Mitochondrial respiratory chain complexes Gene Family

HGNC Family ID 639

Genes

Symbol Name
ATP5A1 ATP synthase, H+ transporting, mitochondrial F1 complex, alpha subunit 1, cardiac muscle
ATP5B ATP synthase, H+ transporting, mitochondrial F1 complex, beta polypeptide
ATP5C1 ATP synthase, H+ transporting, mitochondrial F1 complex, gamma polypeptide 1
ATP5D ATP synthase, H+ transporting, mitochondrial F1 complex, delta subunit
ATP5E ATP synthase, H+ transporting, mitochondrial F1 complex, epsilon subunit
ATP5F1 ATP synthase, H+ transporting, mitochondrial Fo complex, subunit B1
ATP5G1 ATP synthase, H+ transporting, mitochondrial Fo complex, subunit C1 (subunit 9)
ATP5G2 ATP synthase, H+ transporting, mitochondrial Fo complex, subunit C2 (subunit 9)
ATP5G3 ATP synthase, H+ transporting, mitochondrial Fo complex, subunit C3 (subunit 9)
ATP5H ATP synthase, H+ transporting, mitochondrial Fo complex, subunit d
ATP5I ATP synthase, H+ transporting, mitochondrial Fo complex, subunit E
ATP5J ATP synthase, H+ transporting, mitochondrial Fo complex, subunit F6
ATP5J2 ATP synthase, H+ transporting, mitochondrial Fo complex, subunit F2
ATP5L ATP synthase, H+ transporting, mitochondrial Fo complex, subunit G
ATP5L2 ATP synthase, H+ transporting, mitochondrial Fo complex, subunit G2
ATP5O ATP synthase, H+ transporting, mitochondrial F1 complex, O subunit
ATPIF1 ATPase inhibitory factor 1
COX4I1 cytochrome c oxidase subunit IV isoform 1
COX4I2 cytochrome c oxidase subunit IV isoform 2 (lung)
COX5A cytochrome c oxidase subunit Va
COX5B cytochrome c oxidase subunit Vb
COX6A1 cytochrome c oxidase subunit VIa polypeptide 1
COX6A2 cytochrome c oxidase subunit VIa polypeptide 2
COX6B1 cytochrome c oxidase subunit VIb polypeptide 1 (ubiquitous)
COX6B2 cytochrome c oxidase subunit VIb polypeptide 2 (testis)
COX6C cytochrome c oxidase subunit VIc
COX7A1 cytochrome c oxidase subunit VIIa polypeptide 1 (muscle)
COX7A2 cytochrome c oxidase subunit VIIa polypeptide 2 (liver)
COX7B cytochrome c oxidase subunit VIIb
COX7B2 cytochrome c oxidase subunit VIIb2
COX7C cytochrome c oxidase subunit VIIc
COX8A cytochrome c oxidase subunit VIIIA (ubiquitous)
COX8C cytochrome c oxidase subunit VIIIC
CYC1 cytochrome c-1
MT-ATP6 ATP synthase F0 subunit 6
MT-ATP8 ATP synthase F0 subunit 8
MT-CO1 cytochrome c oxidase subunit I
MT-CO2 cytochrome c oxidase subunit II
MT-CO3 cytochrome c oxidase III
MT-CYB cytochrome b
MT-ND1 NADH dehydrogenase, subunit 1 (complex I)
MT-ND2 MTND2
MT-ND3 NADH dehydrogenase, subunit 3 (complex I)
MT-ND4 NADH dehydrogenase, subunit 4 (complex I)
MT-ND4L NADH dehydrogenase, subunit 4L (complex I)
MT-ND5 NADH dehydrogenase, subunit 5 (complex I)
MT-ND6 NADH dehydrogenase, subunit 6 (complex I)
NDUFA1 NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 1, 7.5kDa
NDUFA10 NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 10, 42kDa
NDUFA11 NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 11, 14.7kDa
NDUFA12 NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 12
NDUFA13 NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 13
NDUFA2 NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 2, 8kDa
NDUFA3 NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 3, 9kDa
NDUFA5 NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 5
NDUFA6 NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 6, 14kDa
NDUFA7 NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 7, 14.5kDa
NDUFA8 NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 8, 19kDa
NDUFA9 NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 9, 39kDa
NDUFAB1 NADH dehydrogenase (ubiquinone) 1, alpha/beta subcomplex, 1, 8kDa
NDUFB1 NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 1, 7kDa
NDUFB10 NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 10, 22kDa
NDUFB11 NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 11, 17.3kDa
NDUFB2 NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 2, 8kDa
NDUFB3 NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 3, 12kDa
NDUFB4 NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 4, 15kDa
NDUFB5 NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 5, 16kDa
NDUFB6 NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 6, 17kDa
NDUFB7 NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 7, 18kDa
NDUFB8 NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 8, 19kDa
NDUFB9 NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 9, 22kDa
NDUFC1 NADH dehydrogenase (ubiquinone) 1, subcomplex unknown, 1, 6kDa
NDUFC2 NADH dehydrogenase (ubiquinone) 1, subcomplex unknown, 2, 14.5kDa
NDUFS1 NADH dehydrogenase (ubiquinone) Fe-S protein 1, 75kDa (NADH-coenzyme Q reductase)
NDUFS2 NADH dehydrogenase (ubiquinone) Fe-S protein 2, 49kDa (NADH-coenzyme Q reductase)
NDUFS3 NADH dehydrogenase (ubiquinone) Fe-S protein 3, 30kDa (NADH-coenzyme Q reductase)
NDUFS4 NADH dehydrogenase (ubiquinone) Fe-S protein 4, 18kDa (NADH-coenzyme Q reductase)
NDUFS5 NADH dehydrogenase (ubiquinone) Fe-S protein 5, 15kDa (NADH-coenzyme Q reductase)
NDUFS6 NADH dehydrogenase (ubiquinone) Fe-S protein 6, 13kDa (NADH-coenzyme Q reductase)
NDUFS7 NADH dehydrogenase (ubiquinone) Fe-S protein 7, 20kDa (NADH-coenzyme Q reductase)
NDUFS8 NADH dehydrogenase (ubiquinone) Fe-S protein 8, 23kDa (NADH-coenzyme Q reductase)
NDUFV1 NADH dehydrogenase (ubiquinone) flavoprotein 1, 51kDa
NDUFV2 NADH dehydrogenase (ubiquinone) flavoprotein 2, 24kDa
NDUFV3 NADH dehydrogenase (ubiquinone) flavoprotein 3, 10kDa
SDHA succinate dehydrogenase complex, subunit A, flavoprotein (Fp)
SDHB succinate dehydrogenase complex, subunit B, iron sulfur (Ip)
SDHC succinate dehydrogenase complex, subunit C, integral membrane protein, 15kDa
SDHD succinate dehydrogenase complex, subunit D, integral membrane protein
UQCR10 ubiquinol-cytochrome c reductase, complex III subunit X
UQCR11 ubiquinol-cytochrome c reductase, complex III subunit XI
UQCRB ubiquinol-cytochrome c reductase binding protein
UQCRC1 ubiquinol-cytochrome c reductase core protein I
UQCRC2 ubiquinol-cytochrome c reductase core protein II
UQCRFS1 ubiquinol-cytochrome c reductase, Rieske iron-sulfur polypeptide 1
UQCRH ubiquinol-cytochrome c reductase hinge protein
UQCRQ ubiquinol-cytochrome c reductase, complex III subunit VII, 9.5kDa