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Target intelligence / Profile preview
Cytochrome c oxidase copper chaperone COX11 (COX11)
- Target
- COX11
- Molecular classification
- Enzyme, Mitochondrial protein, Copper chaperone, Membrane protein
01
Overview
Cytochrome c oxidase copper chaperone COX11 is an essential mitochondrial inner membrane protein involved in the terminal assembly of the cytochrome c oxidase (Complex IV) of the mitochondrial respiratory chain. While not a structural subunit, COX11 facilitates the insertion of copper into the CuB site of subunit I, enabling proper enzymatic activity and electron transfer from cytochrome c to oxygen. Disease associations include mitochondrial complex IV deficiency, with potential clinical manifestations like epilepsy[1][2][3][5][6].
- Other names
- Cytochrome c oxidase assembly protein COX11, mitochondrialCOX11PCytochrome c oxidase subunit 11MC4DN23COX11 homologCytochrome c oxidase copper chaperoneCytochrome c oxidase assembly protein
02
Biological functions
Assembly of cytochrome c oxidase complexCopper ion binding and insertionMitochondrial respiratory chainElectron transfer
03
Disease associations
Mitochondrial complex IV deficiencyEpilepsy
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