Heme A synthase COX15 (COX15)

Heme A synthase COX15 is an enzyme localized to the mitochondrial inner membrane that catalyzes the key final step in the biosynthesis of heme A from heme O via two successive hydroxylations of the methyl group at position C8. Heme A is an essential prosthetic group for cytochrome c oxidase (complex IV), the terminal electron acceptor in the mitochondrial respiratory chain, which is responsible for the transfer of electrons from cytochrome c to oxygen, driving oxidative phosphorylation. COX15 is indispensable for cytochrome c oxidase assembly; mutations or loss can cause severe mitochondrial disorders, including fatal infantile myopathy. COX15 has homologs in many eukaryotes and forms transient complexes with other assembly factors during the maturation and stabilization of respiratory complex IV[1][2][3].

Other names

Cytochrome c oxidase assembly protein COX15 homologHeme A synthase COX15COX15 homologCEMCOX2HASMC4DN6Cytochrome c oxidase assembly proteinCytochrome c oxidase assembly homolog 15Cytochrome c oxidase subunit 15

Not applicable—COX15 is an essential enzyme for heme A synthesis; no drugs are known to target COX15 directly for therapeutic purposes.