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COQ2

MIM.609825

Saturday 18 February 2006

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)