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: Michael H Parkinson, Jörg B Schulz, Paola Giunti
• Journal: Journal of neurochemistry (J Neurochem) Vol. 126 Suppl 1 Pg. 125-41 (Aug 2013) ISSN: 1471-4159 [Electronic] England
• PMID: 23859348 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't, Review)
• Copyright: © 2013 International Society for Neurochemistry.

Chemical References

• Antioxidants
• Ubiquinone
• coenzyme Q10
• idebenone

Topics

• Antioxidants (therapeutic use)
• Friedreich Ataxia (drug therapy)
• Humans
• Ubiquinone (analogs & derivatives, therapeutic use)