MT-CO1 and MT-CO2 complexes associate, installing heme moieties

COX7B + COX7C + MT-CO1:Cu2+:Mg2+:PE:CL:MITRAC:TIMM21:COX4:COX5A:COX6C + MT-CO2:Mg2+:CuA + 3 heme A + COX8A,C → holo-MT-CO1,2 complex + TIMM21

Reactome

R-HSA-9865579

Compartment

mitochondrial inner membrane

Rhea

None recorded

Catalysed by

• copper chaperone activity of metallochaperone complex [mitochondrial inner membrane]

Literature

1. Role of cytochrome c oxidase nuclear-encoded subunits in health and disease Physiol Res, 2020
2. Cooperation between COA6 and SCO2 in COX2 maturation during cytochrome c oxidase assembly links two mitochondrial cardiomyopathies Cell Metab., 2015
3. SURF1, encoding a factor involved in the biogenesis of cytochrome c oxidase, is mutated in Leigh syndrome Nat Genet, 1998
4. Assembly of cytochrome-c oxidase in the absence of assembly protein Surf1p leads to loss of the active site heme J Biol Chem, 2005
5. Surf1, associated with Leigh syndrome in humans, is a heme-binding protein in bacterial oxidase biogenesis J Biol Chem, 2009
6. Biosynthesis and trafficking of heme o and heme a : new structural insights and their implications for reaction mechanisms and prenylated heme transfer Crit Rev Biochem Mol Biol, 2021