Co‐enzyme Q10 and idebenone use in Friedreich's ataxia

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...

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Bibliographic Details
Published in:Journal of neurochemistry 2013-08, Vol.126, p.125-141
Main Authors: Parkinson, Michael H, Schulz, Jörg B., Giunti, Paola
Format: Article
Language:English
Subjects:
Antioxidants - therapeutic use
Ataxia
clinical trials
co‐enzyme Q10
Enzymes
Friedreich Ataxia - drug therapy
Friedreich's ataxia
Humans
idebenone
Mitochondria
Neurodegeneration
Proteins
Ubiquinone - analogs & derivatives
Ubiquinone - therapeutic use
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Summary: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.
ISSN:0022-3042
1471-4159
DOI:10.1111/jnc.12322