Coenzyme Q biosynthesis in health and disease

CoQ10 deficiency is unique among mitochondrial disorders since an effective treatment is available. Many patients respond to oral CoQ10 supplementation. Nevertheless, treatment is still problematic because of the low bioavailability of the compound, and novel pharmacological approaches are currently being investigated. This article is part of a Special Issue entitled 'EBEC 2016: 19th European Bioenergetics Conference, Riva del Garda, Italy, July 2-6, 2016', edited by Prof. Paolo Bernardi.