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 dehydrogenase (ubiquinone) 1 alpha subcomplex [PATH:hsa00190]
NADH dehydrogenase (ubiquinone) 1 beta subcomplex [PATH:hsa00190]
Succinate dehydrogenase (ubiquinone) [PATH:hsa00190]
Cytochrome bc1 complex respiratory unit [PATH:hsa00190]
Cytochrome bc1 complex [PATH:hsa00190]
Cytochrome c oxidase [PATH:hsa00190]
F-type ATPase, eukaryotes [PATH:hsa00190]
V-type ATPase, eukaryotes [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)
H01200  
Fatal infantile cardioencephalomyopathy
H01222  
Cowden-like syndrome
H01347  
MELAS Syndrome
H01354  
Leigh syndrome
H01355  
Kearns-Sayre Syndrome
H01356  
Myoclonic Epilepsy and Ragged-Red Fiber Disease
H01363  
NARP syndrome
H01365  
Leber hereditary optic neuropathy and dystonia (LDYT)
H01368  
Cytochrome c oxidase (COX) deficiency
H01369  
ATP synthase deficiency
Drug
D00355  
Lansoprazole (JP17/USP/INN)
D00455  
Omeprazole (JP17/USP/INN)
D00724  
Sodium rabeprazole (JP17)
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:ubiquinone oxidoreductase core subunit S1 [KO:K03934] [EC:1.6.99.3 1.6.5.3]
4720  
NDUFS2; NADH:ubiquinone oxidoreductase core subunit S2 [KO:K03935] [EC:1.6.99.3 1.6.5.3]
4722  
NDUFS3; NADH:ubiquinone oxidoreductase core subunit S3 [KO:K03936] [EC:1.6.99.3 1.6.5.3]
4724  
NDUFS4; NADH:ubiquinone oxidoreductase subunit S4 [KO:K03937]
4725  
NDUFS5; NADH:ubiquinone oxidoreductase subunit S5 [KO:K03938]
4726  
NDUFS6; NADH:ubiquinone oxidoreductase subunit S6 [KO:K03939]
374291  
NDUFS7; NADH:ubiquinone oxidoreductase core subunit S7 [KO:K03940] [EC:1.6.99.3 1.6.5.3]
4728  
NDUFS8; NADH:ubiquinone oxidoreductase core subunit S8 [KO:K03941] [EC:1.6.99.3 1.6.5.3]
4723  
NDUFV1; NADH:ubiquinone oxidoreductase core subunit V1 [KO:K03942] [EC:1.6.99.3 1.6.5.3]
4729  
NDUFV2; NADH:ubiquinone oxidoreductase core subunit V2 [KO:K03943] [EC:1.6.99.3 1.6.5.3]
4731  
NDUFV3; NADH:ubiquinone oxidoreductase subunit V3 [KO:K03944]
4694  
NDUFA1; NADH:ubiquinone oxidoreductase subunit A1 [KO:K03945]
4695  
NDUFA2; NADH:ubiquinone oxidoreductase subunit A2 [KO:K03946]
4696  
NDUFA3; NADH:ubiquinone oxidoreductase subunit A3 [KO:K03947]
4697  
NDUFA4; NDUFA4, mitochondrial complex associated [KO:K03948]
56901  
NDUFA4L2; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 4-like 2 [KO:K03948]
4698  
NDUFA5; NADH:ubiquinone oxidoreductase subunit A5 [KO:K03949]
4700  
NDUFA6; NADH:ubiquinone oxidoreductase subunit A6 [KO:K03950]
4701  
NDUFA7; NADH:ubiquinone oxidoreductase subunit A7 [KO:K03951]
4702  
NDUFA8; NADH:ubiquinone oxidoreductase subunit A8 [KO:K03952]
4704  
NDUFA9; NADH:ubiquinone oxidoreductase subunit A9 [KO:K03953]
4705  
NDUFA10; NADH:ubiquinone oxidoreductase subunit A10 [KO:K03954]
4706  
NDUFAB1; NADH:ubiquinone oxidoreductase subunit AB1 [KO:K03955]
126328  
NDUFA11; NADH:ubiquinone oxidoreductase subunit A11 [KO:K03956]
55967  
NDUFA12; NADH:ubiquinone oxidoreductase subunit A12 [KO:K11352]
51079  
NDUFA13; NADH:ubiquinone oxidoreductase subunit A13 [KO:K11353]
4707  
NDUFB1; NADH:ubiquinone oxidoreductase subunit B1 [KO:K03957]
4708  
NDUFB2; NADH:ubiquinone oxidoreductase subunit B2 [KO:K03958]
4709  
NDUFB3; NADH:ubiquinone oxidoreductase subunit B3 [KO:K03959]
4710  
NDUFB4; NADH:ubiquinone oxidoreductase subunit B4 [KO:K03960]
4711  
NDUFB5; NADH:ubiquinone oxidoreductase subunit B5 [KO:K03961]
4712  
NDUFB6; NADH:ubiquinone oxidoreductase subunit B6 [KO:K03962]
4713  
NDUFB7; NADH:ubiquinone oxidoreductase subunit B7 [KO:K03963]
4714  
NDUFB8; NADH:ubiquinone oxidoreductase subunit B8 [KO:K03964]
4715  
NDUFB9; NADH:ubiquinone oxidoreductase subunit B9 [KO:K03965]
4716  
NDUFB10; NADH:ubiquinone oxidoreductase subunit B10 [KO:K03966]
54539  
NDUFB11; NADH:ubiquinone oxidoreductase subunit B11 [KO:K11351]
4717  
NDUFC1; NADH:ubiquinone oxidoreductase subunit C1 [KO:K03967]
4718  
NDUFC2; NADH:ubiquinone oxidoreductase subunit C2 [KO:K03968]
NDUFC2-KCTD14; NDUFC2-KCTD14 readthrough [KO:K03968]
6389  
SDHA; succinate dehydrogenase complex flavoprotein subunit A [KO:K00234] [EC:1.3.5.1]
6390  
SDHB; succinate dehydrogenase complex iron sulfur subunit B [KO:K00235] [EC:1.3.5.1]
6391  
SDHC; succinate dehydrogenase complex subunit C [KO:K00236]
6392  
SDHD; succinate dehydrogenase complex subunit D [KO:K00237]
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 c1 [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 [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; COX10 heme A:farnesyltransferase cytochrome c oxidase assembly factor [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 4I2 [KO:K02263]
1327  
COX4I1; cytochrome c oxidase subunit 4I1 [KO:K02263]
9377  
COX5A; cytochrome c oxidase subunit 5A [KO:K02264]
1329  
COX5B; cytochrome c oxidase subunit 5B [KO:K02265]
1337  
COX6A1; cytochrome c oxidase subunit 6A1 [KO:K02266]
1339  
COX6A2; cytochrome c oxidase subunit 6A2 [KO:K02266]
1340  
COX6B1; cytochrome c oxidase subunit 6B1 [KO:K02267]
125965  
COX6B2; cytochrome c oxidase subunit 6B2 [KO:K02267]
1345  
COX6C; cytochrome c oxidase subunit 6C [KO:K02268]
1346  
COX7A1; cytochrome c oxidase subunit 7A1 [KO:K02270]
1347  
COX7A2; cytochrome c oxidase subunit 7A2 [KO:K02270]
9167  
COX7A2L; cytochrome c oxidase subunit 7A2 like [KO:K02270]
1349  
COX7B; cytochrome c oxidase subunit 7B [KO:K02271]
170712  
COX7B2; cytochrome c oxidase subunit 7B2 [KO:K02271]
1350  
COX7C; cytochrome c oxidase subunit 7C [KO:K02272]
341947  
COX8C; cytochrome c oxidase subunit 8C [KO:K02273]
1351  
COX8A; cytochrome c oxidase subunit 8A [KO:K02273]
1353  
COX11; COX11 cytochrome c oxidase copper chaperone [KO:K02258]
1355  
COX15; COX15 cytochrome c oxidase assembly homolog [KO:K02259]
10063  
COX17; COX17 cytochrome c oxidase copper chaperone [KO:K02260]
498  
ATP5A1; ATP synthase, H+ transporting, mitochondrial F1 complex, alpha subunit 1, cardiac muscle [KO:K02132]
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]
513  
ATP5D; ATP synthase, H+ transporting, mitochondrial F1 complex, delta subunit [KO:K02134]
514  
ATP5E; ATP synthase, H+ transporting, mitochondrial F1 complex, epsilon subunit [KO:K02135]
539  
ATP5O; ATP synthase, H+ transporting, mitochondrial F1 complex, O subunit [KO:K02137]
4508  
ATP6; ATP synthase F0 subunit 6 [KO:K02126]
515  
ATP5F1; ATP synthase, H+ transporting, mitochondrial Fo complex subunit B1 [KO:K02127]
516  
ATP5G1; ATP synthase, H+ transporting, mitochondrial Fo complex subunit C1 (subunit 9) [KO:K02128]
517  
ATP5G2; ATP synthase, H+ transporting, mitochondrial Fo complex subunit C2 (subunit 9) [KO:K02128]
518  
ATP5G3; ATP synthase, H+ transporting, mitochondrial Fo complex subunit C3 (subunit 9) [KO:K02128]
10476  
ATP5H; ATP synthase, H+ transporting, mitochondrial Fo complex subunit D [KO:K02138]
521  
ATP5I; ATP synthase, H+ transporting, mitochondrial Fo complex subunit E [KO:K02129]
9551  
ATP5J2; ATP synthase, H+ transporting, mitochondrial Fo complex subunit F2 [KO:K02130]
10632  
ATP5L; ATP synthase, H+ transporting, mitochondrial Fo complex subunit G [KO:K02140]
522  
ATP5J; ATP synthase, H+ transporting, mitochondrial Fo complex subunit F6 [KO:K02131]
4509  
ATP8; ATP synthase F0 subunit 8 [KO:K02125]
523  
ATP6V1A; ATPase H+ transporting V1 subunit A [KO:K02145] [EC:3.6.3.14]
525  
ATP6V1B1; ATPase H+ transporting V1 subunit B1 [KO:K02147]
526  
ATP6V1B2; ATPase H+ transporting V1 subunit B2 [KO:K02147]
245973  
ATP6V1C2; ATPase H+ transporting V1 subunit C2 [KO:K02148]
528  
ATP6V1C1; ATPase H+ transporting V1 subunit C1 [KO:K02148]
51382  
ATP6V1D; ATPase H+ transporting V1 subunit D [KO:K02149]
90423  
ATP6V1E2; ATPase H+ transporting V1 subunit E2 [KO:K02150]
529  
ATP6V1E1; ATPase H+ transporting V1 subunit E1 [KO:K02150]
9296  
ATP6V1F; ATPase H+ transporting V1 subunit F [KO:K02151]
9550  
ATP6V1G1; ATPase H+ transporting V1 subunit G1 [KO:K02152]
127124  
ATP6V1G3; ATPase H+ transporting V1 subunit G3 [KO:K02152]
534  
ATP6V1G2; ATPase H+ transporting V1 subunit G2 [KO:K02152]
51606  
ATP6V1H; ATPase H+ transporting V1 subunit H [KO:K02144]
10312  
TCIRG1; T-cell immune regulator 1, ATPase H+ transporting V0 subunit a3 [KO:K02154]
23545  
ATP6V0A2; ATPase H+ transporting V0 subunit a2 [KO:K02154]
50617  
ATP6V0A4; ATPase H+ transporting V0 subunit a4 [KO:K02154]
535  
ATP6V0A1; ATPase H+ transporting V0 subunit a1 [KO:K02154]
527  
ATP6V0C; ATPase H+ transporting V0 subunit c [KO:K02155]
533  
ATP6V0B; ATPase H+ transporting V0 subunit b [KO:K03661]
9114  
ATP6V0D1; ATPase H+ transporting V0 subunit d1 [KO:K02146]
245972  
ATP6V0D2; ATPase H+ transporting V0 subunit d2 [KO:K02146]
8992  
ATP6V0E1; ATPase H+ transporting V0 subunit e1 [KO:K02153]
155066  
ATP6V0E2; ATPase H+ transporting V0 subunit e2 [KO:K02153]
537  
ATP6AP1; ATPase H+ transporting accessory protein 1 [KO:K03662]
495  
ATP4A; ATPase H+/K+ transporting alpha subunit [KO:K01542] [EC:3.6.3.10]
496  
ATP4B; ATPase H+/K+ transporting beta subunit [KO:K01543] [EC:3.6.3.10]
479  
ATP12A; ATPase H+/K+ transporting non-gastric alpha2 subunit [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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