This report describes general characteristics of the group of diseases classified as primary coenzyme Q10 deficiency (COQ10D). COQ10D is characterized by a reduction of coenzyme Q10 (ubiquinone) levels associated with mutation of one of the genes involved in the biosynthesis of coenzyme Q10. A comprehensive list of COQ10D subtypes, as defined by OMIM, can be found by following the link in the "OMIM phenotypic series" section, below. A subset of these are listed in the table below, with links to more detailed reports for subtypes that have been investigated using fly models.
[updated Apr. 2022 by FlyBase; FBrf0222196]