期刊
JOURNAL OF NEUROCHEMISTRY
卷 126, 期 -, 页码 125-141出版社
WILEY
DOI: 10.1111/jnc.12322
关键词
clinical trials; co-enzyme Q(10); Friedreich's ataxia; idebenone
资金
- European Commission [HEALTH-F2-2010-242193]
- Department of Health's National Institute for Health Research Biomedical Research Centers funding scheme
- Dementias and Neurodegenerative Diseases Research Network (DeNDRoN)
- Santhera
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 15years, 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 Q(10) and idebenone.
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