KEGG   PATHWAY: hsa00190Help
Entry
hsa00190                    Pathway                                
Name
Oxidative phosphorylation - Homo sapiens (human)
Class
Metabolism; Energy metabolism
BRITE hierarchy
Pathway map
Oxidative phosphorylation
hsa00190

All organismsOrtholog table
Module
NADH:ubiquinone oxidoreductase, mitochondria [PATH:hsa00190]
NADH dehydrogenase (ubiquinone) Fe-S protein/flavoprotein complex, mitochondria [PATH:hsa00190]
NADH:quinone oxidoreductase, prokaryotes [PATH:hsa00190]
NAD(P)H:quinone oxidoreductase, chloroplasts and cyanobacteria [PATH:hsa00190]
NADH dehydrogenase (ubiquinone) 1 alpha subcomplex [PATH:hsa00190]
NADH dehydrogenase (ubiquinone) 1 beta subcomplex [PATH:hsa00190]
Succinate dehydrogenase (ubiquinone) [PATH:hsa00190]
Succinate dehydrogenase, prokaryotes [PATH:hsa00190]
Fumarate reductase, prokaryotes [PATH:hsa00190]
Cytochrome bc1 complex respiratory unit [PATH:hsa00190]
Cytochrome bc1 complex [PATH:hsa00190]
Cytochrome d ubiquinol oxidase [PATH:hsa00190]
Cytochrome c oxidase [PATH:hsa00190]
Cytochrome c oxidase, prokaryotes [PATH:hsa00190]
Cytochrome c oxidase, cbb3-type [PATH:hsa00190]
F-type ATPase, eukaryotes [PATH:hsa00190]
V-type ATPase, eukaryotes [PATH:hsa00190]
Cytochrome aa3-600 menaquinol oxidase [PATH:hsa00190]
Cytochrome o ubiquinol oxidase [PATH:hsa00190]
Disease
H00068  
Leber optic atrophy
H00473  
Mitochondrial respiratory chain deficiencies (MRCD)
H00920  
Exocrine pancreatic insufficiency, dyserythropoietic anemia, and calvarial hyperostosis
H01177  
Infantile bilateral striatal necrosis (IBSN)
H01222  
Cowden-like syndrome
Drug
D00355  
Lansoprazole (JAN/USP/INN)
D00455  
Omeprazole (JP16/USP/INN)
D00724  
Sodium rabeprazole (JP16)
D01207  
Omeprazole sodium (USAN)
D01920  
Tenatoprazole (JAN/INN)
D01984  
Esomeprazole magnesium hydrate (JAN)
D02593  
Pantoprazole Sodium Hydrate (JAN)
D04056  
Esomeprazole sodium (USAN)
D05259  
Omeprazole magnesium (USAN)
D05261  
Omeprazole sodium injection (JAN)
D05353  
Pantoprazole (USAN/INN)
D05900  
Timoprazole (INN)
D05901  
Picoprazole (INN)
D05906  
Leminoprazole (INN)
D07917  
Esomeprazole (INN)
D08463  
Rabeprazole (INN)
D08903  
Dexlansoprazole (INN/USAN)
D09339  
Esomeprazole potassium (USAN)
Organism
Homo sapiens (human) [GN:hsa]
Gene
4535  
ND1; NADH dehydrogenase, subunit 1 (complex I) [KO:K03878] [EC:1.6.5.3]
4536  
ND2; MTND2 [KO:K03879] [EC:1.6.5.3]
4537  
ND3; NADH dehydrogenase, subunit 3 (complex I) [KO:K03880] [EC:1.6.5.3]
4538  
ND4; NADH dehydrogenase, subunit 4 (complex I) [KO:K03881] [EC:1.6.5.3]
4539  
ND4L; NADH dehydrogenase, subunit 4L (complex I) [KO:K03882] [EC:1.6.5.3]
4540  
ND5; NADH dehydrogenase, subunit 5 (complex I) [KO:K03883] [EC:1.6.5.3]
4541  
ND6; NADH dehydrogenase, subunit 6 (complex I) [KO:K03884] [EC:1.6.5.3]
4719  
NDUFS1; NADH dehydrogenase (ubiquinone) Fe-S protein 1, 75kDa (NADH-coenzyme Q reductase) [KO:K03934] [EC:1.6.99.3 1.6.5.3]
4720  
NDUFS2; NADH dehydrogenase (ubiquinone) Fe-S protein 2, 49kDa (NADH-coenzyme Q reductase) [KO:K03935] [EC:1.6.99.3 1.6.5.3]
4722  
NDUFS3; NADH dehydrogenase (ubiquinone) Fe-S protein 3, 30kDa (NADH-coenzyme Q reductase) [KO:K03936] [EC:1.6.99.3 1.6.5.3]
4724  
NDUFS4; NADH dehydrogenase (ubiquinone) Fe-S protein 4, 18kDa (NADH-coenzyme Q reductase) [KO:K03937] [EC:1.6.99.3 1.6.5.3]
4725  
NDUFS5; NADH dehydrogenase (ubiquinone) Fe-S protein 5, 15kDa (NADH-coenzyme Q reductase) [KO:K03938] [EC:1.6.99.3 1.6.5.3]
4726  
NDUFS6; NADH dehydrogenase (ubiquinone) Fe-S protein 6, 13kDa (NADH-coenzyme Q reductase) [KO:K03939] [EC:1.6.99.3 1.6.5.3]
374291  
NDUFS7; NADH dehydrogenase (ubiquinone) Fe-S protein 7, 20kDa (NADH-coenzyme Q reductase) [KO:K03940] [EC:1.6.99.3 1.6.5.3]
4728  
NDUFS8; NADH dehydrogenase (ubiquinone) Fe-S protein 8, 23kDa (NADH-coenzyme Q reductase) [KO:K03941] [EC:1.6.99.3 1.6.5.3]
4723  
NDUFV1; NADH dehydrogenase (ubiquinone) flavoprotein 1, 51kDa [KO:K03942] [EC:1.6.99.3 1.6.5.3]
4729  
NDUFV2; NADH dehydrogenase (ubiquinone) flavoprotein 2, 24kDa [KO:K03943] [EC:1.6.99.3 1.6.5.3]
4731  
NDUFV3; NADH dehydrogenase (ubiquinone) flavoprotein 3, 10kDa [KO:K03944] [EC:1.6.99.3 1.6.5.3]
4694  
NDUFA1; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 1, 7.5kDa [KO:K03945] [EC:1.6.99.3 1.6.5.3]
4695  
NDUFA2; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 2, 8kDa [KO:K03946] [EC:1.6.99.3 1.6.5.3]
4696  
NDUFA3; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 3, 9kDa [KO:K03947] [EC:1.6.99.3 1.6.5.3]
4697  
NDUFA4; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 4, 9kDa [KO:K03948] [EC:1.6.99.3 1.6.5.3]
56901  
NDUFA4L2; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 4-like 2 [KO:K03948] [EC:1.6.99.3 1.6.5.3]
4698  
NDUFA5; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 5, 13kDa [KO:K03949] [EC:1.6.99.3 1.6.5.3]
4700  
NDUFA6; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 6, 14kDa [KO:K03950] [EC:1.6.99.3 1.6.5.3]
4701  
NDUFA7; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 7, 14.5kDa [KO:K03951] [EC:1.6.99.3 1.6.5.3]
4702  
NDUFA8; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 8, 19kDa [KO:K03952] [EC:1.6.99.3 1.6.5.3]
4704  
NDUFA9; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 9, 39kDa [KO:K03953] [EC:1.6.99.3 1.6.5.3]
4705  
NDUFA10; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 10, 42kDa [KO:K03954] [EC:1.6.99.3 1.6.5.3]
4706  
NDUFAB1; NADH dehydrogenase (ubiquinone) 1, alpha/beta subcomplex, 1, 8kDa [KO:K03955] [EC:1.6.99.3 1.6.5.3]
126328  
NDUFA11; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 11, 14.7kDa [KO:K03956] [EC:1.6.5.3]
55967  
NDUFA12; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 12 [KO:K11352] [EC:1.6.99.3 1.6.5.3]
51079  
NDUFA13; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 13 [KO:K11353] [EC:1.6.99.3 1.6.5.3]
4707  
NDUFB1; NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 1, 7kDa [KO:K03957] [EC:1.6.99.3 1.6.5.3]
4708  
NDUFB2; NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 2, 8kDa [KO:K03958] [EC:1.6.99.3 1.6.5.3]
4709  
NDUFB3; NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 3, 12kDa [KO:K03959] [EC:1.6.99.3 1.6.5.3]
4710  
NDUFB4; NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 4, 15kDa [KO:K03960] [EC:1.6.99.3 1.6.5.3]
4711  
NDUFB5; NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 5, 16kDa [KO:K03961] [EC:1.6.99.3 1.6.5.3]
4712  
NDUFB6; NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 6, 17kDa [KO:K03962] [EC:1.6.99.3 1.6.5.3]
4713  
NDUFB7; NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 7, 18kDa [KO:K03963] [EC:1.6.99.3 1.6.5.3]
4714  
NDUFB8; NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 8, 19kDa [KO:K03964] [EC:1.6.99.3 1.6.5.3]
4715  
NDUFB9; NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 9, 22kDa [KO:K03965] [EC:1.6.99.3 1.6.5.3]
4716  
NDUFB10; NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 10, 22kDa [KO:K03966] [EC:1.6.99.3 1.6.5.3]
54539  
NDUFB11; NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 11, 17.3kDa [KO:K11351] [EC:1.6.99.3 1.6.5.3]
4717  
NDUFC1; NADH dehydrogenase (ubiquinone) 1, subcomplex unknown, 1, 6kDa [KO:K03967] [EC:1.6.99.3 1.6.5.3]
4718  
NDUFC2; NADH dehydrogenase (ubiquinone) 1, subcomplex unknown, 2, 14.5kDa [KO:K03968] [EC:1.6.99.3 1.6.5.3]
NDUFC2-KCTD14; NDUFC2-KCTD14 readthrough [KO:K03968] [EC:1.6.99.3 1.6.5.3]
6391  
SDHC; succinate dehydrogenase complex, subunit C, integral membrane protein, 15kDa [KO:K00236]
6392  
SDHD; succinate dehydrogenase complex, subunit D, integral membrane protein [KO:K00237]
6389  
SDHA; succinate dehydrogenase complex, subunit A, flavoprotein (Fp) [KO:K00234] [EC:1.3.5.1]
6390  
SDHB; succinate dehydrogenase complex, subunit B, iron sulfur (Ip) [KO:K00235] [EC:1.3.5.1]
7386  
UQCRFS1; ubiquinol-cytochrome c reductase, Rieske iron-sulfur polypeptide 1 [KO:K00411] [EC:1.10.2.2]
4519  
CYTB; cytochrome b [KO:K00412]
1537  
CYC1; cytochrome c-1 [KO:K00413]
7384  
UQCRC1; ubiquinol-cytochrome c reductase core protein I [KO:K00414]
7385  
UQCRC2; ubiquinol-cytochrome c reductase core protein II [KO:K00415]
7388  
UQCRH; ubiquinol-cytochrome c reductase hinge protein [KO:K00416]
440567  
UQCRHL; ubiquinol-cytochrome c reductase hinge protein-like [KO:K00416]
7381  
UQCRB; ubiquinol-cytochrome c reductase binding protein [KO:K00417]
27089  
UQCRQ; ubiquinol-cytochrome c reductase, complex III subunit VII, 9.5kDa [KO:K00418]
29796  
UQCR10; ubiquinol-cytochrome c reductase, complex III subunit X [KO:K00419]
10975  
UQCR11; ubiquinol-cytochrome c reductase, complex III subunit XI [KO:K00420]
1352  
COX10; cytochrome c oxidase assembly homolog 10 (yeast) [KO:K02257] [EC:2.5.1.-]
4514  
COX3; cytochrome c oxidase III [KO:K02262]
4512  
COX1; cytochrome c oxidase subunit I [KO:K02256] [EC:1.9.3.1]
4513  
COX2; cytochrome c oxidase subunit II [KO:K02261]
84701  
COX4I2; cytochrome c oxidase subunit IV isoform 2 (lung) [KO:K02263]
1327  
COX4I1; cytochrome c oxidase subunit IV isoform 1 [KO:K02263]
9377  
COX5A; cytochrome c oxidase subunit Va [KO:K02264]
1329  
COX5B; cytochrome c oxidase subunit Vb [KO:K02265]
1337  
COX6A1; cytochrome c oxidase subunit VIa polypeptide 1 [KO:K02266]
1339  
COX6A2; cytochrome c oxidase subunit VIa polypeptide 2 [KO:K02266]
1340  
COX6B1; cytochrome c oxidase subunit VIb polypeptide 1 (ubiquitous) [KO:K02267]
125965  
COX6B2; cytochrome c oxidase subunit VIb polypeptide 2 (testis) [KO:K02267]
1345  
COX6C; cytochrome c oxidase subunit VIc [KO:K02268]
1346  
COX7A1; cytochrome c oxidase subunit VIIa polypeptide 1 (muscle) [KO:K02270]
1347  
COX7A2; cytochrome c oxidase subunit VIIa polypeptide 2 (liver) [KO:K02270]
9167  
COX7A2L; cytochrome c oxidase subunit VIIa polypeptide 2 like [KO:K02270]
1349  
COX7B; cytochrome c oxidase subunit VIIb [KO:K02271]
170712  
COX7B2; cytochrome c oxidase subunit VIIb2 [KO:K02271]
1350  
COX7C; cytochrome c oxidase subunit VIIc [KO:K02272]
341947  
COX8C; cytochrome c oxidase subunit VIIIC [KO:K02273]
1351  
COX8A; cytochrome c oxidase subunit VIIIA (ubiquitous) [KO:K02273]
1353  
COX11; cytochrome c oxidase assembly homolog 11 (yeast) [KO:K02258]
1355  
COX15; cytochrome c oxidase assembly homolog 15 (yeast) [KO:K02259]
10063  
COX17; cytochrome c oxidase assembly homolog 17 (yeast) [KO:K02260]
514  
ATP5E; ATP synthase, H+ transporting, mitochondrial F1 complex, epsilon subunit [KO:K02135] [EC:3.6.3.14]
513  
ATP5D; ATP synthase, H+ transporting, mitochondrial F1 complex, delta subunit [KO:K02134] [EC:3.6.3.14]
506  
ATP5B; ATP synthase, H+ transporting, mitochondrial F1 complex, beta polypeptide [KO:K02133] [EC:3.6.3.14]
509  
ATP5C1; ATP synthase, H+ transporting, mitochondrial F1 complex, gamma polypeptide 1 [KO:K02136] [EC:3.6.3.14]
498  
ATP5A1; ATP synthase, H+ transporting, mitochondrial F1 complex, alpha subunit 1, cardiac muscle [KO:K02132] [EC:3.6.3.14]
539  
ATP5O; ATP synthase, H+ transporting, mitochondrial F1 complex, O subunit [KO:K02137] [EC:3.6.3.14]
4509  
ATP8; ATP synthase F0 subunit 8 [KO:K02125] [EC:3.6.3.14]
516  
ATP5G1; ATP synthase, H+ transporting, mitochondrial Fo complex, subunit C1 (subunit 9) [KO:K02128] [EC:3.6.3.14]
517  
ATP5G2; ATP synthase, H+ transporting, mitochondrial Fo complex, subunit C2 (subunit 9) [KO:K02128] [EC:3.6.3.14]
518  
ATP5G3; ATP synthase, H+ transporting, mitochondrial Fo complex, subunit C3 (subunit 9) [KO:K02128] [EC:3.6.3.14]
4508  
ATP6; ATP synthase F0 subunit 6 [KO:K02126] [EC:3.6.3.14]
515  
ATP5F1; ATP synthase, H+ transporting, mitochondrial Fo complex, subunit B1 [KO:K02127] [EC:3.6.3.14]
521  
ATP5I; ATP synthase, H+ transporting, mitochondrial Fo complex, subunit E [KO:K02129] [EC:3.6.3.14]
522  
ATP5J; ATP synthase, H+ transporting, mitochondrial Fo complex, subunit F6 [KO:K02131] [EC:3.6.3.14]
9551  
ATP5J2; ATP synthase, H+ transporting, mitochondrial Fo complex, subunit F2 [KO:K02130] [EC:3.6.3.14]
10476  
ATP5H; ATP synthase, H+ transporting, mitochondrial Fo complex, subunit d [KO:K02138] [EC:3.6.3.14]
10632  
ATP5L; ATP synthase, H+ transporting, mitochondrial Fo complex, subunit G [KO:K02140] [EC:3.6.3.14]
9114  
ATP6V0D1; ATPase, H+ transporting, lysosomal 38kDa, V0 subunit d1 [KO:K02146] [EC:3.6.3.14]
245972  
ATP6V0D2; ATPase, H+ transporting, lysosomal 38kDa, V0 subunit d2 [KO:K02146] [EC:3.6.3.14]
9296  
ATP6V1F; ATPase, H+ transporting, lysosomal 14kDa, V1 subunit F [KO:K02151] [EC:3.6.3.14]
245973  
ATP6V1C2; ATPase, H+ transporting, lysosomal 42kDa, V1 subunit C2 [KO:K02148] [EC:3.6.3.14]
528  
ATP6V1C1; ATPase, H+ transporting, lysosomal 42kDa, V1 subunit C1 [KO:K02148] [EC:3.6.3.14]
523  
ATP6V1A; ATPase, H+ transporting, lysosomal 70kDa, V1 subunit A [KO:K02145] [EC:3.6.3.14]
525  
ATP6V1B1; ATPase, H+ transporting, lysosomal 56/58kDa, V1 subunit B1 [KO:K02147] [EC:3.6.3.14]
526  
ATP6V1B2; ATPase, H+ transporting, lysosomal 56/58kDa, V1 subunit B2 [KO:K02147] [EC:3.6.3.14]
90423  
ATP6V1E2; ATPase, H+ transporting, lysosomal 31kDa, V1 subunit E2 [KO:K02150] [EC:3.6.3.14]
529  
ATP6V1E1; ATPase, H+ transporting, lysosomal 31kDa, V1 subunit E1 [KO:K02150] [EC:3.6.3.14]
527  
ATP6V0C; ATPase, H+ transporting, lysosomal 16kDa, V0 subunit c [KO:K02155] [EC:3.6.3.14]
533  
ATP6V0B; ATPase, H+ transporting, lysosomal 21kDa, V0 subunit b [KO:K03661] [EC:3.6.3.14]
10312  
TCIRG1; T-cell, immune regulator 1, ATPase, H+ transporting, lysosomal V0 subunit A3 [KO:K02154] [EC:3.6.3.14]
23545  
ATP6V0A2; ATPase, H+ transporting, lysosomal V0 subunit a2 [KO:K02154] [EC:3.6.3.14]
50617  
ATP6V0A4; ATPase, H+ transporting, lysosomal V0 subunit a4 [KO:K02154] [EC:3.6.3.14]
535  
ATP6V0A1; ATPase, H+ transporting, lysosomal V0 subunit a1 [KO:K02154] [EC:3.6.3.14]
51382  
ATP6V1D; ATPase, H+ transporting, lysosomal 34kDa, V1 subunit D [KO:K02149] [EC:3.6.3.14]
8992  
ATP6V0E1; ATPase, H+ transporting, lysosomal 9kDa, V0 subunit e1 [KO:K02153] [EC:3.6.3.14]
155066  
ATP6V0E2; ATPase, H+ transporting V0 subunit e2 [KO:K02153] [EC:3.6.3.14]
9550  
ATP6V1G1; ATPase, H+ transporting, lysosomal 13kDa, V1 subunit G1 [KO:K02152] [EC:3.6.3.14]
127124  
ATP6V1G3; ATPase, H+ transporting, lysosomal 13kDa, V1 subunit G3 [KO:K02152] [EC:3.6.3.14]
534  
ATP6V1G2; ATPase, H+ transporting, lysosomal 13kDa, V1 subunit G2 [KO:K02152] [EC:3.6.3.14]
51606  
ATP6V1H; ATPase, H+ transporting, lysosomal 50/57kDa, V1 subunit H [KO:K02144] [EC:3.6.3.14]
537  
ATP6AP1; ATPase, H+ transporting, lysosomal accessory protein 1 [KO:K03662] [EC:3.6.3.14]
495  
ATP4A; ATPase, H+/K+ exchanging, alpha polypeptide [KO:K01542] [EC:3.6.3.10]
496  
ATP4B; ATPase, H+/K+ exchanging, beta polypeptide [KO:K01543] [EC:3.6.3.10]
479  
ATP12A; ATPase, H+/K+ transporting, nongastric, alpha polypeptide [KO:K01544] [EC:3.6.3.10]
27068  
PPA2; pyrophosphatase (inorganic) 2 [KO:K01507] [EC:3.6.1.1]
5464  
PPA1; pyrophosphatase (inorganic) 1 [KO:K01507] [EC:3.6.1.1]
64077  
LHPP; phospholysine phosphohistidine inorganic pyrophosphate phosphatase [KO:K11725] [EC:3.1.3.- 3.6.1.1]
Compound
C00001  
H2O
C00002  
ATP
C00003  
NAD+
C00004  
NADH
C00007  
Oxygen
C00008  
ADP
C00009  
Orthophosphate
C00013  
Diphosphate
C00042  
Succinate
C00061  
FMN
C00080  
H+
C00122  
Fumarate
C00390  
Ubiquinol
C00399  
Ubiquinone
C00524  
Cytochrome c
C00536  
Triphosphate
Reference
  Authors
Sazanov LA, Hinchliffe P.
  Title
Structure of the hydrophilic domain of respiratory complex I from Thermus thermophilus.
  Journal
Science 311:1430-6 (2006)
Reference
  Authors
Hinchliffe P, Carroll J, Sazanov LA.
  Title
Identification of a novel subunit of respiratory complex I from Thermus thermophilus.
  Journal
Biochemistry 45:4413-20 (2006)
KO pathway
 
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