COX15 transforms heme O to heme A
2 hydrogen acceptor + H2O + heme O → 2 hydrogen donor + heme A
- Reactome
- R-HSA-2995334
- Compartment
- mitochondrial matrix · mitochondrial inner membrane
- Rhea
- RHEA:63388
Inputs
- 2 hydrogen acceptorCHEBI:13193, A, mitochondrial matrix
- H2OCHEBI:15377, H2O, mitochondrial matrix
- heme OCHEBI:60530, C49H56FeN4O5, mitochondrial inner membrane
Outputs
- 2 hydrogen donorCHEBI:17499, H2A, mitochondrial matrix
- heme ACHEBI:61715, C49H54FeN4O6, mitochondrial inner membrane
Catalysed by
- heme A synthase activity of COX15 [mitochondrial inner membrane]EC 1.17.99.9; UniProt Q7KZN9
Literature
- Mutations in COX15 produce a defect in the mitochondrial heme biosynthetic pathway, causing early-onset fatal hypertrophic cardiomyopathy Am. J. Hum. Genet., 2003
- Novel mutations in COX15 in a long surviving Leigh syndrome patient with cytochrome c oxidase deficiency J. Med. Genet., 2005
- Functional and genetic studies demonstrate that mutation in the COX15 gene can cause Leigh syndrome J. Med. Genet., 2004
- Identification and characterization of human cDNAs specific to BCS1, PET112, SCO1, COX15, and COX11, five genes involved in the formation and function of the mitochondrial respiratory chain Genomics, 1998
- 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