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Neurology India
Medknow Publications on behalf of the Neurological Society of India
ISSN: 0028-3886
EISSN: 0028-3886
Vol. 51, No. 2, 2003, pp. 176-182
Bioline Code: ni03058
Full paper language: English
Document type: Research Article
Document available free of charge

Neurology India, Vol. 51, No. 2, 2003, pp. 176-182

 en Review Article - Friedreich's ataxia yesterday, today and tomorrow
A. Chakravarty

Abstract

The present review traces the origin of Friedreich's Ataxia (FA) from the time of Nikolaus Friedreich in the mid-nineteenth century. The early hesitation on the part of the neurological community in accepting FA as a distinct entity, rather than a variant form of tabes dorsalis and multiple sclerosis, has been highlighted. Research within the last 6-7 years, has firmly established FA as a trinucleotide repeat disorder, the location of the offending gene, and the disease-related gene product, frataxin. Frataxin is now thought to interfere with the mitochondrial oxidative process and enhance iron accumulation. However, whether this iron accumulation is a primary causative event for symptom production is not clear and iron chelators are unlikely to be helpful in therapy. Of great promise is the use of free radical scavengers and antioxidants. One such agent idebenone, a short chain analogue of co-enzyme Q10, may have a future.

Keywords
Friedreich's Ataxia, Historical aspects, Molecular genetics, Frataxion, mitochondriopathy, Antioxidants, Idebenone

 
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