Electron transport chain: OXPHOS system in mitochondria (WP111)

Homo sapiens

An electron transport chain(ETC) couples a chemical reaction between an electron donor (such as NADH) and an electron acceptor (such as O2) to the transfer of H+ ions across a membrane, through a set of mediating biochemical reactions. These H+ ions are used to produce adenosine triphosphate (ATP), the main energy intermediate in living organisms, as they move back across the membrane. In mitochondria, it is the conversion of oxygen to water, NADH to NAD+ and succinate to fumarate that drives the transfer of H+ ions. Source: Wikipedia ([[wikipedia:Electron_transport_chain]]) Proteins on this pathway have targeted assays available via the [https://assays.cancer.gov/available_assays?wp_id=WP111 CPTAC Assay Portal]


Kdahlquist , Alex Pico , Kristina Hanspers , Ferry Jagers , Christine Chichester , Egon Willighagen , Martina Summer-Kutmon , Denise Slenter , and Eric Weitz


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Homo sapiens



Pathway Ontology

electron transport chain pathway classic metabolic pathway


Label Type Compact URI Comment
O2 Metabolite hmdb:HMDB0001377
H+ Metabolite chebi:15378
ATP Metabolite kegg.compound:C00002
Succinate Metabolite kegg.compound:C00042
e- Metabolite kegg.compound:C05359
FADH2 Metabolite kegg.compound:C01352
H+ Metabolite chebi:15378
H+ Metabolite chebi:15378
FAD Metabolite kegg.compound:C00016
H+ Metabolite chebi:15378
H+ Metabolite chebi:15378
NAD+ Metabolite kegg.compound:C00003
H2O Metabolite hmdb:HMDB0002111
H+ Metabolite chebi:15378
H+ Metabolite chebi:15378
e- Metabolite kegg.compound:C05359
e- Metabolite kegg.compound:C05359
Cytochrome C Metabolite kegg.compound:C00524
NADH Metabolite kegg.compound:C00004
Ubiquinone Metabolite hmdb:HMDB0002012
ATP5A1 GeneProduct ncbigene:498
NDUFB6 GeneProduct ncbigene:4712
ATP5I GeneProduct ncbigene:521
SLC25A5 GeneProduct ncbigene:292
COX15 GeneProduct ncbigene:1355
COX6B1 GeneProduct ncbigene:1340
UCRC GeneProduct ncbigene:29796
COX6C GeneProduct ncbigene:1345
ATP5J2 GeneProduct ncbigene:9551
ATP5O GeneProduct ncbigene:539
ATP5G2 GeneProduct ncbigene:517
NDUFC1 GeneProduct ncbigene:4717
ATP5B GeneProduct ncbigene:506
NDUFA8 GeneProduct ncbigene:4702
UQCR GeneProduct ncbigene:10975
NDUFA3 GeneProduct ncbigene:4696
SLC25A14 GeneProduct ncbigene:9016
NDUFV2 GeneProduct ncbigene:4729
NDUFA6 GeneProduct ncbigene:4700
COX2 GeneProduct ncbigene:4513
NDUFB9 GeneProduct ncbigene:4715
NDUFS6 GeneProduct ncbigene:4726
UQCRFS1 GeneProduct ncbigene:7386
NDUFA2 GeneProduct ncbigene:4695
ND4L GeneProduct ncbigene:4539
ATP8 GeneProduct ncbigene:4509
NDUFS5 GeneProduct ncbigene:4725
COX5A GeneProduct ncbigene:9377
UQCRC2 GeneProduct ncbigene:7385
COX11 GeneProduct ncbigene:1353
ATP5J GeneProduct ncbigene:522
SDHB GeneProduct ncbigene:6390
COX1 GeneProduct ncbigene:4512
COX7A2L GeneProduct ncbigene:9167
NDUFS7 GeneProduct ncbigene:374291
COX6A1 GeneProduct ncbigene:1337
SLC25A27 GeneProduct ncbigene:9481
NDUFS4 GeneProduct ncbigene:4724
COX7A1 GeneProduct ncbigene:1346
COX4I1 GeneProduct ncbigene:1327
COX7A3 GeneProduct uniprot:O60397
SLC25A6 GeneProduct ncbigene:293
ATP5L GeneProduct ncbigene:10632
NDUFB1 GeneProduct ncbigene:4707
UCP3 GeneProduct ncbigene:7352
UQCRH GeneProduct ncbigene:7388
NDUFA9 GeneProduct ncbigene:4704
NDUFB3 GeneProduct ncbigene:4709
ATP5G1 GeneProduct ncbigene:516
NDUFB4 GeneProduct ncbigene:4710
COX7A2 GeneProduct ncbigene:1347
ND6 GeneProduct ncbigene:4541
ATP5S GeneProduct ncbigene:27109
SDHA GeneProduct ncbigene:6389
ND2 GeneProduct ncbigene:4536
NDUFAB1 GeneProduct ncbigene:4706
COX7B GeneProduct ncbigene:1349
QP-C GeneProduct ncbigene:27089
SLC25A4 GeneProduct ncbigene:291
UQCRB GeneProduct ncbigene:7381
ND5 GeneProduct ncbigene:4540
DAP13 GeneProduct ncbigene:55967
SCO1 GeneProduct ncbigene:6341
SDHD GeneProduct ncbigene:6392
ATPIF1 GeneProduct ncbigene:93974
NDUFB7 GeneProduct ncbigene:4713
NDUFA1 GeneProduct ncbigene:4694
UQCRC1 GeneProduct ncbigene:7384
NDUFB5 GeneProduct ncbigene:4711
COX5B GeneProduct ncbigene:1329
ATP6 GeneProduct ncbigene:4508
COX3 GeneProduct ncbigene:4514
NDUFS3 GeneProduct ncbigene:4722
COX8A GeneProduct ncbigene:1351
ND1 GeneProduct ncbigene:4535
NDUFV1 GeneProduct ncbigene:4723
SDHC GeneProduct ncbigene:6391
ATP5G3 GeneProduct ncbigene:518
COX6A2 GeneProduct ncbigene:1339
SURF1 GeneProduct ncbigene:6834
NDUFS1 GeneProduct ncbigene:4719
NDUFA7 GeneProduct ncbigene:4701
ND4 GeneProduct ncbigene:4538
ATP5H GeneProduct ncbigene:10476
NDUFC2 GeneProduct ncbigene:4718
NDUFB2 GeneProduct ncbigene:4708
NDUFS2 GeneProduct ncbigene:4720
COX7C GeneProduct ncbigene:1350
ATP5D GeneProduct ncbigene:513
NDUFB10 GeneProduct ncbigene:4716
COX17 GeneProduct ncbigene:10063
NDUFA4 GeneProduct ncbigene:4697
NDUFA5 GeneProduct ncbigene:4698
ATP5F1 GeneProduct ncbigene:515
ATP5E GeneProduct ncbigene:514
ATP5C1 GeneProduct ncbigene:509
NDUFV3 GeneProduct ncbigene:4731
NDUFA10 GeneProduct ncbigene:4705
UCP2 GeneProduct ncbigene:7351
ND3 GeneProduct ncbigene:4537
NDUFS8 GeneProduct ncbigene:4728
NDUFB8 GeneProduct ncbigene:4714
CYTB GeneProduct ncbigene:4519
UCP1 GeneProduct ncbigene:7350
NDUFA11 GeneProduct ncbigene:126328
NDUFA13 GeneProduct hgnc.symbol:NDUFA13


  1. NDUFA4 is a subunit of complex IV of the mammalian electron transport chain. Balsa E, Marco R, Perales-Clemente E, Szklarczyk R, Calvo E, Landázuri MO, et al. Cell Metab. 2012 Sep 5;16(3):378–86. PubMed Europe PMC Scholia
  2. NDUFA4 mutations underlie dysfunction of a cytochrome c oxidase subunit linked to human neurological disease. Pitceathly RDS, Rahman S, Wedatilake Y, Polke JM, Cirak S, Foley AR, et al. Cell Rep. 2013 Jun 27;3(6):1795–805. PubMed Europe PMC Scholia
  3. Assembly of mammalian oxidative phosphorylation complexes I-V and supercomplexes. Signes A, Fernandez-Vizarra E. Essays Biochem. 2018 Jul 20;62(3):255–70. PubMed Europe PMC Scholia