DHB is hydroxylated to DHDB by COQ6

Stable Identifier
R-HSA-2162187
Type
Reaction [transition]
Species
Homo sapiens
Compartment
mitochondrial inner membrane, mitochondrial matrix
ReviewStatus
5/5
Locations in the PathwayBrowser
Expand all
General
SVG | 
PNG
Low
Medium
High
 | PPTX  | SBGN
DHB is hydroxylated to DHDB by COQ6
Click the image above or here to open this reaction in the Pathway Browser
The layout of this reaction may differ from that in the pathway view due to the constraints in pathway layout

A flavin-dependent monooxygenase involved in ubiquinone/ubiquinol biosynthesis, COQ6 (Heeringa et al. 2011) catalyses the C5-hydroxylation of 3-decaprenyl-4-hydroxybenzoic acid (DHB) to 3,4-dihydroxy-5-decaprenylbenzoic acid (DHDB). COQ6 is a peripheral membrane protein that localizes to the matrix side of the inner mitochondrial membrane (Gin et al. 2003). This reaction involving COQ6 is inferred from the equivalent reaction in yeast (Ozeir et al. 2011, Gin et al. 2003).

Literature References
PubMed ID Title Journal Year
12721307 The Saccharomyces cerevisiae COQ6 gene encodes a mitochondrial flavin-dependent monooxygenase required for coenzyme Q biosynthesis

Tzagoloff, A, Rothman, SC, Jonassen, T, Hsu, AY, Gin, P, Lee, PT, Clarke, CF

J Biol Chem 2003
21540551 COQ6 mutations in human patients produce nephrotic syndrome with sensorineural deafness

Nürnberg, P, Prokisch, H, Becker, C, Bakkaloglu, A, Killen, PD, Berdeli, A, McLaughlin, HM, Casarin, A, Varpizen, S, Zenker, M, Müller, D, Topaloglu, R, Airik, R, Krick, S, Raphael, Y, Zhou, W, Faul, C, Clarke, CF, Vega-Warner, V, Vlangos, CN, Wiggins, RC, Navas, P, Xie, LX, Gbadegesin, R, Ji, Z, Pertegato, V, Heeringa, SF, Trevisson, E, Mir, S, Doimo, M, Akman, S, Schoeb, DS, Reiser, J, Sloan, AJ, Salviati, L, Ovunc, B, Nürnberg, G, Mundel, P, Chernin, G, Kispert, A, Soliman, NA, Beissert, A, Rötig, A, Wang, S, Hildebrandt, F, Giorgi, G, Hurd, TW, Ashraf, S, Ozaltin, F, Santos-Ocaña, C, Hinkes, B, Bakkaloglu, SA, Kusakabe, T, Chaki, M, Saisawat, P, Matejas, V

J Clin Invest 2011
21944752 Coenzyme Q biosynthesis: Coq6 is required for the C5-hydroxylation reaction and substrate analogs rescue Coq6 deficiency

Ozeir, M, Fontecave, M, Lill, R, Pierrel, F, Webert, H, Mühlenhoff, U

Chem Biol 2011
Participants
Input
Output
Participates
as an event of
Catalyst Activity

oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen, NAD(P)H as one donor, and incorporation of one atom of oxygen of COQ6:FAD [mitochondrial inner membrane]

Physical Entity
COQ6:FAD [mitochondrial inner membrane] (Homo sapiens)
Activity
oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen, NAD(P)H as one donor, and incorporation of one atom of oxygen (GO:0016709)
Inferred From
HHB is hydroxylated to DHHB by Coq6 (Saccharomyces cerevisiae)
Authored
Williams, MG  (2012-03-19)
Reviewed
Kawamukai, M  (2013-02-05)
Created
Williams, MG  (2012-03-15)
Cite Us!