DHB is hydroxylated to DHDB by COQ6

Stable Identifier
R-HSA-2162187
Type
Reaction [transition]
Species
Homo sapiens
Compartment
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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
21540551 COQ6 mutations in human patients produce nephrotic syndrome with sensorineural deafness

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

J Clin Invest 2011
12721307 The Saccharomyces cerevisiae COQ6 gene encodes a mitochondrial flavin-dependent monooxygenase required for coenzyme Q biosynthesis

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

J Biol Chem 2003
21944752 Coenzyme Q biosynthesis: Coq6 is required for the C5-hydroxylation reaction and substrate analogs rescue Coq6 deficiency

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

Chem Biol 2011
Participants
Participant Of
Catalyst Activity
Catalyst Activity
Title
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
Activity
Inferred From
Authored
Reviewed
Created