COQ6 hydroxylates DHB
DHB + O2 + H+ + (2Fe-2S)(1+) → DHDB + H2O + (2Fe-2S)2+
- Reactome
- R-HSA-2162187
- Compartment
- mitochondrial inner membrane · mitochondrial matrix
- Rhea
- None recorded
Inputs
- DHBCHEBI:84503, C57H85O3, mitochondrial inner membrane
- O2CHEBI:15379, O2, mitochondrial matrix
- H+CHEBI:15378, H, mitochondrial matrix
- (2Fe-2S)(1+)CHEBI:33738, Fe2S2, mitochondrial matrix
Outputs
- DHDBCHEBI:62793, C57H85O4, mitochondrial inner membrane
- H2OCHEBI:15377, H2O, mitochondrial matrix
- (2Fe-2S)2+CHEBI:33737, Fe2S2, mitochondrial matrix
Catalysed by
- 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…EC 1.14.13
Literature
- COQ6 mutations in human patients produce nephrotic syndrome with sensorineural deafness J Clin Invest, 2011
- Coenzyme Q biosynthesis: Coq6 is required for the C5-hydroxylation reaction and substrate analogs rescue Coq6 deficiency Chem Biol, 2011
- The Saccharomyces cerevisiae COQ6 gene encodes a mitochondrial flavin-dependent monooxygenase required for coenzyme Q biosynthesis J Biol Chem, 2003
- Mitochondrial Protein Interaction Mapping Identifies Regulators of Respiratory Chain Function Mol Cell, 2016
- ADCK4 mutations promote steroid-resistant nephrotic syndrome through CoQ10 biosynthesis disruption J Clin Invest, 2013
- Adolescence Onset Primary Coenzyme Q10 Deficiency With Rare CoQ8A Gene Mutation: A Case Report and Review of Literature Clin Med Insights Case Rep, 2023