Cytochrome C Oxidase Assembly Protein 10 (COX10)

The COX10 gene encodes a cytochrome c oxidase (COX) assembly protein involved in the mitochondrial heme biosynthetic pathway. COX10 catalyzes the farnesylation of a vinyl group at position C2, resulting in the conversion of protoheme (heme B) to heme O. The COX10 protein is required for the expression of functional COX.the putative promoter region of COX10 lacks conventional TATA and CAAT boxes, but does have several potential transcription factor binding sites. Northern blot analysis showed that the gene was transcribed as a 3-kb mRNA in all tissues examined, with highest levels observed in heart, skeletal muscle, and testis. COX10 gene plays an important role in the farnesylation of protoheme, a prosthetic group of the cytochrome oxidase complex, and is a candidate gene in cytochrome oxidase deficiencies.