COQ2 explained

Para-hydroxybenzoate—polyprenyltransferase, mitochondrial is an enzyme that in humans is encoded by the COQ2 gene.^{[1] [2]}

CoQ (ubiquinone) serves as a redox carrier in the mitochondrial respiratory chain and is a lipid-soluble antioxidant. COQ2, or parahydroxybenzoate-polyprenyltransferase (EC 2.5.1.39), catalyzes one of the final reactions in the biosynthesis of CoQ, the prenylation of parahydroxybenzoate with an all-trans polyprenyl group (Forsgren et al., 2004).[supplied by OMIM]

Further reading

• Dias Neto E, Correa RG, Verjovski-Almeida S . Shotgun sequencing of the human transcriptome with ORF expressed sequence tags . Proc. Natl. Acad. Sci. U.S.A. . 97 . 7 . 3491–6 . 2000 . 10737800 . 10.1073/pnas.97.7.3491 . 16267 . etal. 2000PNAS...97.3491D . free .
• Strausberg RL, Feingold EA, Grouse LH . Generation and initial analysis of more than 15,000 full-length human and mouse cDNA sequences . Proc. Natl. Acad. Sci. U.S.A. . 99 . 26 . 16899–903 . 2003 . 12477932 . 10.1073/pnas.242603899 . 139241 . etal . 2002PNAS...9916899M. free .
• Gerhard DS, Wagner L, Feingold EA . The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC) . Genome Res. . 14 . 10B . 2121–7 . 2004 . 15489334 . 10.1101/gr.2596504 . 528928 . etal.
• Esaka Y, Nagahara Y, Hasome Y . Coenzyme Q2 induced p53-dependent apoptosis . Biochim. Biophys. Acta . 1724 . 1–2 . 49–58 . 2005 . 15905035 . 10.1016/j.bbagen.2005.04.013 . etal.
• Quinzii C, Naini A, Salviati L . A mutation in para-hydroxybenzoate-polyprenyl transferase (COQ2) causes primary coenzyme Q10 deficiency . Am. J. Hum. Genet. . 78 . 2 . 345–9 . 2007 . 16400613 . 10.1086/500092 . 1380241 . etal.
• Burón MI, Hermán MD, Alcaín FJ, Villalba JM . Stimulation of polyprenyl 4-hydroxybenzoate transferase activity by sodium cholate and 3-[(cholamidopropyl)dimethylammonio]-1-propanesulfonate . Anal. Biochem. . 353 . 1 . 15–21 . 2006 . 16643834 . 10.1016/j.ab.2006.03.029 .
• González-Aragón D, Burón MI, López-Lluch G . Coenzyme Q and the regulation of intracellular steady-state levels of superoxide in HL-60 cells . BioFactors . 25 . 1–4 . 31–41 . 2006 . 16873928 . 10.1002/biof.5520250105 . 45053711 . etal.
• Mollet J, Giurgea I, Schlemmer D . Prenyldiphosphate synthase, subunit 1 (PDSS1) and OH-benzoate polyprenyltransferase (COQ2) mutations in ubiquinone deficiency and oxidative phosphorylation disorders . J. Clin. Invest. . 117 . 3 . 765–72 . 2007 . 17332895 . 10.1172/JCI29089 . 1804361 . etal.
• López-Martín JM, Salviati L, Trevisson E . Missense mutation of the COQ2 gene causes defects of bioenergetics and de novo pyrimidine synthesis . Hum. Mol. Genet. . 16 . 9 . 1091–7 . 2007 . 17374725 . 10.1093/hmg/ddm058 . etal. 4345105 .
• Oh J, Ban MR, Miskie BA . Genetic determinants of statin intolerance . Lipids in Health and Disease . 6 . 7 . 2007 . 17376224 . 10.1186/1476-511X-6-7 . 1832194 . etal . free .
• Diomedi-Camassei F, Di Giandomenico S, Santorelli FM . COQ2 nephropathy: a newly described inherited mitochondriopathy with primary renal involvement . J. Am. Soc. Nephrol. . 18 . 10 . 2773–80 . 2007. 17855635 . 10.1681/ASN.2006080833 . etal. free .

Notes and References

1. Forsgren M, Attersand A, Lake S, Grunler J, Swiezewska E, Dallner G, Climent I . Isolation and functional expression of human COQ2, a gene encoding a polyprenyl transferase involved in the synthesis of CoQ . Biochem J . 382 . Pt 2 . 519–26 . Aug 2004 . 15153069 . 1133808 . 10.1042/BJ20040261 .
2. Web site: Entrez Gene: COQ2 coenzyme Q2 homolog, prenyltransferase (yeast).