KEGG   PATHWAY: hsa00190Help
Entry
hsa00190                    Pathway                                

Name
Oxidative phosphorylation - Homo sapiens (human)
Class
09100 Metabolism; 09102 Energy metabolism
BRITE hierarchy
Pathway map
hsa00190  Oxidative phosphorylation
hsa00190

Ortholog table
Module
hsa_M00142  NADH:ubiquinone oxidoreductase, mitochondria [PATH:hsa00190]
hsa_M00143  NADH dehydrogenase (ubiquinone) Fe-S protein/flavoprotein complex, mitochondria [PATH:hsa00190]
hsa_M00146  NADH dehydrogenase (ubiquinone) 1 alpha subcomplex [PATH:hsa00190]
hsa_M00147  NADH dehydrogenase (ubiquinone) 1 beta subcomplex [PATH:hsa00190]
hsa_M00148  Succinate dehydrogenase (ubiquinone) [PATH:hsa00190]
hsa_M00151  Cytochrome bc1 complex respiratory unit [PATH:hsa00190]
hsa_M00152  Cytochrome bc1 complex [PATH:hsa00190]
hsa_M00154  Cytochrome c oxidase [PATH:hsa00190]
hsa_M00158  F-type ATPase, eukaryotes [PATH:hsa00190]
hsa_M00160  V-type ATPase, eukaryotes [PATH:hsa00190]
Disease
H00068  Leber hereditary optic atrophy (LHON)
H00473  Mitochondrial complex I deficiency
H00920  Exocrine pancreatic insufficiency, dyserythropoietic anemia, and calvarial hyperostosis
H01177  Infantile bilateral striatal necrosis (IBSN)
H01200  Fatal infantile cardioencephalomyopathy
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
H01904  Microphthalmia with linear skin defects (MLS) syndrome
H02005  Mitochondrial complex II deficiency
H02086  Mitochondrial complex III deficiency
Drug
D07917  Esomeprazole (INN)
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; NDUFA4, mitochondrial complex associated 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]
100532726  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 1 [KO:K00414]
7385  UQCRC2; ubiquinol-cytochrome c reductase core protein 2 [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:2.5.1.141]
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  ATP5F1A; ATP synthase F1 subunit alpha [KO:K02132]
506  ATP5F1B; ATP synthase F1 subunit beta [KO:K02133] [EC:3.6.3.14]
509  ATP5F1C; ATP synthase F1 subunit gamma [KO:K02136]
513  ATP5F1D; ATP synthase F1 subunit delta [KO:K02134]
514  ATP5F1E; ATP synthase F1 subunit epsilon [KO:K02135]
539  ATP5PO; ATP synthase peripheral stalk subunit OSCP [KO:K02137]
4508  ATP6; ATP synthase F0 subunit 6 [KO:K02126]
515  ATP5PB; ATP synthase peripheral stalk-membrane subunit b [KO:K02127]
516  ATP5MC1; ATP synthase membrane subunit c locus 1 [KO:K02128]
517  ATP5MC2; ATP synthase membrane subunit c locus 2 [KO:K02128]
518  ATP5MC3; ATP synthase membrane subunit c locus 3 [KO:K02128]
10476  ATP5PD; ATP synthase peripheral stalk subunit d [KO:K02138]
521  ATP5ME; ATP synthase membrane subunit e [KO:K02129]
9551  ATP5MF; ATP synthase membrane subunit f [KO:K02130]
10632  ATP5MG; ATP synthase membrane subunit g [KO:K02140]
522  ATP5PF; ATP synthase peripheral stalk 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 subunit alpha [KO:K01542] [EC:3.6.3.10]
496  ATP4B; ATPase H+/K+ transporting subunit beta [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)
DOI:10.1126/science.1123809
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)
DOI:10.1021/bi0600998
KO pathway
ko00190   

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