Para-hydroxybenzoate--polyprenyltransferase, mitochondrial (COQ2)

Para-hydroxybenzoate--polyprenyltransferase, mitochondrial (COQ2), is a mitochondrial enzyme that catalyzes the prenylation of para-hydroxybenzoate with an all-trans polyprenyl group, a key step in the biosynthesis of coenzyme Q10 (ubiquinone), a critical redox carrier in the respiratory chain and a lipid-soluble antioxidant. COQ2 function is essential for mitochondrial ATP production and cellular protection against oxidative damage. Mutations in COQ2 are responsible for primary CoQ10 deficiency syndromes, which can manifest as encephalomyopathy, nephropathy, myopathy, or multisystem disorders, and are implicated as genetic risk factors for multiple system atrophy, a progressive neurodegenerative disease[1][2][3][4][5]. Deficiency of coenzyme Q10 due to COQ2 malfunction impairs oxidative phosphorylation and increases vulnerability to free radical-induced cellular damage. Coenzyme Q10 supplementation is the main therapy for affected patients, although efficacy may vary, and the enzyme itself is not currently targeted with direct pharmacological modulators.

Other names

4-HB polyprenyltransferase4-hydroxybenzoate decaprenyltransferase4-hydroxybenzoate polyprenyltransferase, mitochondrialCL640Coenzyme Q2 4-hydroxybenzoate polyprenyltransferaseCoenzyme Q2 homolog, prenyltransferaseCOQ10D1FLJ26072MSA1Para-hydroxybenzoate-polyprenyltransferase, mitochondrialPHB:polyprenyltransferasePHB:PPT[2][3]

Coenzyme Q10 supplementation restores deficient electron transport and antioxidant activity due to loss-of-function mutations or variants in COQ2[2][1][3]