COQ6 hydroxylates DHB

Stable Identifier
R-HSA-2162187
Type
Reaction [transition]
Species
Homo sapiens
Compartment
Synonyms
DHB is hydroxylated to DHDB by COQ6
ReviewStatus
4/5
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Flavin-dependent monooxygenase COQ6 (Heeringa et al. 2011) catalyses the C5-hydroxylation of 3-decaprenyl-4-hydroxybenzoic acid (DHB) to 3,4-dihydroxy-5-decaprenylbenzoic acid (DHDB). The electrons needed likely come from the iron-sulfur cluster on a ferredoxin that is recycled with NADPH. 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, where hexaprenyl sidechains occur instead of decaprenyl moieties in human (Ozeir et al. 2011, Gin et al. 2003). Both COQ8A and COQ8B bind to COQ6, presumably during formation of a hypothetical multienzyme COQ complex. Both COQ8A,B are required for Q10 biosynthesis (Ashraf et al., 2013; Floyd et al., 2016; reviewed in Hojabri et al., 2023; Liang et al., 2023).
Literature References
PubMed ID Title Journal Year
27499296 Mitochondrial Protein Interaction Mapping Identifies Regulators of Respiratory Chain Function

Stefely, JA, Taylor, RW, Dolan, BK, Westphall, MS, Wilkerson, EM, Veling, MT, Wrobel, RL, Kim, JP, Jochem, A, Cho, H, Coon, JJ, Xia, C, Ulbrich, A, Gromek, KA, Rensvold, JW, Bohl, SL, Beebe, ET, Pagliarini, DJ, Werner, KM, Alston, CL, Floyd, BJ, Prokisch, H, Kremer, LS, Minogue, CE

Mol Cell 2016
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
36843884 Clinical features and gene variation analysis of COQ8B nephropathy: Report of seven cases

Yang, Q, Zhang, Y, Liang, R, Chen, H, Yang, H, Wu, D, Law, CF, Yu, S, Zhang, G, Wang, A, Wang, M, Jiao, J, Chen, X

Front Pediatr 2022
24270420 ADCK4 mutations promote steroid-resistant nephrotic syndrome through CoQ10 biosynthesis disruption

Salviati, L, Washburn, J, Choi, M, Barua, M, Gee, HY, Han, Z, Saleem, MA, Gok, F, Clarke, CF, Vega-Warner, V, Allen, SJ, Evans, J, Esteve-Rudd, J, Lifton, RP, Nitschké, P, El Desoky, S, Bole-Feysot, C, Levy, S, Airik, R, Ashraf, S, Pei, Y, Haberberger, B, Bierzynska, A, Woerner, S, Paterson, AD, Xie, LX, Bockenhauer, D, Antignac, C, Al-Hamed, MH, Hacihamdioglu, DO, Cochat, P, Kleta, R, Lovric, S, Pollak, M, Wiggins, RC, Avila-Casado, C, Cattran, DC, Zhou, W, Williams, DS, Hildebrandt, F, Prokisch, H, Kari, JA, Otto, EA, Song, X, Fang, H

J Clin Invest 2013
37476682 Adolescence Onset Primary Coenzyme Q10 Deficiency With Rare CoQ8A Gene Mutation: A Case Report and Review of Literature

Nejad Biglari, H, Irilouzadian, R, Sarmadian, R, Hojabri, M, Gilani, A

Clin Med Insights Case Rep 2023
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
Participates
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 COQ3:COQ4:COQ5:COQ6:COQ7:COQ9 [mitochondrial inner membrane]

This event is regulated
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