{"symbol":"COQ2","synonyms":["CL640","MSA1","PHB:PPT","COQ10D1"],"name":"coenzyme Q2 4-hydroxybenzoate polyprenyltransferase","description":"This gene encodes an enzyme that functions in the final steps in the biosynthesis of CoQ (ubiquinone), a redox carrier in the mitochondrial respiratory chain and a lipid-soluble antioxidant. This enzyme, which is part of the coenzyme Q10 pathway, catalyzes the prenylation of parahydroxybenzoate with an all-trans polyprenyl group. Mutations in this gene cause coenzyme Q10 deficiency, a mitochondrial encephalomyopathy, and also COQ2 nephropathy, an inherited form of mitochondriopathy with primary renal involvement. [provided by RefSeq, Oct 2009]","ncbiEntrezGeneId":27235,"ncbiEntrezGeneUrl":"http://www.ncbi.nlm.nih.gov/gene/27235","proteins":[{"symbol":"COQ2_HUMAN","href":"/api/1.0/protein/COQ2_HUMAN"}],"hgncRootFamilies":[]}