Protohaem IX farnesyltransferase, mitochondria (IPR016315)

Short name: Protohaem_IX_farnesylTrfase_mt

Overlapping homologous superfamilies


Family relationships


This entry represents protohaem IX (haem A) farnesyltransferase (EC:2.5.1), the product of the COX10 gene. This enzyme occurs in the mitochondrial membrane and catalyses the first step in the conversion of protohaem to the haem A prosthetic groups of the enzyme, namely the conversion of protohaem IX and farnesyl diphosphate to haem O. Haem O and/or haem A are specifically synthesised for the haem-copper respiratory oxidases [PMID: 7885224]. Deficiencies in COX10 are a cause of Leigh syndrome (LS) (OMIM:256000), which is a severe neurological disorder characterised by bilaterally symmetrical necrotic lesions in subcortical brain regions [PMID: 12928484].

GO terms

Biological Process

GO:0006783 heme biosynthetic process

Molecular Function

GO:0008495 protoheme IX farnesyltransferase activity

Cellular Component

GO:0031966 mitochondrial membrane

Contributing signatures

Signatures from InterPro member databases are used to construct an entry.