COQ7:COQ9 octamer hydroxylates DMQ10H2

Stable Identifier
Reaction [transition]
Homo sapiens
DMQ10H2 is hydroxylated to DeMQ10H2 by COQ7
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5-Demethoxyubiquinone hydroxylase COQ7 catalyzes the hydroxylation of 6-methoxy-3-methyl-2-decaprenyl-1,4-benzoquinol (DMQ10H2, DMQ) to 3-demethylubiquinol-10 (DeMQ10H2, DeMQ), using NADH and oxygen. COQ7 forms a heterooctameric complex with ubiquinone biosynthesis protein COQ9, a lipid-binding protein presenting the substrate to COQ7 activity. In this complex, COQ7 binds two iron ions and forms a heterodimer with COQ9, with four of these dimers forming an octameric cage (Vajo et al., 1999; Lohman et al., 2014; Manicki et al., 2022). This reaction was first studied in yeast (Marbois & Clarke, 1996; Tran et al., 2006). Both COQ8A and COQ8B bind to COQ7, presumably during formation of a hypothetical multienzyme COQ complex. COQ8A,B are required for the assembly of the complex but may not be stably incorporated into it, so only the components identified by Floyd et al. are annotated (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
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
10501970 Conservation of the Caenorhabditis elegans timing gene clk-1 from yeast to human: a gene required for ubiquinone biosynthesis with potential implications for aging

Ho, N, Francomano, CA, Jonassen, T, King, LM, Wilkin, DJ, Munnich, A, Vajo, Z, Clarke, CF

Mamm Genome 1999
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
36306796 Structure and functionality of a multimeric human COQ7:COQ9 complex

Frost, A, Pagliarini, DJ, Guerra, RM, Dal Peraro, M, Aydin, H, Abriata, LA, Manicki, M, Coon, JJ, Overmyer, KA

Mol Cell 2022
16624818 Complementation of Saccharomyces cerevisiae coq7 mutants by mitochondrial targeting of the Escherichia coli UbiF polypeptide: two functions of yeast Coq7 polypeptide in coenzyme Q biosynthesis

Marbois, B, Jonassen, T, Gin, P, Gulmezian, M, Tran, UC, Clarke, CF

J Biol Chem 2006
25339443 Mitochondrial COQ9 is a lipid-binding protein that associates with COQ7 to enable coenzyme Q biosynthesis

Sukumar, S, Luna-Sánchez, M, Stefely, JA, Tong, L, Everett, JK, Westphall, MS, Xiao, R, Lew, S, Wrobel, RL, Lohman, DC, Jochem, A, Coon, JJ, Ulbrich, A, Stefely, MS, Beebe, ET, Minogue, CE, Pagliarini, DJ, Mitchell, JC, Wang, H, López, LC, Seetharaman, J, Forouhar, F

Proc. Natl. Acad. Sci. U.S.A. 2014
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
8621692 The COQ7 gene encodes a protein in Saccharomyces cerevisiae necessary for ubiquinone biosynthesis

Marbois, BN, Clarke, CF

J Biol Chem 1996
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
Orthologous Events
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