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- Title
Co-enzyme Q<sub>10</sub> and idebenone use in Friedreich's ataxia.
- Authors
Parkinson, Michael H; Schulz, Jörg B.; Giunti, Paola
- 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.
- Subjects
UBIQUINONES; NEURODEGENERATION; ADENOSINE triphosphate; FRIEDREICH'S ataxia; CLINICAL trials; CARDIOMYOPATHIES; FRATAXIN; MITOCHONDRIAL proteins; OXIDATIVE stress
- Publication
Journal of Neurochemistry, 2013, Vol 126, p125
- ISSN
0022-3042
- Publication type
Article
- DOI
10.1111/jnc.12322