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Friedreich's Ataxia (FRDA) is a devastating orphan disease, with no specific treatment. The disease is caused by reduced expression of the protein frataxin, which results in mitochondrial defects and oxidative damage. Levels of residual frataxin critically affect onset and progression of the disease. Understanding the molecular mechanisms that regulate frataxin stability and degradation, may therefore be exploited for the design of effective therapeutics. Here we show that frataxin is degradeddoi:10.1093/hmg/ddq566 pmid:21216878 fatcat:gupwrtxamzezfmgn36667j7omy