COQ2


Para-hydroxybenzoate—polyprenyltransferase, mitochondrial is an enzyme that in humans is encoded by the COQ2 gene.
CoQ serves as a redox carrier in the mitochondrial respiratory chain and is a lipid-soluble antioxidant. COQ2, or parahydroxybenzoate-polyprenyltransferase, catalyzes one of the final reactions in the biosynthesis of CoQ, the prenylation of parahydroxybenzoate with an all-trans polyprenyl group.

Role in pathology

Homozygous or compound heterozygous mutations of the COQ2 gene cause primary coenzyme Q10 deficiency 1, a mitochondrial disease.