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Cytochrome c oxidase (COX) is the terminal enzyme in the electron transfer chain, functioning as a transmembrane proton pump that builds an electrochemical gradient with chemical energy from the reduction of O2. Cytochrome c oxidase assembly protein COX11 is an intracellular mitochondrial membrane protein necessary for the construction of an active COX complex. COX11 contains a single transmembrane helix downstream of the N-terminal, mitochondrial targeting sequence and a C-terminal Cu(I)-binding domain. The assembly of COX requires the delivery of metal cofactors. Along with COX12 and SCO1/2, COX11 acts as a metal ion chaperone necessary for copper insertion into CuA and CuB redox-active copper centers of COX in eukaryotes.
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Protein Aliases: COX11 cytochrome c oxidase copper chaperone; COX11 homolog, cytochrome c oxidase assembly protein; Cytochrome c oxidase assembly protein COX11, mitochondrial; cytochrome c oxidase subunit 11
Gene Aliases: 2010004I09Rik; COX11; COX11P
Molecular Function: chaperone