Cytochrome c oxidase assembly protein COX16 homolog, mitochondrial (COX16)

Cytochrome c oxidase assembly protein COX16 homolog, mitochondrial (COX16), is a conserved protein located in the mitochondrial inner membrane that is essential for the biogenesis and assembly of cytochrome c oxidase (complex IV of the mitochondrial respiratory chain)[2][4][5]. COX16 specifically interacts with newly synthesized COX2 (cytochrome c oxidase subunit II) and copper chaperones such as SCO1, SCO2, and COA6 to facilitate incorporation of copper into the CuA center of COX2—a critical step for enzyme activity[1][2][4]. COX16 also supports the association of COX2 with assembly intermediates containing COX1 (subunit I), effectively merging the COX1 and COX2 assembly lines during mitochondrial complex IV maturation[1]. Mutations or loss of COX16 disrupt cytochrome c oxidase assembly, leading to reduced complex IV activity and mitochondrial dysfunction, with clinical associations including various forms of mitochondrial complex IV deficiency[2]. COX16 is not considered a typical therapeutic target, such as a receptor or enzyme targeted by drugs, but instead acts as an essential chaperone-like assembly factor for mitochondrial function[2][4][1].

Other names

C14orf112HSPC203PTD019hCOX16MC4DN22cytochrome c oxidase assembly factor COX16COX16 homologcytochrome c oxidase assembly homolog