Official Full Name
coenzyme Q2 4-hydroxybenzoate polyprenyltransferase
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.
COQ2; coenzyme Q2 homolog, prenyltransferase (yeast); 4-hydroxybenzoate polyprenyltransferase, mitochondrial; 4 hydroxybenzoate polyprenyltransferase; CL640; FLJ26072; MGC91278; 2310002F18Rik; FLJ13014; hCOQ2; MGC124824; OTTMUSP00000032111; Para-hydroxybenzoate--polyprenyltransferase, mitochondrial; PHB:polyprenyltransferase; RGD1306722; COQ2 homolog; para-hydroxybenzoate-polyprenyltransferase, mitochondrial; coenzyme Q2 4-hydroxybenzoate polyprenyltransferase; zgc:162600; coenzyme Q2 homolog, prenyltransferase