Abstract
Friedreich's Ataxia (FA) is the most common recessive ataxia, affecting 1-2 in 50,000 Caucasians. FA is caused by a deficiency in frataxin, a mitochondrial protein encoded by the FRDA gene and involved in iron trafficking-related processes. The main areas affected are the central nervous system (particularly the spinocerebellar system) and cardiac tissue. Although some treatments based upon administration of anti-oxidants have proven to be successful for treating the cardiac condition, therapies aimed at alleviating the neurological degeneration have failed so far.
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