COQ6

Coenzyme Q6 monooxygenase is a is_associated_with::protein that in humans is encoded by the COQ6 is_associated_with::gene.

Function
The protein encoded by this gene belongs to the ubiH/COQ6 family. It is an evolutionarily conserved monooxygenase required for the biosynthesis of is_associated_with::coenzyme Q10 (or ubiquinone), which is an essential component of the mitochondrial is_associated_with::electron transport chain, and one of the most potent lipophilic is_associated_with::antioxidants implicated in the protection of cell damage by is_associated_with::reactive oxygen species. knockdown of this gene in mouse and zebrafish results in decreased growth due to increased is_associated_with::apoptosis.

Clinical significance
Mutations in this gene are associated with autosomal recessive coenzyme Q10 deficiency-6 (COQ10D6), which manifests as is_associated_with::nephrotic syndrome with is_associated_with::sensorineural deafness. Alternatively spliced transcript variants encoding different isoforms have been described for this gene.