Journal
SCIENCE
Volume 334, Issue 6056, Pages 690-693Publisher
AMER ASSOC ADVANCEMENT SCIENCE
DOI: 10.1126/science.1212673
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Funding
- NIH (Baylor College of Medicine-Intellectual and Developmental Disabilities Research Center) [NS27699, NS27699-20S1-ARRA, HD24064]
- NIH [1F32NS055545, NS022920, NS045667]
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Spinocerebellar ataxia type 1 (SCA1) is a fatal neurodegenerative disease caused by expansion of a translated CAG repeat in Ataxin-1 (ATXN1). To determine the long-term effects of exercise, we implemented a mild exercise regimen in a mouse model of SCA1 and found a considerable improvement in survival accompanied by up-regulation of epidermal growth factor and consequential down-regulation of Capicua, which is an ATXN1 interactor. Offspring of Capicua mutant mice bred to SCA1 mice showed significant improvement of all disease phenotypes. Although polyglutamine-expanded Atxn1 caused some loss of Capicua function, further reduction of Capicua levels-either genetically or by exercise-mitigated the disease phenotypes by dampening the toxic gain of function. Thus, exercise might have long-term beneficial effects in other ataxias and neurodegenerative diseases.
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