title: Co-enzyme Q10 and idebenone use in Friedreich's ataxia.
abstract: Friedreich's ataxia is a debilitating progressive neurodegenerative disease associated with cardiomyopathy and other features. The underlying cause is a deficiency of the mitochondrial protein frataxin which causes mitochondrial iron deposition, increased oxidative stress and impaired adenosine triphosphate production. Over the last 15 years, multiple clinical trials have assessed the efficacy of antioxidant agents in this disease. This article reviews trials of the two most important agents, namely co-enzyme Q10 and idebenone.
authors.affiliation: Department of Molecular Neuroscience, UCL Institute of Neurology, London, UK.
authors.forename: Michael H
authors.initials: MH
authors.lastname: Parkinson
authors.forename: Jörg B
authors.initials: JB
authors.lastname: Schulz
authors.forename: Paola
authors.initials: P
authors.lastname: Giunti
mesh_terms: D000975:Antioxidants / Q000627:therapeutic use*; D005621:Friedreich Ataxia / Q000188:drug therapy*; D006801:Humans; D014451:Ubiquinone / Q000031:analogs & derivatives* / Q000627:therapeutic use
chemical_list: D000975:Antioxidants; D014451:Ubiquinone; C024989:coenzyme Q10; C036619:idebenone
keywords: Friedreich's ataxia; clinical trials; co-enzyme Q10; idebenone