p-hydroxybenzoate-polyprenyltransferase, mitochondrial (PHB:polyprenyltransferase, hCOQ2, COQ2, CL640)

Function

• Coenzyme Q (CoQ) biosynthesis, 2nd step
• catalyzes prenylation of p-hydroxybenzoate with an all-trans polyprenyl group
• mediates 2nd step in final reaction sequence of CoQ biosynthesis, condensation of the polyisoprenoid side chain with p-hydroxybenzoate

Structure

• belongs to the ubiA prenyltransferase family

Compartment

mitochondrial membrane

Alternative splicing

named isoforms=3

Expression

• widely expressed
• high expressison in skeletal muscle, adrenal, heart

Pathology

• defects in COQ2 are a cause of coenzyme Q10 deficiency

More general terms

• transmembrane 9 protein
• prenyltransferase

Database

• OMIM: https://mirror.omim.org/entry/607426
• UniProt: http://www.uniprot.org/uniprot/Q96H96.html