COQ2

COQ2
Identifiers
Aliases	COQ2, CL640, COQ10D1, MSA1, PHB:PPT, coenzyme Q2, polyprenyltransferase
External IDs	OMIM: 609825; MGI: 1919133; HomoloGene: 69192; GeneCards: COQ2; OMA:COQ2 - orthologs
Gene location (Human) Chr. Chromosome 4 (human) Band 4q21.23 Start 83,261,536 bp End 83,284,914 bp
Gene location (Mouse) Chr. Chromosome 5 (mouse) Band 5|5 E4 Start 100,802,589 bp End 100,823,006 bp
RNA expression pattern Bgee Human Mouse (ortholog) Top expressed in Skeletal muscle tissue of biceps brachii Skeletal muscle tissue of rectus abdominis gingival epithelium vastus lateralis muscle monocyte body of tongue triceps brachii muscle muscle of thigh glutes deltoid muscle Top expressed in left ventricle interventricular septum proximal tubule quadriceps femoris muscle right kidney muscle of thigh upper arm neural layer of retina muscle tissue visual cortex More reference expression data BioGPS More reference expression data
Gene ontology Molecular function transferase activity 4-hydroxybenzoate nonaprenyltransferase activity prenyltransferase activity 4-hydroxybenzoate decaprenyltransferase activity transferase activity, transferring alkyl or aryl (other than methyl) groups Cellular component integral component of membrane mitochondrial inner membrane membrane mitochondrion integral component of mitochondrial inner membrane Biological process glycerol metabolic process isoprenoid biosynthetic process ubiquinone biosynthetic process Sources:Amigo / QuickGO
Orthologs Species Human Mouse Entrez 27235 71883 Ensembl ENSG00000173085 ENSMUSG00000029319 UniProt Q96H96 Q66JT7 RefSeq (mRNA) NM_015697 NM_001358921 NM_027978 RefSeq (protein) NP_056512 NP_001345850 NP_082254 Location (UCSC) Chr 4: 83.26 – 83.28 Mb Chr 5: 100.8 – 100.82 Mb PubMed search
Wikidata
View/Edit Human View/Edit Mouse

Para-hydroxybenzoate—polyprenyltransferase, mitochondrial is an enzyme that in humans is encoded by the COQ2 gene.

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]

External links

• Human COQ2 genome location and COQ2 gene details page in the UCSC Genome Browser.

Further reading

• Dias Neto E, Correa RG, Verjovski-Almeida S, et al. (2000). "Shotgun sequencing of the human transcriptome with ORF expressed sequence tags". Proc. Natl. Acad. Sci. U.S.A. 97 (7): 3491–6. Bibcode:2000PNAS...97.3491D. doi:10.1073/pnas.97.7.3491. PMC 16267. PMID 10737800.
• Strausberg RL, Feingold EA, Grouse LH, et al. (2003). "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. Bibcode:2002PNAS...9916899M. doi:10.1073/pnas.242603899. PMC 139241. PMID 12477932.
• Gerhard DS, Wagner L, Feingold EA, et al. (2004). "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)". Genome Res. 14 (10B): 2121–7. doi:10.1101/gr.2596504. PMC 528928. PMID 15489334.
• Esaka Y, Nagahara Y, Hasome Y, et al. (2005). "Coenzyme Q2 induced p53-dependent apoptosis". Biochim. Biophys. Acta. 1724 (1–2): 49–58. doi:10.1016/j.bbagen.2005.04.013. PMID 15905035.
• Quinzii C, Naini A, Salviati L, et al. (2007). "A mutation in para-hydroxybenzoate-polyprenyl transferase (COQ2) causes primary coenzyme Q10 deficiency". Am. J. Hum. Genet. 78 (2): 345–9. doi:10.1086/500092. PMC 1380241. PMID 16400613.
• Burón MI, Hermán MD, Alcaín FJ, Villalba JM (2006). "Stimulation of polyprenyl 4-hydroxybenzoate transferase activity by sodium cholate and 3-[(cholamidopropyl)dimethylammonio]-1-propanesulfonate". Anal. Biochem. 353 (1): 15–21. doi:10.1016/j.ab.2006.03.029. PMID 16643834.
• González-Aragón D, Burón MI, López-Lluch G, et al. (2006). "Coenzyme Q and the regulation of intracellular steady-state levels of superoxide in HL-60 cells". BioFactors. 25 (1–4): 31–41. doi:10.1002/biof.5520250105. PMID 16873928. S2CID 45053711.
• Mollet J, Giurgea I, Schlemmer D, et al. (2007). "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. doi:10.1172/JCI29089. PMC 1804361. PMID 17332895.
• López-Martín JM, Salviati L, Trevisson E, et al. (2007). "Missense mutation of the COQ2 gene causes defects of bioenergetics and de novo pyrimidine synthesis". Hum. Mol. Genet. 16 (9): 1091–7. doi:10.1093/hmg/ddm058. PMC 4345105. PMID 17374725.
• Oh J, Ban MR, Miskie BA, et al. (2007). "Genetic determinants of statin intolerance". Lipids in Health and Disease. 6: 7. doi:10.1186/1476-511X-6-7. PMC 1832194. PMID 17376224.
• Diomedi-Camassei F, Di Giandomenico S, Santorelli FM, et al. (2007). "COQ2 nephropathy: a newly described inherited mitochondriopathy with primary renal involvement". J. Am. Soc. Nephrol. 18 (10): 2773–80. doi:10.1681/ASN.2006080833. PMID 17855635.