Recombinant Human Cytochrome c oxidase subunit 3 (MT-CO3), Truncated

Uniprot Id

P00414

Target Species

Human

Target Name

MT-CO3

Target Full Name

Cytochrome c oxidase subunit 3

Target 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 using 4 electrons from cytochrome c in the IMS and 4 protons from the mitochondrial matrix

Target Involvement

Leber hereditary optic neuropathy (LHON); Mitochondrial complex IV deficiency (MT-C4D); Recurrent myoglobinuria mitochondrial (RM-MT)

Target Subcellular Location

Mitochondrion inner membrane; Multi-pass membrane protein.

Target Protein Families

Cytochrome c oxidase subunit 3 family

Target Synonyms

MT-CO3; COIII; COXIII; MTCO3; Cytochrome c oxidase subunit 3; Cytochrome c oxidase polypeptide III