, mitochondrial K22073 IBA57; transferase CAF17, mitochondrial [EC:2.1.-.-]
D 104312508 NFU1; NFU1 iron-sulfur cluster scaffold homolog, mitochondrial isoform X1 K22074 NFU1; NFU1 iron-sulfur cluster scaffold homolog, mitochondrial
D 104321593 SFXN1; sideroflexin-1 isoform X1 K23500 SFXN1_3; sideroflexin-1/3
D 104310914 SFXN4; sideroflexin-4 K23502 SFXN4; sideroflexin-4
D 104321342 SFXN5; sideroflexin-5 isoform X1 K23503 SFXN5; sideroflexin-5
C Other regulator of mitochondrial biogenesis
D 104311793 carnitine O-palmitoyltransferase 1, liver isoform-like isoform X1 K08765 CPT1A; carnitine O-palmitoyltransferase 1, liver isoform [EC:2.3.1.21]
D 104325563 DHTKD1; probable 2-oxoglutarate dehydrogenase E1 component DHKTD1, mitochondrial K15791 DHKTD1; 2-oxoadipate dehydrogenase E1 component [EC:1.2.4.-]
D 104323217 FBXL4; F-box/LRR-repeat protein 4 isoform X1 K10270 FBXL4; F-box and leucine-rich repeat protein 4
D 104313600 HCFC2; host cell factor 2 isoform X1 K27390 HCFC2; host cell factor 2
D 104320272 HTT; huntingtin K04533 HD; huntingtin
D 104314813 steroid hormone receptor ERR2-like K08553 NR3B2; estrogen-related receptor beta
D 104315933 ESRRG; estrogen-related receptor gamma isoform X1 K08554 NR3B3; estrogen-related receptor gamma
D 104317368 NRIP1; nuclear receptor-interacting protein 1 K17965 NRIP1; nuclear receptor-interacting protein 1
D 104316671 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 104325064 PRKAA1; 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 104310957 LONP1; lon protease homolog, mitochondrial K08675 PRSS15; ATP-dependent Lon protease [EC:3.4.21.53]
D 104316408 LOW QUALITY PROTEIN: NAD-dependent protein deacetylase sirtuin-1-like K11411 SIRT1; NAD-dependent protein deacetylase sirtuin 1 [EC:2.3.1.286]
D 104323783 SERAC1; protein SERAC1 isoform X1 K23504 SERAC1; protein SERAC1
D 104319427 PGC-1 and ERR-induced regulator in muscle protein 1-like K26738 PERM1; PGC-1 and ERR-induced regulator in muscle protein 1
D 104317219 ES1 protein homolog, mitochondrial-like K27835 GATD3; glutamine amidotransferase-like class 1 domain-containing protein 3, mitochondrial
D 104325261 ES1 protein homolog, mitochondrial-like isoform X1 K27835 GATD3; glutamine amidotransferase-like class 1 domain-containing protein 3, mitochondrial
D 104315771 TANGO2; transport and Golgi organization protein 2 homolog isoform X1 K28098 TANGO2; transport and Golgi organization protein 2
D 104312355 UPF0565 protein C2orf69 homolog K28271 C2ORF69; mitochondrial protein C2orf69
D 104316470 FAM214A; protein FAM214A isoform X1 K28528 ATOS; protein Atossa
D 104324496 FAM214B; LOW QUALITY PROTEIN: protein FAM214B K28528 ATOS; protein Atossa
D 104319963 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 104315888 NDUFAF1; complex I intermediate-associated protein 30, mitochondrial K18159 NDUFAF1; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 1
D 104313303 NDUFAF2; mimitin, mitochondrial K18160 NDUFAF2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 2
D 104312805 NDUFAF4; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 4 K18161 NDUFAF4; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 4
D 104318076 NDUFAF5; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 5 K18162 NDUFAF5; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 5 [EC:2.1.1.-]
D 104310646 NDUFAF6; NADH dehydrogenase (ubiquinone) complex I, assembly factor 6 K18163 NDUFAF6; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 6
D 104316884 NDUFAF7; NADH dehydrogenase [ubiquinone] complex I, assembly factor 7 K18164 NDUFAF7; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 7
D 104315495 ACAD9; acyl-CoA dehydrogenase family member 9, mitochondrial K15980 ACAD9; acyl-CoA dehydrogenase family member 9 [EC:1.3.99.-]
D 104321697 NUBPL; LOW QUALITY PROTEIN: iron-sulfur protein NUBPL K03593 mrp; ATP-binding protein involved in chromosome partitioning
D 104319911 RTN4IP1; reticulon-4-interacting protein 1, mitochondrial K23164 RTN4IP1; reticulon-4-interacting protein 1, mitochondrial
C Complex-II assembly factors
D 104317749 SDHAF2; succinate dehydrogenase assembly factor 2, mitochondrial K18168 SDHAF2; succinate dehydrogenase assembly factor 2
C Complex-III assembly factors
D 104323451 mitochondrial chaperone BCS1 K08900 BCS1; mitochondrial chaperone BCS1
D 104313503 TTC19; tetratricopeptide repeat protein 19, mitochondrial K18169 TTC19; tetratricopeptide repeat protein 19, mitochondrial
D 104321847 LYRM7; LOW QUALITY PROTEIN: complex III assembly factor LYRM7 K18170 LYRM7; complex III assembly factor LYRM7
D 104314735 ubiquinol-cytochrome-c reductase complex assembly factor 1-like K17662 CBP3; cytochrome b pre-mRNA-processing protein 3
D 104313839 ubiquinol-cytochrome-c reductase complex assembly factor 2 K17682 MNF1; mitochondrial nucleoid factor 1
D 104318148 uncharacterized protein C12orf73 homolog K27341 UQCC6; ubiquinol-cytochrome c reductase complex assembly factor 6
D 104318830 uncharacterized protein C7orf73 homolog K27319 STMP1; short transmembrane mitochondrial protein 1
C Complex-IV assembly factors
D 104315078 CMC1; COX assembly mitochondrial protein homolog K18171 CMC1; COX assembly mitochondrial protein 1
D 104313227 CMC2; COX assembly mitochondrial protein 2 homolog K18172 CMC2; COX assembly mitochondrial protein 2
D 104319590 cytochrome c oxidase assembly factor 1 homolog K18173 COA1; cytochrome c oxidase assembly factor 1
D 104315013 cytochrome c oxidase assembly factor 5 K18178 COA5; cytochrome c oxidase assembly factor 5
D 104316179 cytochrome c oxidase assembly factor 7 K18180 COA7; cytochrome c oxidase assembly factor 7
D 104316254 protoheme IX farnesyltransferase, mitochondrial-like K02257 COX10; heme o synthase [EC:2.5.1.141]
D 104313350 cytochrome c oxidase assembly protein COX11, mitochondrial K02258 COX11; cytochrome c oxidase assembly protein subunit 11
D 104311602 cytochrome c oxidase assembly protein COX15 homolog K02259 COX15; heme a synthase [EC:1.17.99.9]
D 104318565 cytochrome c oxidase assembly protein COX16 homolog, mitochondrial K18182 COX16; cytochrome c oxidase assembly protein subunit 16
D 104316860 cytochrome c oxidase copper chaperone K02260 COX17; cytochrome c oxidase assembly protein subunit 17
D 104315258 mitochondrial inner membrane protein COX18 K17797 COX18; mitochondrial inner membrane protein COX18
D 104325441 cytochrome c oxidase protein 20 homolog K18184 COX20; cytochrome c oxidase assembly protein subunit 20
D 104320608 PET117; protein PET117 homolog, mitochondrial K18188 PET117; protein PET117
D 104312422 SURF1; surfeit locus protein 1 K14998 SURF1; surfeit locus 1 family protein
D 104317676 protein SCO1 homolog, mitochondrial K07152 SCO1; protein SCO1
D 104317606 LRPPRC; leucine-rich PPR motif-containing protein, mitochondrial K17964 LRPPRC; leucine-rich PPR motif-containing protein, mitochondrial
D 104321426 APOPT1; LOW QUALITY PROTEIN: apoptogenic protein 1, mitochondrial K23506 APOPT1; apoptogenic protein 1
D 104318830 uncharacterized protein C7orf73 homolog K27319 STMP1; short transmembrane mitochondrial protein 1
D 104320589 HIGD1A; HIG1 domain family member 1A, mitochondrial isoform X1 K28216 HIGD1A_C; HIG1 domain family member 1A/C, mitochondrial
C Complex-V assembly factors
D 104316016 ATPAF1; ATP synthase mitochondrial F1 complex assembly factor 1 isoform X1 K07555 ATPeAF1; ATP synthase mitochondrial F1 complex assembly factor 1
D 104321156 ATPAF2; ATP synthase mitochondrial F1 complex assembly factor 2 K07556 ATPeAF2; ATP synthase mitochondrial F1 complex assembly factor 2
D 104312376 TMEM70; transmembrane protein 70, mitochondrial K17966 TMEM70; transmembrane protein 70, mitochondrial
D 104319314 6.8 kDa mitochondrial proteolipid K18193 MP68; 6.8 kDa mitochondrial proteolipid
D 104312533 USMG5; up-regulated during skeletal muscle growth protein 5 K18194 USMG5; up-regulated during skeletal muscle growth protein 5
B Mitochondrial dynamics
C Fission and Fusion factors
D 104322953 MFN1; mitofusin-1 isoform X1 K21356 MFN1; mitofusin 1 [EC:3.6.5.-]
D 104321845 MFN2; mitofusin-2 isoform X1 K06030 MFN2; mitofusin 2 [EC:3.6.5.-]
D 104321837 OPA1; dynamin-like 120 kDa protein, mitochondrial K17079 OPA1; optic atrophy protein 1 [EC:3.6.5.5]
D 104315219 PARL; presenilins-associated rhomboid-like protein, mitochondrial K09650 PARL; rhomboid-like protein [EC:3.4.21.105]
D 104312285 BAK1; bcl-2 homologous antagonist/killer isoform X1 K14021 BAK; Bcl-2 homologous antagonist/killer
D 104320019 DNM1L; dynamin-1-like protein K17065 DNM1L; dynamin 1-like protein [EC:3.6.5.5]
D 104312461 SUMO2; small ubiquitin-related modifier 2 K12160 SUMO; small ubiquitin-related modifier
D 104314400 SUMO1; small ubiquitin-related modifier 1 K12160 SUMO; small ubiquitin-related modifier
D 104324891 small ubiquitin-related modifier 2-A-like K12160 SUMO; small ubiquitin-related modifier
D 104311449 endophilin-B1-like K11248 SH3GLB1; endophilin-B1
D 104313943 MTCH1; mitochondrial carrier homolog 1 K17885 MTCH; mitochondrial carrier
D 104311965 MFF; mitochondrial fission factor isoform X1 K22076 MFF; mitochondrial fission factor
D 104316070 GDAP1; ganglioside-induced differentiation-associated protein 1 K22077 GDAP1; ganglioside-induced differentiation-associated protein 1
D 104323304 VAT1; synaptic vesicle membrane protein VAT-1 homolog K23167 VAT1; synaptic vesicle membrane protein VAT-1
D 104312223 MIEF1; mitochondrial dynamics protein MID51 K23507 MIEF1_2; mitochondrial dynamics protein MID51/49
D 104319158 LOW QUALITY PROTEIN: mitochondrial dynamics protein MID49-like K23507 MIEF1_2; mitochondrial dynamics protein MID51/49
D 104322903 FAM73B; protein FAM73B K27289 MIGA; mitoguardin
D 104324817 FAM73A; protein FAM73A K27289 MIGA; mitoguardin
C Actin cytoskeleton organization
D 104312470 actin, cytoplasmic type 5 K05692 ACTB_G1; actin beta/gamma 1
D 104324128 actin, cytoplasmic 2-like K05692 ACTB_G1; actin beta/gamma 1
D 104324189 LOW QUALITY PROTEIN: actin, muscle-like K05692 ACTB_G1; actin beta/gamma 1
D 104323910 N-alpha-acetyltransferase 20-like K17972 NAA20; N-terminal acetyltransferase B complex catalytic subunit [EC:2.3.1.254]
D 104310836 NAA25; N-alpha-acetyltransferase 25, NatB auxiliary subunit K17973 NAA25; N-terminal acetyltransferase B complex non-catalytic subunit
C Mitochondrial movement
D 104315666 PHB; prohibitin K17080 PHB1; prohibitin 1
D 104324394 PHB2; prohibitin-2 K17081 PHB2; prohibitin 2
D 104315425 KTN1; LOW QUALITY PROTEIN: kinectin K17975 KTN1; kinectin
C Retention of mitochondria
D 104320019 DNM1L; dynamin-1-like protein K17065 DNM1L; dynamin 1-like protein [EC:3.6.5.5]
D 104312841 LOW QUALITY PROTEIN: unconventional myosin-Va-like K10357 MYO5; myosin V
D 104323098 MYO5B; unconventional myosin-Vb K10357 MYO5; myosin V
D 104323401 unconventional myosin-Vc-like K10357 MYO5; myosin V
D 104319745 LOW QUALITY PROTEIN: E3 ubiquitin-protein ligase XIAP-like K04725 XIAP; E3 ubiquitin-protein ligase XIAP [EC:2.3.2.27]
D 104312210 DIABLO; diablo homolog, mitochondrial K10522 DIABLO; diablo
C Morphogenesis factors
D 104315470 sorting nexin-25-like K17887 SNX25; sorting nexin-25
D 104311511 RHOT1; mitochondrial Rho GTPase 1 isoform X1 K07870 RHOT1; mitochondrial Rho GTPase 1 [EC:3.6.5.-]
D 104323721 IMMT; mitochondrial inner membrane protein isoform X1 K17785 IMMT; MICOS complex subunit MIC60
D 104314026 DAP3; 28S ribosomal protein S29, mitochondrial K17408 DAP3; small subunit ribosomal protein S29
D 104318612 NEDD4; E3 ubiquitin-protein ligase NEDD4 isoform X1 K10591 NEDD4; E3 ubiquitin-protein ligase NEDD4 [EC:2.3.2.26]
D 104311276 oxidation resistance protein 1-like K25437 OXR1; oxidation resistance protein 1
D 104324464 OXR1; oxidation resistance protein 1 K25437 OXR1; oxidation resistance protein 1
D 104325533 MSTO1; protein misato homolog 1 K27320 MSTO1; protein misato
D 104323076 PRELID1; PRELI domain-containing protein 1, mitochondrial K27964 PRELID1; PRELI domain-containing protein 1, mitochondrial
D 104321839 PRELID2; PRELI domain-containing protein 2 K27965 PRELID2; PRELI domain-containing protein 2 mitochondrial
D 104311788 LOW QUALITY PROTEIN: protein slowmo homolog 2-like K27966 PRELID3; PRELI domain-containing protein 3, mitochondrial
D 104312100 LOW QUALITY PROTEIN: protein slowmo homolog 1-like K27966 PRELID3; PRELI domain-containing protein 3, mitochondrial
B Mitophagy factors
C Autophagy-related proteins
D 104313875 ATG2B; LOW QUALITY PROTEIN: autophagy-related protein 2 homolog B K17906 ATG2; autophagy-related protein 2
D 104322441 ATG3; ubiquitin-like-conjugating enzyme ATG3 isoform X1 K08343 ATG3; ubiquitin-like-conjugating enzyme ATG3
D 104315393 ATG4B; cysteine protease ATG4B K08342 ATG4; cysteine protease ATG4 [EC:3.4.22.-]
D 104319480 ATG4C; cysteine protease ATG4C isoform X1 K08342 ATG4; cysteine protease ATG4 [EC:3.4.22.-]
D 104323591 ATG4A; cysteine protease ATG4A K08342 ATG4; cysteine protease ATG4 [EC:3.4.22.-]
D 104324638 ATG5; autophagy protein 5 K08339 ATG5; autophagy-related protein 5
D 104321428 BECN1; beclin-1 K08334 BECN; beclin
D 104313128 ATG7; ubiquitin-like modifier-activating enzyme ATG7 isoform X1 K08337 ATG7; ubiquitin-like modifier-activating enzyme ATG7
D 104312284 gamma-aminobutyric acid receptor-associated protein-like 1 K08341 GABARAP; GABA(A) receptor-associated protein
D 104312765 GABARAPL2; gamma-aminobutyric acid receptor-associated protein-like 2 K08341 GABARAP; GABA(A) receptor-associated protein
D 104314262 LOW QUALITY PROTEIN: autophagy-related protein 9A-like K17907 ATG9; autophagy-related protein 9
D 104313666 ATG10; ubiquitin-like-conjugating enzyme ATG10 K17888 ATG10L; ubiquitin-like-conjugating enzyme ATG10
D 104320080 ATG14; beclin 1-associated autophagy-related key regulator K17889 ATG14L; beclin 1-associated autophagy-related key regulator
D 104310901 ATG16L1; autophagy-related protein 16-1 isoform X1 K17890 ATG16L1; autophagy-related protein 16-1
D 104323233 WIPI1; WD repeat domain phosphoinositide-interacting protein 1 K17908 WIPI1_2; autophagy-related protein 18
D 104325146 WIPI2; WD repeat domain phosphoinositide-interacting protein 2 isoform X1 K17908 WIPI1_2; autophagy-related protein 18
D 104321317 vacuolar protein 8-like K08332 VAC8; vacuolar protein 8
C Parkin-dependent mechanism factors
D 104314468 RNF41; E3 ubiquitin-protein ligase NRDP1 isoform X1 K11981 RNF41; E3 ubiquitin-protein ligase NRDP1 [EC:2.3.2.27]
D 104317055 LOW QUALITY PROTEIN: protein CLEC16A-like K19513 CLEC16A; protein CLEC16A
D 104322161 PINK1; serine/threonine-protein kinase PINK1, mitochondrial K05688 PINK1; PTEN induced putative kinase 1 [EC:2.7.11.1]
D 104315219 PARL; presenilins-associated rhomboid-like protein, mitochondrial K09650 PARL; rhomboid-like protein [EC:3.4.21.105]
D 104320159 SQSTM1; sequestosome-1 isoform X1 K14381 SQSTM1; sequestosome 1
D 104323476 TAX1BP1; tax1-binding protein 1 isoform X1 K21347 TAX1BP1; Tax1-binding protein 1
D 104311629 CALCOCO2; calcium-binding and coiled-coil domain-containing protein 2 K21348 CALCOCO2; calcium-binding and coiled-coil domain-containing protein 2
D 104319772 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 104314372 ATG13; autophagy-related protein 13 isoform X1 K08331 ATG13; autophagy-related protein 13
D 104324662 ULK1; LOW QUALITY PROTEIN: serine/threonine-protein kinase ULK1 K21357 ULK1; serine/threonine-protein kinase ULK1 [EC:2.7.11.1]
D 104322247 ULK2; serine/threonine-protein kinase ULK2 isoform X1 K08269 ULK2; serine/threonine-protein kinase ULK2 [EC:2.7.11.1]
D 104325424 RB1CC1; RB1-inducible coiled-coil protein 1 K17589 RB1CC1; RB1-inducible coiled-coil protein 1
D 104318183 HSP90AA1; heat shock protein HSP 90-alpha K04079 HSP90A; molecular chaperone HtpG
D 104324659 heat shock protein HSP 90-beta isoform X1 K04079 HSP90A; molecular chaperone HtpG
D 104323372 hsp90 co-chaperone Cdc37-like K09554 CDC37; cell division cycle protein 37
D 104313938 SMURF2; E3 ubiquitin-protein ligase SMURF2 K04678 SMURF; E3 ubiquitin ligase SMURF1/2 [EC:2.3.2.26]
D 104316073 SMURF1; E3 ubiquitin-protein ligase SMURF1 K04678 SMURF; E3 ubiquitin ligase SMURF1/2 [EC:2.3.2.26]
D 104322069 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 104313278 FUN14 domain-containing protein 1-like K17986 FUNDC1; FUN14 domain-containing protein 1
C Other mitophagy factors
D 104316671 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 104325064 PRKAA1; 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 104316750 microtubule-associated proteins 1A/1B light chain 3C-like K10435 MAP1LC; microtubule-associated protein 1 light chain
D 104318595 microtubule-associated proteins 1A/1B light chain 3C-like K10435 MAP1LC; microtubule-associated protein 1 light chain
D 104322193 MAP1LC3A; microtubule-associated proteins 1A/1B light chain 3A K10435 MAP1LC; microtubule-associated protein 1 light chain
D 104322772 MAP1LC3B; microtubule-associated proteins 1A/1B light chain 3B K10435 MAP1LC; microtubule-associated protein 1 light chain
D 104313669 NBR1; next to BRCA1 gene 1 protein K17987 NBR1; next to BRCA1 gene 1 protein
D 104310641 TECPR1; tectonin beta-propeller repeat-containing protein 1 isoform X1 K17988 TECPR1; tectonin beta-propeller repeat-containing protein 1
D 104313288 ZFYVE1; LOW QUALITY PROTEIN: zinc finger FYVE domain-containing protein 1 K17603 ZFYVE1; zinc finger FYVE domain-containing protein 1
D 104325619 PLA2G6; 85/88 kDa calcium-independent phospholipase A2 K16343 PLA2G6; calcium-independent phospholipase A2 [EC:3.1.1.4]
D 104310360 protein C19orf12 homolog K23168 MPAN; protein MPAN
D 104320665 FUN14 domain-containing protein 2-like K24308 FUNDC2; FUN14 domain-containing protein 2
!
#
#[ BRITE | KEGG2 | KEGG ]
#Last updated: September 5, 2025