Gene Full_Name MIM_number OMIM_Link Entrez_Gene_ID MT-ND1 mitochondrially encoded NADH dehydrogenase 1 [Homo sapiens (human)] 516000 http://omim.org/entry/516000 4535 MT-ND2 mitochondrially encoded NADH dehydrogenase 2 [Homo sapiens (human)] 516001 http://omim.org/entry/516001 4536 MT-ND3 mitochondrially encoded NADH dehydrogenase 3 [Homo sapiens (human)] 516002 http://omim.org/entry/516002 4537 MT-ND4 mitochondrially encoded NADH dehydrogenase 4 [Homo sapiens (human)] 516003 http://omim.org/entry/516003 4538 MT-ND4L mitochondrially encoded NADH 4L dehydrogenase [Homo sapiens (human)] 516004 http://omim.org/entry/516004 4539 MT-ND5 mitochondrially encoded NADH dehydrogenase 5 [Homo sapiens (human)] 516005 http://omim.org/entry/516005 4540 MT-ND6 mitochondrially encoded NADH dehydrogenase 6 [Homo sapiens (human)] 516006 http://omim.org/entry/516006 4541 NDUFA1 NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 1, 7.5kDa[Homo sapiens] 300078 http://omim.org/entry/300078 4694 NDUFA10 NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 10, 42kDa[Homo sapiens] 603835 http://omim.org/entry/603835 4705 NDUFA11 NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 11, 14.7kDa[Homo sapiens] 612638 http://omim.org/entry/612638 126328 NDUFA12 NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 12[Homo sapiens] 614530 http://omim.org/entry/614530 55967 NDUFA13 NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 13[Homo sapiens] 609435 http://omim.org/entry/609435 51079 NDUFA2 NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 2, 8kDa[Homo sapiens] 602137 http://omim.org/entry/602137 4695 NDUFA9 NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 9, 39kDa[Homo sapiens] 603834 http://omim.org/entry/603834 4704 NDUFB11 NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 11, 17.3kDa [Homo sapiens (human)] 300403 http://omim.org/entry/300403 54539 NDUFB3 NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 3, 12kDa[Homo sapiens] 603839 http://omim.org/entry/603839 4709 NDUFB9 NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 9, 22kDa[Homo sapiens] 601445 http://omim.org/entry/601445 4715 NDUFS1 NADH dehydrogenase (ubiquinone) Fe-S protein 1, 75kDa (NADH-coenzyme Q reductase)[Homo sapiens] 157655 http://omim.org/entry/157655 4719 NDUFS2 NADH dehydrogenase (ubiquinone) Fe-S protein 2, 49kDa (NADH-coenzyme Q reductase)[Homo sapiens] 602985 http://omim.org/entry/602985 4720 NDUFS3 NADH dehydrogenase (ubiquinone) Fe-S protein 3, 30kDa (NADH-coenzyme Q reductase)[Homo sapiens] 603846 http://omim.org/entry/603846 4722 NDUFS4 NADH dehydrogenase (ubiquinone) Fe-S protein 4, 18kDa (NADH-coenzyme Q reductase)[Homo sapiens] 602694 http://omim.org/entry/602694 4724 NDUFS6 NADH dehydrogenase (ubiquinone) Fe-S protein 6, 13kDa (NADH-coenzyme Q reductase)[Homo sapiens] 603848 http://omim.org/entry/603848 4726 NDUFS7 NADH dehydrogenase (ubiquinone) Fe-S protein 7, 20kDa (NADH-coenzyme Q reductase)[Homo sapiens] 601825 http://omim.org/entry/601825 374291 NDUFS8 NADH dehydrogenase (ubiquinone) Fe-S protein 8, 23kDa (NADH-coenzyme Q reductase)[Homo sapiens] 602141 http://omim.org/entry/602141 4728 NDUFV1 NADH dehydrogenase (ubiquinone) flavoprotein 1, 51kDa[Homo sapiens] 161015 http://omim.org/entry/161015 4723 NDUFV2 NADH dehydrogenase (ubiquinone) flavoprotein 2, 24kDa[Homo sapiens] 600532 http://omim.org/entry/600532 4729 SDHA succinate dehydrogenase complex, subunit A, flavoprotein (Fp)[Homo sapiens] 600857 http://omim.org/entry/600857 6389 SDHB succinate dehydrogenase complex, subunit B, iron sulfur (Ip)[Homo sapiens] 185470 http://omim.org/entry/185470 6390 SDHC succinate dehydrogenase complex, subunit C, integral membrane protein, 15kDa[Homo sapiens] 602413 http://omim.org/entry/602413 6391 SDHD succinate dehydrogenase complex, subunit D, integral membrane protein[Homo sapiens] 602690 http://omim.org/entry/602690 6392 CYC1 cytochrome c-1[Homo sapiens] 123980 http://omim.org/entry/123980 1537 MT-CYB mitochondrially encoded cytochrome b [Homo sapiens (human)] 516020 http://omim.org/entry/516020 4519 UQCRB ubiquinol-cytochrome c reductase binding protein[Homo sapiens] 191330 http://omim.org/entry/191330 7381 UQCRC2 ubiquinol-cytochrome c reductase core protein II[Homo sapiens] 191329 http://omim.org/entry/191329 7385 UQCRQ ubiquinol-cytochrome c reductase, complex III subunit VII, 9.5kDa[Homo sapiens] 612080 http://omim.org/entry/612080 27089 COX4I2 cytochrome c oxidase subunit IV isoform 2 (lung)[Homo sapiens] 607976 http://omim.org/entry/607976 84701 COX6A1 cytochrome c oxidase subunit VIa polypeptide 1[Homo sapiens] 602072 http://omim.org/entry/602072 1337 COX6B1 cytochrome c oxidase subunit VIb polypeptide 1 (ubiquitous)[Homo sapiens] 124089 http://omim.org/entry/124089 1340 COX7B cytochrome c oxidase subunit VIIb[Homo sapiens] 603792 http://omim.org/entry/603792 1349 COX8A cytochrome c oxidase subunit VIIIA (ubiquitous) [Homo sapiens (human)] 123870 http://omim.org/entry/123870 1351 MT-CO1 mitochondrially encoded cytochrome c oxidase I [Homo sapiens (human)] 516030 http://omim.org/entry/516030 4512 MT-CO2 mitochondrially encoded cytochrome c oxidase II [Homo sapiens (human)] 516040 http://omim.org/entry/516040 4513 MT-CO3 mitochondrially encoded cytochrome c oxidase III [Homo sapiens (human)] 516050 http://omim.org/entry/516050 4514 NDUFA4 NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 4, 9kDa[Homo sapiens] 603833 http://omim.org/entry/603833 4697 TACO1 translational activator of mitochondrially encoded cytochrome c oxidase I[Homo sapiens] 612958 http://omim.org/entry/612958 51204 ATP5A1 ATP synthase, H+ transporting, mitochondrial F1 complex, alpha subunit 1, cardiac muscle[Homo sapiens] 164360 http://omim.org/entry/164360 498 ATP5E ATP synthase, H+ transporting, mitochondrial F1 complex, epsilon subunit[Homo sapiens] 606153 http://omim.org/entry/606153 514 MT-ATP6 mitochondrially encoded ATP synthase 6 [Homo sapiens (human)] 516060 http://omim.org/entry/516060 4508 MT-ATP8 mitochondrially encoded ATP synthase 8 [Homo sapiens (human)] 516070 http://omim.org/entry/516070 4509 ACAD9 acyl-CoA dehydrogenase family, member 9[Homo sapiens] 611103 http://omim.org/entry/611103 28976 FOXRED1 FAD-dependent oxidoreductase domain containing 1[Homo sapiens] 613622 http://omim.org/entry/613622 55572 NDUFAF1 NADH dehydrogenase (ubiquinone) complex I, assembly factor 1[Homo sapiens] 606934 http://omim.org/entry/606934 51103 NDUFAF2 NADH dehydrogenase (ubiquinone) complex I, assembly factor 2[Homo sapiens] 609653 http://omim.org/entry/609653 91942 NDUFAF3 NADH dehydrogenase (ubiquinone) complex I, assembly factor 3[Homo sapiens] 612911 http://omim.org/entry/612911 25915 NDUFAF4 NADH dehydrogenase (ubiquinone) complex I, assembly factor 4[Homo sapiens] 611776 http://omim.org/entry/611776 29078 NDUFAF5 NADH dehydrogenase (ubiquinone) complex I, assembly factor 5[Homo sapiens] 612360 http://omim.org/entry/612360 79133 NDUFAF6 NADH dehydrogenase (ubiquinone) complex I, assembly factor 6[Homo sapiens] 612392 http://omim.org/entry/612392 137682 TMEM126B transmembrane protein 126B [Homo sapiens (human)] 615533 http://omim.org/entry/615533 55863 SDHAF1 succinate dehydrogenase complex assembly factor 1[Homo sapiens] 612848 http://omim.org/entry/612848 644096 SDHAF2 succinate dehydrogenase complex assembly factor 2[Homo sapiens] 613019 http://omim.org/entry/613019 54949 BCS1L BC1 (ubiquinol-cytochrome c reductase) synthesis-like[Homo sapiens] 603647 http://omim.org/entry/603647 617 LYRM7 LYR motif containing 7[Homo sapiens] 615831 http://omim.org/entry/615831 90624 TTC19 tetratricopeptide repeat domain 19[Homo sapiens] 613814 http://omim.org/entry/613814 54902 UQCC2 ubiquinol-cytochrome c reductase complex assembly factor 2[Homo sapiens] 614461 http://omim.org/entry/614461 84300 UQCC3 ubiquinol-cytochrome c reductase complex assembly factor 3[Homo sapiens] 616097 http://omim.org/entry/616097 790955 CEP89 centrosomal protein 89kDa[Homo sapiens] 615470 http://omim.org/entry/615470 84902 COA5 cytochrome c oxidase assembly factor 5[Homo sapiens] 613920 http://omim.org/entry/613920 493753 COA7 cytochrome c oxidase assembly factor 7 (putative) [Homo sapiens (human)] 615623 http://omim.org/entry/615623 65260 COX14 cytochrome c oxidase assembly homolog 14 (S. cerevisiae)[Homo sapiens] 614478 http://omim.org/entry/614478 84987 COX20 COX20 Cox2 chaperone homolog (S. cerevisiae)[Homo sapiens] 614698 http://omim.org/entry/614698 116228 FASTKD2 FAST kinase domains 2[Homo sapiens] 612322 http://omim.org/entry/612322 22868 PET100 PET100 homolog (S. cerevisiae)[Homo sapiens] 614770 http://omim.org/entry/614770 100131801 SURF1 surfeit 1[Homo sapiens] 185620 http://omim.org/entry/185620 6834 ATPAF2 ATP synthase mitochondrial F1 complex assembly factor 2[Homo sapiens] 608918 http://omim.org/entry/608918 91647 TMEM70 transmembrane protein 70[Homo sapiens] 612418 http://omim.org/entry/612418 54968 C10orf2 chromosome 10 open reading frame 2[Homo sapiens] 606075 http://omim.org/entry/606075 56652 DNA2 DNA replication helicase 2 homolog (yeast)[Homo sapiens] 601810 http://omim.org/entry/601810 1763 MGME1 mitochondrial genome maintenance exonuclease 1[Homo sapiens] 615076 http://omim.org/entry/615076 92667 POLG polymerase (DNA directed), gamma[Homo sapiens] 174763 http://omim.org/entry/174763 5428 POLG2 polymerase (DNA directed), gamma 2, accessory subunit[Homo sapiens] 604983 http://omim.org/entry/604983 11232 RNASEH1 ribonuclease H1 [Homo sapiens (human)] 604123 http://omim.org/entry/604123 246243 ABAT 4-aminobutyrate aminotransferase [Homo sapiens (human)] 137150 http://omim.org/entry/137150 18 DGUOK deoxyguanosine kinase[Homo sapiens] 601465 http://omim.org/entry/601465 1716 MPV17 MpV17 mitochondrial inner membrane protein[Homo sapiens] 137960 http://omim.org/entry/137960 4358 RRM2B ribonucleotide reductase M2 B (TP53 inducible)[Homo sapiens] 604712 http://omim.org/entry/604712 50484 SAMHD1 SAM domain and HD domain 1[Homo sapiens] 606754 http://omim.org/entry/606754 25939 SLC25A4 "solute carrier family 25 (mitochondrial carrier; adenine nucleotide translocator), member 4[Homo sapiens]" 103220 http://omim.org/entry/103220 291 SUCLA2 succinate-CoA ligase, ADP-forming, beta subunit[Homo sapiens] 603921 http://omim.org/entry/603921 8803 SUCLG1 succinate-CoA ligase, alpha subunit[Homo sapiens] 611224 http://omim.org/entry/611224 8802 TK2 thymidine kinase 2, mitochondrial[Homo sapiens] 188250 http://omim.org/entry/188250 7084 TYMP thymidine phosphorylase[Homo sapiens] 131222 http://omim.org/entry/131222 1890 ELAC2 elaC ribonuclease Z 2[Homo sapiens] 605367 http://omim.org/entry/605367 60528 GTPBP3 GTP binding protein 3 (mitochondrial)[] 608536 http://omim.org/entry/608536 84705 HSD17B10 hydroxysteroid (17-beta) dehydrogenase 10[Homo sapiens] 300256 http://omim.org/entry/300256 3028 LRPPRC leucine-rich pentatricopeptide repeat containing[Homo sapiens] 607544 http://omim.org/entry/607544 10128 MTO1 mitochondrial translation optimization 1 homolog (S. cerevisiae)[Homo sapiens] 614667 http://omim.org/entry/614667 25821 MTPAP mitochondrial poly(A) polymerase[Homo sapiens] 613669 http://omim.org/entry/613669 55149 NSUN3 NOP2/Sun RNA methyltransferase family member 3 [Homo sapiens (human)] http://omim.org/entry/ 63899 PNPT1 polyribonucleotide nucleotidyltransferase 1[Homo sapiens] 610316 http://omim.org/entry/610316 87178 PUS1 pseudouridylate synthase 1[Homo sapiens] 608109 http://omim.org/entry/608109 80324 TRIT1 tRNA isopentenyltransferase 1[Homo sapiens] http://omim.org/entry/ 54802 TRMT10C tRNA methyltransferase 10C, mitochondrial RNase P subunit [Homo sapiens (human)] 615423 http://omim.org/entry/615423 54931 TRMT5 tRNA methyltransferase 5 [Homo sapiens (human)] 611023 http://omim.org/entry/611023 57570 TRMU tRNA 5-methylaminomethyl-2-thiouridylate methyltransferase[Homo sapiens] 610230 http://omim.org/entry/610230 55687 TRNT1 tRNA nucleotidyl transferase, CCA-adding, 1[Homo sapiens] 612907 http://omim.org/entry/612907 51095 C12orf65 chromosome 12 open reading frame 65[Homo sapiens] 613541 http://omim.org/entry/613541 91574 GFM1 G elongation factor, mitochondrial 1[Homo sapiens] 606639 http://omim.org/entry/606639 85476 GFM2 G elongation factor, mitochondrial 2 [Homo sapiens (human)] 606544 http://omim.org/entry/606544 84340 RMND1 required for meiotic nuclear division 1 homolog (S. cerevisiae)[Homo sapiens] 614917 http://omim.org/entry/614917 55005 TSFM Ts translation elongation factor, mitochondrial[Homo sapiens] 604723 http://omim.org/entry/604723 10102 TUFM Tu translation elongation factor, mitochondrial[Homo sapiens] 602389 http://omim.org/entry/602389 7284 MRPL12 mitochondrial ribosomal protein L12[Homo sapiens] 602375 http://omim.org/entry/602375 6182 MRPL3 mitochondrial ribosomal protein L3[Homo sapiens] 607118 http://omim.org/entry/607118 11222 MRPL44 mitochondrial ribosomal protein L44[Homo sapiens] 611849 http://omim.org/entry/611849 65080 MRPS16 mitochondrial ribosomal protein S16[Homo sapiens] 609204 http://omim.org/entry/609204 51021 MRPS22 mitochondrial ribosomal protein S22[Homo sapiens] 605810 http://omim.org/entry/605810 56945 MRPS23 mitochondrial ribosomal protein S23 [Homo sapiens (human)] 611985 http://omim.org/entry/611985 51649 MRPS7 mitochondrial ribosomal protein S7[Homo sapiens] 611974 http://omim.org/entry/611974 51081 MT-RNR1 mitochondrially encoded 12S RNA [Homo sapiens (human)] 561000 http://omim.org/entry/561000 4549 MT-TA mitochondrially encoded tRNA alanine [Homo sapiens (human)] 590000 http://omim.org/entry/590000 4553 MT-TC mitochondrially encoded tRNA cysteine [Homo sapiens (human)] 590020 http://omim.org/entry/590020 4511 MT-TD mitochondrially encoded tRNA aspartic acid [Homo sapiens (human)] 590015 http://omim.org/entry/590015 4555 MT-TE mitochondrially encoded tRNA glutamic acid [Homo sapiens (human)] 590025 http://omim.org/entry/590025 4556 MT-TF mitochondrially encoded tRNA phenylalanine [Homo sapiens (human)] 590070 http://omim.org/entry/590070 4558 MT-TG mitochondrially encoded tRNA glycine [Homo sapiens (human)] 590035 http://omim.org/entry/590035 4563 MT-TH mitochondrially encoded tRNA histidine [Homo sapiens (human)] 590040 http://omim.org/entry/590040 4564 MT-TI mitochondrially encoded tRNA isoleucine [Homo sapiens (human)] 590045 http://omim.org/entry/590045 4565 MT-TK mitochondrially encoded tRNA lysine [Homo sapiens (human)] 590060 http://omim.org/entry/590060 4566 MT-TL1 mitochondrially encoded tRNA leucine 1 (UUA/G) [Homo sapiens (human)] 590050 http://omim.org/entry/590050 4567 MT-TL2 mitochondrially encoded tRNA leucine 2 (CUN) [Homo sapiens (human)] 590055 http://omim.org/entry/590055 4568 MT-TM mitochondrially encoded tRNA methionine [Homo sapiens (human)] 590065 http://omim.org/entry/590065 4569 MT-TN mitochondrially encoded tRNA asparagine [Homo sapiens (human)] 590010 http://omim.org/entry/590010 4570 MT-TP mitochondrially encoded tRNA proline [Homo sapiens (human)] 590075 http://omim.org/entry/590075 4571 MT-TQ mitochondrially encoded tRNA glutamine [Homo sapiens (human)] 590030 http://omim.org/entry/590030 4572 MT-TR mitochondrially encoded tRNA arginine [Homo sapiens (human)] 590005 http://omim.org/entry/590005 4573 MT-TS1 mitochondrially encoded tRNA serine 1 (UCN) [Homo sapiens (human)] 590080 http://omim.org/entry/590080 4574 MT-TS2 mitochondrially encoded tRNA serine 2 (AGU/C) [Homo sapiens (human)] 590085 http://omim.org/entry/590085 4575 MT-TT mitochondrially encoded tRNA threonine [Homo sapiens (human)] 590090 http://omim.org/entry/590090 4576 MT-TV mitochondrially encoded tRNA valine [Homo sapiens (human)] 590105 http://omim.org/entry/590105 4577 MT-TW mitochondrially encoded tRNA tryptophan [Homo sapiens (human)] 590095 http://omim.org/entry/590095 4578 MT-TY mitochondrially encoded tRNA tyrosine [Homo sapiens (human)] 590100 http://omim.org/entry/590100 4579 AARS2 alanyl-tRNA synthetase 2, mitochondrial[Homo sapiens] 612035 http://omim.org/entry/612035 57505 CARS2 cysteinyl-tRNA synthetase 2, mitochondrial (putative)[Homo sapiens] 612800 http://omim.org/entry/612800 79587 DARS2 aspartyl-tRNA synthetase 2, mitochondrial[Homo sapiens] 610956 http://omim.org/entry/610956 55157 EARS2 glutamyl-tRNA synthetase 2, mitochondrial[Homo sapiens] 612799 http://omim.org/entry/612799 124454 FARS2 phenylalanyl-tRNA synthetase 2, mitochondrial[Homo sapiens] 611592 http://omim.org/entry/611592 10667 GARS glycyl-tRNA synthetase[Homo sapiens] 600287 http://omim.org/entry/600287 2617 HARS2 histidyl-tRNA synthetase 2, mitochondrial[Homo sapiens] 600783 http://omim.org/entry/600783 23438 IARS2 isoleucyl-tRNA synthetase 2, mitochondrial[Homo sapiens] 612801 http://omim.org/entry/612801 55699 KARS lysyl-tRNA synthetase[Homo sapiens] 601421 http://omim.org/entry/601421 3735 LARS2 leucyl-tRNA synthetase 2, mitochondrial[Homo sapiens] 604544 http://omim.org/entry/604544 23395 MARS2 methionyl-tRNA synthetase 2, mitochondrial[Homo sapiens] 609728 http://omim.org/entry/609728 92935 MTFMT mitochondrial methionyl-tRNA formyltransferase[Homo sapiens] 611766 http://omim.org/entry/611766 123263 NARS2 asparaginyl-tRNA synthetase 2, mitochondrial (putative)[Homo sapiens] 612803 http://omim.org/entry/612803 79731 PARS2 prolyl-tRNA synthetase 2, mitochondrial (putative) [Homo sapiens (human)] 612036 http://omim.org/entry/612036 25973 QRSL1 glutaminyl-tRNA synthase (glutamine-hydrolyzing)-like 1 [Homo sapiens (human)] 617209 http://omim.org/entry/617209 55278 RARS2 arginyl-tRNA synthetase 2, mitochondrial[Homo sapiens] 611524 http://omim.org/entry/611524 57038 SARS2 seryl-tRNA synthetase 2, mitochondrial[Homo sapiens] 612804 http://omim.org/entry/612804 54938 TARS2 threonyl-tRNA synthetase 2, mitochondrial (putative)[Homo sapiens] 612805 http://omim.org/entry/612805 80222 VARS2 valyl-tRNA synthetase 2, mitochondrial[Homo sapiens] 612802 http://omim.org/entry/612802 57176 YARS2 tyrosyl-tRNA synthetase 2, mitochondrial[Homo sapiens] 610957 http://omim.org/entry/610957 51067 DLAT dihydrolipoamide S-acetyltransferase[Homo sapiens] 608770 http://omim.org/entry/608770 1737 PDHA1 pyruvate dehydrogenase (lipoamide) alpha 1[Homo sapiens] 300502 http://omim.org/entry/300502 5160 PDHB pyruvate dehydrogenase (lipoamide) beta[Homo sapiens] 179060 http://omim.org/entry/179060 5162 PDHX pyruvate dehydrogenase complex, component X[Homo sapiens] 608769 http://omim.org/entry/608769 8050 PDK3 pyruvate dehydrogenase kinase, isozyme 3[Homo sapiens] 602526 http://omim.org/entry/602526 5165 PDP1 pyruvate dehyrogenase phosphatase catalytic subunit 1[Homo sapiens] 605993 http://omim.org/entry/605993 54704 ACO2 aconitase 2, mitochondrial[Homo sapiens] 100850 http://omim.org/entry/100850 50 FH fumarate hydratase[Homo sapiens] 136850 http://omim.org/entry/136850 2271 IDH3B isocitrate dehydrogenase 3 (NAD+) beta[Homo sapiens] 604526 http://omim.org/entry/604526 3420 MDH2 malate dehydrogenase 2 [Homo sapiens (human)] 154100 http://omim.org/entry/154100 4191 MPC1 mitochondrial pyruvate carrier 1[Homo sapiens] 614738 http://omim.org/entry/614738 51660 SLC25A12 solute carrier family 25 (aspartate/glutamate carrier), member 12[Homo sapiens] 603667 http://omim.org/entry/603667 8604 SLC25A3 "solute carrier family 25 (mitochondrial carrier; phosphate carrier), member 3[Homo sapiens]" 600370 http://omim.org/entry/600370 5250 CA5A carbonic anhydrase VA, mitochondrial[Homo sapiens] 114761 http://omim.org/entry/114761 763 PC pyruvate carboxylase[Homo sapiens] 608786 http://omim.org/entry/608786 5091 PPA2 pyrophosphatase (inorganic) 2 [Homo sapiens (human)] 609988 http://omim.org/entry/609988 27068 ACAT1 acetyl-CoA acetyltransferase 1[Homo sapiens] 607809 http://omim.org/entry/607809 38 HMGCL 3-hydroxymethyl-3-methylglutaryl-CoA lyase[Homo sapiens] 613898 http://omim.org/entry/613898 3155 HMGCS2 3-hydroxy-3-methylglutaryl-CoA synthase 2 (mitochondrial)[Homo sapiens] 600234 http://omim.org/entry/600234 3158 OXCT1 3-oxoacid CoA transferase 1[Homo sapiens] 601424 http://omim.org/entry/601424 5019 ACADM acyl-CoA dehydrogenase, C-4 to C-12 straight chain[Homo sapiens] 607008 http://omim.org/entry/607008 34 ACADS acyl-CoA dehydrogenase, C-2 to C-3 short chain[Homo sapiens] 606885 http://omim.org/entry/606885 35 ACADSB acyl-CoA dehydrogenase, short/branched chain[Homo sapiens] 600301 http://omim.org/entry/600301 36 ACADVL acyl-CoA dehydrogenase, very long chain[Homo sapiens] 609575 http://omim.org/entry/609575 37 CPT1A carnitine palmitoyltransferase 1A (liver)[Homo sapiens] 600528 http://omim.org/entry/600528 1374 CPT2 carnitine palmitoyltransferase 2[Homo sapiens] 600650 http://omim.org/entry/600650 1376 ETFA electron-transfer-flavoprotein, alpha polypeptide[Homo sapiens] 608053 http://omim.org/entry/608053 2108 ETFB electron-transfer-flavoprotein, beta polypeptide[Homo sapiens] 130410 http://omim.org/entry/130410 2109 ETFDH electron-transferring-flavoprotein dehydrogenase[Homo sapiens] 231675 http://omim.org/entry/231675 2110 HADH hydroxyacyl-CoA dehydrogenase[Homo sapiens] 601609 http://omim.org/entry/601609 3033 HADHA hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit[Homo sapiens] 600890 http://omim.org/entry/600890 3030 HADHB hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), beta subunit[Homo sapiens] 143450 http://omim.org/entry/143450 3032 SLC22A5 solute carrier family 22 (organic cation/carnitine transporter), member 5[Homo sapiens] 603377 http://omim.org/entry/603377 6584 SLC25A20 solute carrier family 25 (carnitine/acylcarnitine translocase), member 20[Homo sapiens] 613698 http://omim.org/entry/613698 788 SLC19A3 solute carrier family 19, member 3[Homo sapiens] 606152 http://omim.org/entry/606152 80704 SLC25A19 solute carrier family 25 (mitochondrial thiamine pyrophosphate carrier), member 19[Homo sapiens] 606521 http://omim.org/entry/606521 60386 TPK1 thiamin pyrophosphokinase 1[Homo sapiens] 606370 http://omim.org/entry/606370 27010 DLD dihydrolipoamide dehydrogenase[Homo sapiens] 238331 http://omim.org/entry/238331 1738 LIAS lipoic acid synthetase[Homo sapiens] 607031 http://omim.org/entry/607031 11019 LIPT1 lipoyltransferase 1[Homo sapiens] 610284 http://omim.org/entry/610284 51601 MECR mitochondrial trans-2-enoyl-CoA reductase [Homo sapiens (human)] 608205 http://omim.org/entry/608205 51102 BOLA3 bolA homolog 3 (E. coli)[Homo sapiens] 613183 http://omim.org/entry/613183 388962 FDX1L ferredoxin 1-like[Homo sapiens] 614585 http://omim.org/entry/614585 112812 FXN frataxin[Homo sapiens] 606829 http://omim.org/entry/606829 2395 GLRX5 glutaredoxin 5[Homo sapiens] 609588 http://omim.org/entry/609588 51218 IBA57 IBA57, iron-sulfur cluster assembly homolog (S. cerevisiae)[Homo sapiens] 615316 http://omim.org/entry/615316 200205 ISCA2 iron-sulfur cluster assembly 2 [Homo sapiens (human)] 615317 http://omim.org/entry/615317 122961 ISCU iron-sulfur cluster scaffold homolog (E. coli)[Homo sapiens] 611911 http://omim.org/entry/611911 23479 LYRM4 LYR motif containing 4[Homo sapiens] 613311 http://omim.org/entry/613311 57128 NFS1 NFS1 cysteine desulfurase[Homo sapiens] 603485 http://omim.org/entry/603485 9054 NFU1 NFU1 iron-sulfur cluster scaffold homolog (S. cerevisiae)[Homo sapiens] 608100 http://omim.org/entry/608100 27247 NUBPL nucleotide binding protein-like[Homo sapiens] 613621 http://omim.org/entry/613621 80224 ADCK3 aarF domain containing kinase 3[Homo sapiens] 606980 http://omim.org/entry/606980 56997 ADCK4 aarF domain containing kinase 4[Homo sapiens] 615567 http://omim.org/entry/615567 79934 COQ2 coenzyme Q2 homolog, prenyltransferase (yeast)[Homo sapiens] 609825 http://omim.org/entry/609825 27235 COQ4 coenzyme Q4 homolog (S. cerevisiae)[Homo sapiens] 612898 http://omim.org/entry/612898 51117 COQ6 coenzyme Q6 homolog, monooxygenase (S. cerevisiae)[Homo sapiens] 614647 http://omim.org/entry/614647 51004 COQ7 coenzyme Q7 homolog, ubiquinone (yeast) [Homo sapiens (human)] 601683 http://omim.org/entry/601683 10229 COQ9 coenzyme Q9 homolog (S. cerevisiae)[Homo sapiens] 612837 http://omim.org/entry/612837 57017 PDSS1 prenyl (decaprenyl) diphosphate synthase, subunit 1[Homo sapiens] 607429 http://omim.org/entry/607429 23590 PDSS2 prenyl (decaprenyl) diphosphate synthase, subunit 2[Homo sapiens] 610564 http://omim.org/entry/610564 57107 BTD biotinidase[Homo sapiens] 609019 http://omim.org/entry/609019 686 HLCS holocarboxylase synthetase (biotin-(proprionyl-CoA-carboxylase (ATP-hydrolysing)) ligase)[Homo sapiens] 609018 http://omim.org/entry/609018 3141 COA6 cytochrome c oxidase assembly factor 6 homolog (S. cerevisiae)[Homo sapiens] 614772 http://omim.org/entry/614772 388753 SCO1 SCO1 cytochrome c oxidase assembly protein[Homo sapiens] 603644 http://omim.org/entry/603644 6341 SCO2 SCO2 cytochrome c oxidase assembly protein[Homo sapiens] 604272 http://omim.org/entry/604272 9997 COX10 cytochrome c oxidase assembly homolog 10 (yeast)[Homo sapiens] 602125 http://omim.org/entry/602125 1352 COX15 cytochrome c oxidase assembly homolog 15 (yeast)[Homo sapiens] 603646 http://omim.org/entry/603646 1355 CYCS cytochrome c, somatic[Homo sapiens] 123970 http://omim.org/entry/123970 54205 HCCS holocytochrome c synthase[Homo sapiens] 300056 http://omim.org/entry/300056 3052 PPOX protoporphyrinogen oxidase[Homo sapiens] 600923 http://omim.org/entry/600923 5498 SLC25A38 solute carrier family 25, member 38[Homo sapiens] 610819 http://omim.org/entry/610819 54977 COASY CoA synthase[Homo sapiens] 609855 http://omim.org/entry/609855 80347 PANK2 pantothenate kinase 2[Homo sapiens] 606157 http://omim.org/entry/606157 80025 SLC25A42 solute carrier family 25, member 42 [Homo sapiens (human)] 610823 http://omim.org/entry/610823 284439 FLAD1 flavin adenine dinucleotide synthetase 1[Homo sapiens] 610595 http://omim.org/entry/610595 80308 SLC25A32 solute carrier family 25 member 32 [Homo sapiens (human)] 610815 http://omim.org/entry/610815 81034 NADK2 NAD kinase 2, mitochondrial[Homo sapiens] 615787 http://omim.org/entry/615787 133686 NAXE NAD(P)HX epimerase [Homo sapiens (human)] 608862 http://omim.org/entry/608862 128240 SFXN4 sideroflexin 4[Homo sapiens] 615564 http://omim.org/entry/615564 119559 SLC25A46 solute carrier family 25 member 46 [Homo sapiens (human)] 610826 http://omim.org/entry/610826 91137 AGK acylglycerol kinase[Homo sapiens] 610345 http://omim.org/entry/610345 55750 SERAC1 serine active site containing 1[Homo sapiens] 614725 http://omim.org/entry/614725 84947 TAZ tafazzin[Homo sapiens] 300394 http://omim.org/entry/300394 6901 AIFM1 apoptosis-inducing factor, mitochondrion-associated, 1[Homo sapiens] 300169 http://omim.org/entry/300169 9131 DNAJC19 DnaJ (Hsp40) homolog, subfamily C, member 19[Homo sapiens] 608977 http://omim.org/entry/608977 131118 GFER growth factor, augmenter of liver regeneration[Homo sapiens] 600924 http://omim.org/entry/600924 2671 PMPCA peptidase (mitochondrial processing) alpha [Homo sapiens (human)] 613036 http://omim.org/entry/613036 23203 TIMM50 translocase of inner mitochondrial membrane 50 [Homo sapiens (human)] 607381 http://omim.org/entry/607381 92609 TIMM8A translocase of inner mitochondrial membrane 8 homolog A (yeast)[Homo sapiens] 300356 http://omim.org/entry/300356 1678 XPNPEP3 X-prolyl aminopeptidase (aminopeptidase P) 3, putative[Homo sapiens] 613553 http://omim.org/entry/613553 63929 AFG3L2 AFG3 ATPase family gene 3-like 2 (S. cerevisiae)[Homo sapiens] 604581 http://omim.org/entry/604581 10939 CLPB ClpB homolog, mitochondrial AAA ATPase chaperonin [Homo sapiens (human)] 616254 http://omim.org/entry/616254 81570 CLPP ClpP caseinolytic peptidase, ATP-dependent, proteolytic subunit homolog (E. coli)[Homo sapiens] 601119 http://omim.org/entry/601119 8192 HSPD1 heat shock 60kDa protein 1 (chaperonin)[Homo sapiens] 118190 http://omim.org/entry/118190 3329 HTRA2 HtrA serine peptidase 2[Homo sapiens] 606441 http://omim.org/entry/606441 27429 LONP1 lon peptidase 1, mitochondrial[Homo sapiens] 605490 http://omim.org/entry/605490 9361 MIPEP mitochondrial intermediate peptidase [Homo sapiens (human)] 602241 http://omim.org/entry/602241 4285 SACS sacsin molecular chaperone [Homo sapiens (human)] 604490 http://omim.org/entry/604490 26278 SPG7 spastic paraplegia 7 (pure and complicated autosomal recessive)[Homo sapiens] 602783 http://omim.org/entry/602783 6687 DNM1L dynamin 1-like[Homo sapiens] 603850 http://omim.org/entry/603850 10059 GDAP1 ganglioside induced differentiation associated protein 1[Homo sapiens] 606598 http://omim.org/entry/606598 54332 MFF mitochondrial fission factor[Homo sapiens] 614785 http://omim.org/entry/614785 56947 MFN2 mitofusin 2[Homo sapiens] 608507 http://omim.org/entry/608507 9927 OPA1 optic atrophy 1 (autosomal dominant)[Homo sapiens] 605290 http://omim.org/entry/605290 4976 SLC25A46 solute carrier family 25, member 46 [Homo sapiens (human)] 610826 http://omim.org/entry/610826 91137 STAT2 signal transducer and activator of transcription 2, 113kDa [Homo sapiens (human)] 600556 http://omim.org/entry/600556 6773 YME1L1 YME1 like 1 ATPase [Homo sapiens (human)] 607472 http://omim.org/entry/607472 10730 MICU1 mitochondrial calcium uptake 1 [Homo sapiens (human)] 605084 http://omim.org/entry/605084 10367 C19orf70 chromosome 19 open reading frame 70 [Homo sapiens (human)] 616658 http://omim.org/entry/616658 125988 ETHE1 ethylmalonic encephalopathy 1[Homo sapiens] 608451 http://omim.org/entry/608451 23474 SLC25A1 "solute carrier family 25 (mitochondrial carrier; citrate transporter), member 1[Homo sapiens]" 190315 http://omim.org/entry/190315 6576 HTT huntingtin[Homo sapiens] 613004 http://omim.org/entry/613004 3064 TXN2 thioredoxin 2 [Homo sapiens (human)] 609063 http://omim.org/entry/609063 25828 D2HGDH D-2-hydroxyglutarate dehydrogenase[Homo sapiens] 609186 http://omim.org/entry/609186 728294 ECHS1 enoyl CoA hydratase, short chain, 1, mitochondrial[Homo sapiens] 602292 http://omim.org/entry/602292 1892 HIBCH 3-hydroxyisobutyryl-CoA hydrolase[Homo sapiens] 610690 http://omim.org/entry/610690 26275 IDH2 isocitrate dehydrogenase 2 (NADP+), mitochondrial[Homo sapiens] 147650 http://omim.org/entry/147650 3418 L2HGDH L-2-hydroxyglutarate dehydrogenase[Homo sapiens] 609584 http://omim.org/entry/609584 79944 APOPT1 apoptogenic 1, mitochondrial[Homo sapiens] 616003 http://omim.org/entry/616003 84334 CHCHD10 coiled-coil-helix-coiled-coil-helix domain containing 10[Homo sapiens] 615903 http://omim.org/entry/615903 400916 FBXL4 F-box and leucine-rich repeat protein 4[] 605654 http://omim.org/entry/605654 26235 OPA3 optic atrophy 3 (autosomal recessive, with chorea and spastic paraplegia)[Homo sapiens] 606580 http://omim.org/entry/606580 80207