| Tissue Specificity | Expressed dominantly in heart and skeletal muscle. |
| Function | Catalyzes the hydroxylation of the 5-methoxy-2-methyl-3-(all-trans-polyprenyl)benzoquinone at the C6 position and participates in the biosynthesis of ubiquinone (Probable). Catalyzes the reaction through a substrate-mediated reduction pathway, whereby NADH shuttles electrons to 5-methoxy-2-methyl-3-(all-trans-decaprenyl)benzoquinone, which then transfers the electrons to the two Fe(3+) centers. The binding of 5-methoxy-2-methyl-3-(all-trans-polyprenyl)benzoquinone (DMQn) mediates reduction of the diiron center by nicotinamide adenine dinucleotide (NADH) and initiates oxygen activation for subsequent DMQ hydroxylation. The physiological substrates are 5-methoxy-2-methyl-3-(all-trans-nonaprenyl)benzoquinone (DMQ(9)) and 5-methoxy-2-methyl-3-(all-trans-decaprenyl)benzoquinone (DMQ(10)), however in vitro the enzyme does not have any specificity concerning the length of the polyprenyl tail, and accepts tails of various lengths with similar efficiency. Also has a structural role in the COQ enzyme complex, stabilizing other COQ polypeptides. Involved in lifespan determination in a ubiquinone-independent manner. Plays a role in modulating mitochondrial stress responses, acting in the nucleus, perhaps via regulating gene expression, independent of its characterized mitochondrial function in ubiquinone biosynthesis. |
| Protein Name | Nadph-Dependent 3-Demethoxyubiquinone 3-Hydroxylase - Mitochondrial3-Demethoxyubiquinone 3-HydroxylaseNadhTiming Protein Clk-1 HomologUbiquinone Biosynthesis Monooxygenase Coq7 |
| Database Links | Reactome: R-HSA-2142789 |
| Cellular Localisation | Mitochondrion Inner MembranePeripheral Membrane ProteinMatrix SideMitochondrionNucleusChromosome |
| Alternative Antibody Names | Anti-Nadph-Dependent 3-Demethoxyubiquinone 3-Hydroxylase - Mitochondrial antibodyAnti-3-Demethoxyubiquinone 3-Hydroxylase antibodyAnti-Nadh antibodyAnti-Timing Protein Clk-1 Homolog antibodyAnti-Ubiquinone Biosynthesis Monooxygenase Coq7 antibodyAnti-COQ7 antibody |
Information sourced from Uniprot.org
