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COX17

Function

Copper metallochaperone essential for the assembly of the mitochondrial respiratory chain complex IV (CIV), also known as cytochrome c oxidase. Binds two copper ions and delivers them to the metallochaperone SCO1 which transports the copper ions to the Cu(A) site on the cytochrome c oxidase subunit II (MT-CO2/COX2).

Post-translational modifications

Acetylation by KAT8 promotes assembly of the mitochondrial respiratory chain complex IV (CIV).

Sequence Similarities

Belongs to the COX17 family.

Tissue Specificity

Ubiquitous.

Cellular localization

Alternative names

Cytochrome c oxidase copper chaperone, COX17

swissprot:Q14061 entrezGene:10063 omim:604813