COX10

COX10 also known as Cytochrome c oxidase assembly factor heme A:farnesyltransferase COX10 is a protein that in humans is encoded by the COX10 gene. It is involved in the final stages of the mitochondrial electron transport chain, specifically in the synthesis of heme A, a component of cytochrome c oxidase (COX).

Function

COX10 is a mitochondrial protein involved in the biosynthesis of heme A, a prosthetic group of cytochrome c oxidase (COX). COX is the terminal enzyme of the mitochondrial respiratory chain and is responsible for the transfer of electrons from cytochrome c to oxygen. COX10 catalyzes the conversion of protoheme IX (heme B) to heme O, the penultimate step in the biosynthesis of heme A.

Clinical significance

Mutations in the COX10 gene have been associated with mitochondrial complex IV deficiency, a disorder of the mitochondrial respiratory chain with heterogeneous clinical manifestations, ranging from isolated myopathy to severe multisystem disease affecting several tissues and organs.

Structure

The COX10 protein is a member of the heme A:farnesyltransferase family. It is a multi-pass membrane protein that is located in the inner mitochondrial membrane. The protein is predicted to have 8 transmembrane domains.

See also

• Cytochrome c oxidase
• Mitochondrial complex IV deficiency
• Myopathy