COQ6 (Coenzyme Q10 monooxygenase 6) is also named as CGI-10 and belongs to the ubiH/COQ6 family. It is an evolutionarily conserved flavin-dependent monooxygenase required for biosynthesis of coenzyme Q10, or ubiquinone. It has 3 isoforms with the molecular mass of 51 kDa, 43 kDa and 49 kDa. In inner ear, Coq6 is expressed in spiral ganglion, stria vascularis cells, and spiral ligament. Defects in COQ6 are the cause of coenzyme Q10 deficiency, primary, type 6 (COQ10D6).
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Protein Aliases: Coenzyme Q10 monooxygenase 6; coenzyme Q6 homolog, monooxygenase; coenzyme Q6 monooxygenase; ubiquinone biosynthesis monooxygenase COQ6; Ubiquinone biosynthesis monooxygenase COQ6, mitochondrial
Gene Aliases: 5930427M12Rik; AW742344; CGI-10; CGI10; COQ10D6; COQ6