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- Title
miR-19, miR-101 and miR-130 co-regulate ATXN1 levels to potentially modulate SCA1 pathogenesis.
- Authors
Lee, Yoontae; Samaco, Rodney C; Gatchel, Jennifer R; Thaller, Christina; Orr, Harry T; Zoghbi, Huda Y
- Abstract
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.
- Publication
Nature neuroscience, 2008, Vol 11, Issue 10, p1137
- ISSN
1546-1726
- Publication type
Journal Article
- DOI
10.1038/nn.2183