Isolation and functional expression of human COQ2, a gene encoding a polyprenyl transferase involved in the synthesis of CoQ

Biochem J. 2004 Sep 1;382(Pt 2):519-26. doi: 10.1042/BJ20040261.


The COQ2 gene in Saccharomyces cerevisiae encodes a Coq2 (p-hydroxybenzoate:polyprenyl transferase), which is required in the biosynthetic pathway of CoQ (ubiquinone). This enzyme catalyses the prenylation of p-hydroxybenzoate with an all-trans polyprenyl group. We have isolated cDNA which we believe encodes the human homologue of COQ2 from a human muscle and liver cDNA library. The clone contained an open reading frame of length 1263 bp, which encodes a polypeptide that has sequence homology with the Coq2 homologues in yeast, bacteria and mammals. The human COQ2 gene, when expressed in yeast Coq2 null mutant cells, rescued the growth of this yeast strain in the absence of a non-fermentable carbon source and restored CoQ biosynthesis. However, the rate of CoQ biosynthesis in the rescued cells was lower when compared with that in cells rescued with the yeast COQ2 gene. CoQ formed when cells were incubated with labelled decaprenyl pyrophosphate and nonaprenyl pyrophosphate, showing that the human enzyme is active and that it participates in the biosynthesis of CoQ.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Alkyl and Aryl Transferases / deficiency
  • Alkyl and Aryl Transferases / genetics*
  • Alkyl and Aryl Transferases / metabolism
  • Amino Acid Sequence / genetics
  • Base Sequence / genetics
  • Cloning, Molecular / methods
  • Gene Expression Regulation, Enzymologic / genetics*
  • Genetic Complementation Test / methods
  • Humans
  • Liver / chemistry
  • Liver / metabolism
  • Mitochondrial Proteins / genetics
  • Molecular Sequence Data
  • Muscle, Skeletal / chemistry
  • Muscle, Skeletal / metabolism
  • Mutation / genetics
  • Organ Specificity / genetics
  • Predictive Value of Tests
  • Protein Sorting Signals / genetics
  • RNA, Messenger / genetics
  • Saccharomyces cerevisiae / chemistry
  • Saccharomyces cerevisiae / enzymology
  • Saccharomyces cerevisiae / genetics
  • Saccharomyces cerevisiae / growth & development
  • Saccharomyces cerevisiae Proteins / genetics
  • Sequence Analysis, DNA / methods
  • Ubiquinone / biosynthesis*


  • Mitochondrial Proteins
  • Protein Sorting Signals
  • RNA, Messenger
  • Saccharomyces cerevisiae Proteins
  • Ubiquinone
  • Alkyl and Aryl Transferases
  • 4-hydroxybenzoate polyprenyltransferase

Associated data

  • GENBANK/AJ621061