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Exercise training prevents symptomatic onset of Friedreich's ataxia

The common clinical symptoms of Friedreich’s ataxia (FRDA) include ataxia, muscle weakness, type 2 diabetes and heart failure, which are caused by impaired mitochondrial function due to the loss of frataxin (FXN) expression. Endurance exercise is the most powerful intervention for promoting mitochondrial function; however, its impact on FRDA has not been studied. The Yan Lab takes advantage of mice with genetic knockout and knock-in of the Fxn gene (KIKO mice) to investigate the impact of both endurance and resistance exercise training on the development of exercise intolerance, glucose intolerance and cardiac dysfunction and their association with changes in mitochondrial content and function, antioxidant enzyme expression as well as Fxn and its regulator expression. These preclinical studies may pave the way for clinical studies of the impact of endurance exercise in FRDA patients.  


Zhao H, Lewellen BM, Wilson RJ, Cui D, Drake JC, Zhang M, Yan Z. Long-term voluntary running prevents the onset of symptomatic Friedreich's ataxia in mice. Sci Rep. 2020 Apr 8;10(1):6095. doi: 10.1038/s41598-020-62952-6. PubMed PMID: 32269244; PubMed Central PMCID: PMC7142077.

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