lmmol · Proteins

Cytochrome c oxidase subunit FA4

UniProt O00483 Organism Homo sapiens Gene COXFA4, NDUFA4

Also known as: COXFA4, NDUFA4

Function

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.

Classification

Family (Pfam)
PF06522 B12D
InterPro
B12D
Functional cluster
Photosystem II Reaction-Center & Antimicrobial Peptides

Experimental structures · PDB · 2

A predicted model is available from AlphaFold.

Gene Ontology · 10

Disease associations

Drugs targeting this protein · 2

Related proteins · sequence + function similarity

Co-cited proteins · studied together in the literature

Literature · 12 cited papers

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