Journal
NATURE NEUROSCIENCE
Volume 11, Issue 10, Pages 1137-1139Publisher
NATURE PUBLISHING GROUP
DOI: 10.1038/nn.2183
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Funding
- US National Institutes of Health [NS27699, HD24064, NS22920]
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Spinocerebellar ataxia type 1 is caused by expansion of a translated CAG repeat in ataxin1 (ATXN1). The level of the polyglutamine-expanded protein is one of the factors that contributes to disease severity. Here we found that miR-19, miR-101 and miR-130 co-regulate ataxin1 levels and that their inhibition enhanced the cytotoxicity of polyglutamine-expanded ATXN1 in human cells. We provide a new candidate mechanism for modulating the pathogenesis of neurodegenerative diseases sensitive to protein dosage.
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