The world's first wiki where authorship really matters (Nature Genetics, 2008). Due credit and reputation for authors. Imagine a global collaborative knowledge base for original thoughts. Search thousands of articles and collaborate with scientists around the globe.

wikigene or wiki gene protein drug chemical gene disease author authorship tracking collaborative publishing evolutionary knowledge reputation system wiki2.0 global collaboration genes proteins drugs chemicals diseases compound
Hoffmann, R. A wiki for the life sciences where authorship matters. Nature Genetics (2008)



Gene Review

COQ2  -  coenzyme Q2 4-hydroxybenzoate...

Homo sapiens

Synonyms: 4-hydroxybenzoate polyprenyltransferase, mitochondrial, CL640, COQ10D1, COQ2 homolog, FLJ26072, ...
Welcome! If you are familiar with the subject of this article, you can contribute to this open access knowledge base by deleting incorrect information, restructuring or completely rewriting any text. Read more.

Disease relevance of COQ2

  • The first defect in a CoQ(10) biosynthetic gene, COQ2, was identified in a child with encephalomyopathy and nephrotic syndrome and in a younger sibling with only nephropathy [1].
  • Because mutations in the COQ2 gene are associated with severe inherited myopathy, we hypothesized that common, mild genetic variation in COQ2 would be associated with inter-individual variation in statin intolerance [2].

High impact information on COQ2

  • A Mutation in Para-Hydroxybenzoate-Polyprenyl Transferase (COQ2) Causes Primary Coenzyme Q10 Deficiency [3].
  • In two siblings of consanguineous parents with the infantile form of CoQ(10) deficiency, we identified a homozygous missense mutation in the COQ2 gene, which encodes para-hydroxybenzoate-polyprenyl transferase [3].
  • COQ2 polypeptide, the localization of which was forcibly altered to the endoplasmic reticulum, had the same or a greater effect as mitochondria-localized COQ2 on the increase in UQ in both the yeast and tobacco transformants, indicating that the UQ intermediate is transported from the endoplasmic reticulum to the mitochondria [4].
  • Polyprenyl 4-hydroxybenzoate transferase (Coq2p) plays a central role in ubiquinone biosynthesis [5].
  • Culturing HL-60 cells in the presence of p-aminobenzoate, a competitive inhibitor of polyprenyl-4-hydroxybenzoate transferase (Coq2p), produced a significant decrease of CoQ(10) levels without affecting cell viability [6].

Biological context of COQ2


Associations of COQ2 with chemical compounds


Other interactions of COQ2

  • CONCLUSION: These preliminary pharmacogenetic results, if confirmed, are consistent with the idea that statin intolerance which is manifested primarily through muscle symptoms is associated with genomic variation in COQ2 and thus perhaps with the CoQ10 pathway [2].


  1. Leigh syndrome with nephropathy and CoQ10 deficiency due to decaprenyl diphosphate synthase subunit 2 (PDSS2) mutations. L??pez, L.C., Schuelke, M., Quinzii, C.M., Kanki, T., Rodenburg, R.J., Naini, A., Dimauro, S., Hirano, M. Am. J. Hum. Genet. (2006) [Pubmed]
  2. Genetic determinants of statin intolerance. Oh, J., Ban, M.R., Miskie, B.A., Pollex, R.L., Hegele, R.A. Lipids in health and disease (2007) [Pubmed]
  3. A Mutation in Para-Hydroxybenzoate-Polyprenyl Transferase (COQ2) Causes Primary Coenzyme Q10 Deficiency. Quinzii, C., Naini, A., Salviati, L., Trevisson, E., Navas, P., Dimauro, S., Hirano, M. Am. J. Hum. Genet. (2006) [Pubmed]
  4. Engineering of ubiquinone biosynthesis using the yeast coq2 gene confers oxidative stress tolerance in transgenic tobacco. Ohara, K., Kokado, Y., Yamamoto, H., Sato, F., Yazaki, K. Plant J. (2004) [Pubmed]
  5. Stimulation of polyprenyl 4-hydroxybenzoate transferase activity by sodium cholate and 3-[(cholamidopropyl)dimethylammonio]-1-propanesulfonate. Burón, M.I., Hermán, M.D., Alcaín, F.J., Villalba, J.M. Anal. Biochem. (2006) [Pubmed]
  6. Coenzyme Q and the regulation of intracellular steady-state levels of superoxide in HL-60 cells. González-Aragón, D., Burón, M.I., López-Lluch, G., Hermán, M.D., Gómez-Díaz, C., Navas, P., Villalba, J.M. Biofactors (2005) [Pubmed]
  7. Isolation and functional expression of human COQ2, a gene encoding a polyprenyl transferase involved in the synthesis of CoQ. Forsgren, M., Attersand, A., Lake, S., Grünler, J., Swiezewska, E., Dallner, G., Climent, I. Biochem. J. (2004) [Pubmed]
WikiGenes - Universities