COQ2
MIM.609825
CoQ (ubiquinone) 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.
Pathology
coenzyme Q10 deficiency (MIM.607426)