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Proteins/UNIPROT:O00483
Component of the cytochrome c oxidase, the last enzyme in the mitochondrial electron transport chain which drives oxidative phosphorylation. The respiratory chain contains 3 multisubunit complexes succinate dehydrogenase (complex II, CII), ubiquinol-cytochrome c oxidoreductase (cytochrome b-c1 complex, complex III, CIII) and cytochrome c oxidase (complex IV, CIV), that cooperate to transfer electrons derived from NADH and succinate to molecular oxygen, creating an electrochemical gradient over the inner membrane that drives transmembrane transport and the ATP synthase. Cytochrome c oxidase is the component of the respiratory chain that catalyzes the reduction of oxygen to water. Electrons originating from reduced cytochrome c in the intermembrane space (IMS) are transferred via the dinuclear copper A center (CU(A)) of subunit 2 and heme A of subunit 1 to the active site in subunit 1, a binuclear center (BNC) formed by heme A3 and copper B (CU(B)). The BNC reduces molecular oxygen to 2 water molecules unsing 4 electrons from cytochrome c in the IMS and 4 protons from the mitochondrial matrix. COXFA4 is required for complex IV maintenance..
Function from UniProtKB (O00483), read 2026-09-23. CC BY 4.0.
Curated compounds routed: 0
3 rows
76 statements
76 statements · 4 reference databases · Subject: COXFA4 · Sources: Hetionet, Human-GEM, Reactome, WikiPathways
4 reactions
5 names
Resolves to: COXFA4
Reaction pageLeft to rightBalancedEC 7.1.1.2cytosol, mitochondrial matrix, mitochondrial intermembrane space
Human-GEM MAR06500CC BY 4.0Reference data
4ferrocytochrome c+8proton+dioxygen4ferricytochrome c+4proton+2water
Transported by COXFA4 & MT-CO1 & MT-CO2 & MT-CO3 & COX6C & COX8A & COX5B & COX6A1 & COX4I1 & COX6B1 & COX7C & COX5A & COX7A1 & COX7B & COX7B2 or COXFA4 & MT-CO1 & MT-CO2 & MT-CO3 & COX6C & COX8A & COX5B & COX6A1 & COX4I1 & COX7A2 & COX6B1 & COX7C & COX5A & COX7B & COX7B2 or COXFA4 & MT-CO1 & MT-CO2 & MT-CO3 & COX6C & COX8A & COX5B & COX6A1 & COX4I1 & COX7C & COX5A & COX7A1 & COX7B & COX6B2 & COX7B2 or COXFA4 & MT-CO1 & MT-CO2 & MT-CO3 & COX6C & COX8A & COX5B & COX6A1 & COX4I1 & COX7A2 & COX7C & COX5A & COX7B & COX6B2 & COX7B2 or COXFA4 & MT-CO1 & MT-CO2 & MT-CO3 & COX6C & COX8A & COX5B & COX4I1 & COX6B1 & COX7C & COX5A & COX7A1 & COX7B & COX6A2 & COX7B2 or COXFA4 & MT-CO1 & MT-CO2 & MT-CO3 & COX6C & COX8A & COX5B & COX4I1 & COX7A2 & COX6B1 & COX7C & COX5A & COX7B & COX6A2 & COX7B2 or COXFA4 & MT-CO1 & MT-CO2 & MT-CO3 & COX6C & COX8A & COX5B & COX4I1 & COX7C & COX5A & COX7A1 & COX7B & COX6A2 & COX6B2 & COX7B2 or COXFA4 & MT-CO1 & MT-CO2 & MT-CO3 & COX6C & COX8A & COX5B & COX4I1 & COX7A2 & COX7C & COX5A & COX7B & COX6A2 & COX6B2 & COX7B2 or COXFA4 & MT-CO1 & MT-CO2 & MT-CO3 & COX6C & COX8A & COX5B & COX6A1 & COX6B1 & COX7C & COX5A & COX7A1 & COX7B & COX7B2 & COX4I2 or COXFA4 & MT-CO1 & MT-CO2 & MT-CO3 & COX6C & COX8A & COX5B & COX6A1 & COX7A2 & COX6B1 & COX7C & COX5A & COX7B & COX7B2 & COX4I2 or COXFA4 & MT-CO1 & MT-CO2 & MT-CO3 & COX6C & COX8A & COX5B & COX6A1 & COX7C & COX5A & COX7A1 & COX7B & COX6B2 & COX7B2 & COX4I2 or COXFA4 & MT-CO1 & MT-CO2 & MT-CO3 & COX6C & COX8A & COX5B & COX6A1 & COX7A2 & COX7C & COX5A & COX7B & COX6B2 & COX7B2 & COX4I2 and 4 more alternatives
Reaction pageLeft to rightBalancedEC 7.1.1.9mitochondrial matrix, mitochondrial intermembrane space
Human-GEM MAR06914CC BY 4.0Reference data
5proton+NADH(2-)+ubiquinones4proton+NAD(+)+ubiquinol
Transported by NDUFS8 & COXFA4 & NDUFAB1 & NDUFA1 & NDUFS4 & NDUFB5 & NDUFB3 & NDUFC1 & NDUFA2 & NDUFS5 & NDUFS7 & NDUFS2 & NDUFS6 & NDUFB1 & NDUFS3 & NDUFB6 & NDUFA3 & NDUFB4 & NDUFB8 & NDUFB2 & NDUFA7 & NDUFC2 & NDUFA10 & NDUFB10 & MT-ND1 & MT-ND2 & MT-ND3 & MT-ND4L & MT-ND4 & MT-ND5 & MT-ND6 & NDUFB7 & NDUFV2 & NDUFS1 & NDUFV1 & NDUFA8 & NDUFV3 & NDUFA6 & NDUFA5 & NDUFA9 & NDUFA11 & NDUFB11 & NDUFA13 & NDUFA12 & NDUFB9
Reaction pageLeft to rightBalancedEC 7.1.1.2mitochondrial matrix, mitochondrial intermembrane space
Human-GEM MAR06921CC BY 4.0Reference data
4ferrocytochrome c+7.92proton+dioxygen4ferricytochrome c+4proton+1.96water+0.02superoxide
Transported by COXFA4 & MT-CO1 & MT-CO2 & MT-CO3 & COX6C & COX8A & COX5B & COX6A1 & COX4I1 & COX6B1 & COX7C & COX5A & COX7A1 & COX7B & COX7B2 or COXFA4 & MT-CO1 & MT-CO2 & MT-CO3 & COX6C & COX8A & COX5B & COX6A1 & COX4I1 & COX7A2 & COX6B1 & COX7C & COX5A & COX7B & COX7B2 or COXFA4 & MT-CO1 & MT-CO2 & MT-CO3 & COX6C & COX8A & COX5B & COX6A1 & COX4I1 & COX7C & COX5A & COX7A1 & COX7B & COX6B2 & COX7B2 or COXFA4 & MT-CO1 & MT-CO2 & MT-CO3 & COX6C & COX8A & COX5B & COX6A1 & COX4I1 & COX7A2 & COX7C & COX5A & COX7B & COX6B2 & COX7B2 or COXFA4 & MT-CO1 & MT-CO2 & MT-CO3 & COX6C & COX8A & COX5B & COX4I1 & COX6B1 & COX7C & COX5A & COX7A1 & COX7B & COX6A2 & COX7B2 or COXFA4 & MT-CO1 & MT-CO2 & MT-CO3 & COX6C & COX8A & COX5B & COX4I1 & COX7A2 & COX6B1 & COX7C & COX5A & COX7B & COX6A2 & COX7B2 or COXFA4 & MT-CO1 & MT-CO2 & MT-CO3 & COX6C & COX8A & COX5B & COX4I1 & COX7C & COX5A & COX7A1 & COX7B & COX6A2 & COX6B2 & COX7B2 or COXFA4 & MT-CO1 & MT-CO2 & MT-CO3 & COX6C & COX8A & COX5B & COX4I1 & COX7A2 & COX7C & COX5A & COX7B & COX6A2 & COX6B2 & COX7B2 or COXFA4 & MT-CO1 & MT-CO2 & MT-CO3 & COX6C & COX8A & COX5B & COX6A1 & COX6B1 & COX7C & COX5A & COX7A1 & COX7B & COX7B2 & COX4I2 or COXFA4 & MT-CO1 & MT-CO2 & MT-CO3 & COX6C & COX8A & COX5B & COX6A1 & COX7A2 & COX6B1 & COX7C & COX5A & COX7B & COX7B2 & COX4I2 or COXFA4 & MT-CO1 & MT-CO2 & MT-CO3 & COX6C & COX8A & COX5B & COX6A1 & COX7C & COX5A & COX7A1 & COX7B & COX6B2 & COX7B2 & COX4I2 or COXFA4 & MT-CO1 & MT-CO2 & MT-CO3 & COX6C & COX8A & COX5B & COX6A1 & COX7A2 & COX7C & COX5A & COX7B & COX6B2 & COX7B2 & COX4I2 and 4 more alternatives
Reaction pageBlockedUnbalancedEC 7.1.1.9mitochondrial matrix, mitochondrial intermembrane space
Human-GEM MAR13081CC BY 4.0Reference data