COQ2

COQ2

Identifiers

COQ2, CL640, COQ10D1, MSA1, PHB:PPT, coenzyme Q2, polyprenyltransferase

External IDs

OMIM: 609825 MGI: 1919133 HomoloGene: 69192 GeneCards: COQ2

Gene location (Human)

Chr.	Chromosome 4 (human)^[1]

Band	4q21.23	Start	83,261,536 bp^[1]
Band	4q21.23	End	83,284,914 bp^[1]

Gene location (Mouse)

Chr.	Chromosome 5 (mouse)^[2]

Band	5\|5 E4	Start	100,802,589 bp^[2]
Band	5\|5 E4	End	100,823,006 bp^[2]

RNA expression pattern

Bgee

Human

Mouse (ortholog)

Top expressed in

vastus lateralis muscle

monocyte

body of tongue

triceps brachii muscle

deltoid muscle

palpebral conjunctiva

right adrenal gland

left adrenal gland

right ventricle

thoracic diaphragm

Top expressed in

left ventricle

interventricular septum

proximal tubule

quadriceps femoris muscle

upper arm

muscle tissue

visual cortex

pancreas

superior frontal gyrus

zone of skin

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

^[3]

^[4]

Wikidata

View/Edit Human

View/Edit Mouse

Para-hydroxybenzoate—polyprenyltransferase, mitochondrial is an enzyme that in humans is encoded by the COQ2 gene.^[5]^[6]

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]^[6]

References

^ ^a ^b ^c GRCh38: Ensembl release 89: ENSG00000173085 - Ensembl, May 2017
^ ^a ^b ^c GRCm38: Ensembl release 89: ENSMUSG00000029319 - Ensembl, May 2017
^ "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
^ "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
^ Forsgren M, Attersand A, Lake S, Grunler J, Swiezewska E, Dallner G, Climent I (Aug 2004). "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. doi:10.1042/BJ20040261. PMC 1133808. PMID 15153069.
^ ^a ^b "Entrez Gene: COQ2 coenzyme Q2 homolog, prenyltransferase (yeast)".

External links

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

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.

Metabolism: Citric acid cycle enzymes

Cycle

Anaplerotic

to acetyl-CoA	Pyruvate dehydrogenase complex (E1, E2, E3) (regulated by Pyruvate dehydrogenase kinase and Pyruvate dehydrogenase phosphatase)
to α-ketoglutaric acid	Glutamate dehydrogenase
to succinyl-CoA	Methylmalonyl-CoA mutase
to oxaloacetic acid	Pyruvate carboxylase Aspartate transaminase

Mitochondrial
electron transport chain/
oxidative phosphorylation

Primary	Complex I/NADH dehydrogenase Complex II/Succinate dehydrogenase Coenzyme Q₁₀ (CoQ10) Complex III/Coenzyme Q - cytochrome c reductase Cytochrome c Complex IV/Cytochrome c oxidase Coenzyme Q₁₀ synthesis: COQ2 COQ3 COQ4 COQ5 COQ6 COQ7 COQ9 COQ10A COQ10B PDSS1 PDSS2
Other	Alternative oxidase Electron-transferring-flavoprotein dehydrogenase

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This page was last edited on 3 December 2023, at 00:50

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References

External links

Further reading