Protein Name coenzyme Q2, polyprenyltransferase
Organism Homo sapiens
Gene ID 27235
Gene Symbol

COQ2

UniProt -
Relationships Total Number of functionally related compound(s) : 38
Total Number of Articles : 48
Description

coenzyme Q2, polyprenyltransferase

Gene Summary

This gene encodes an enzyme that functions in the final steps in the biosynthesis of CoQ (ubiquinone), a redox carrier in the mitochondrial respiratory chain and a lipid-soluble antioxidant. This enzyme, which is part of the coenzyme Q10 pathway, catalyzes the prenylation of parahydroxybenzoate with an all-trans polyprenyl group. Mutations in this gene cause coenzyme Q10 deficiency, a mitochondrial encephalomyopathy, and also COQ2 nephropathy, an inherited form of mitochondriopathy with primary renal involvement. [provided by RefSeq, Oct 2009]

synonyms
  • 4-hydroxybenzoate polyprenyltransferase, mitochondrial
  • 4-HB polyprenyltransferase
  • 4-hydroxybenzoate decaprenyltransferase
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Properties