miR-19, miR-101 and miR-130 co-regulate ATXN1 levels to potentially modulate SCA1 pathogenesis
SCIE
SCOPUS
- Title
- miR-19, miR-101 and miR-130 co-regulate ATXN1 levels to potentially modulate SCA1 pathogenesis
- Authors
- Lee, Y; Samaco, RC; Gatchel, JR; Thaller, C; Orr, HT; Zoghbi, HY
- Date Issued
- 2008-10
- Publisher
- nature publishing group
- 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.
- Keywords
- SPINOCEREBELLAR ATAXIA TYPE-1; TRANSGENIC MICE; TRINUCLEOTIDE REPEAT; NEURODEGENERATION; EXPANSION; MICRORNAS; MODEL
- URI
- https://oasis.postech.ac.kr/handle/2014.oak/16909
- DOI
- 10.1038/NN.2183
- ISSN
- 1097-6256
- Article Type
- Article
- Citation
- Nature Neuroscience, vol. 11, no. 10, page. 1137 - 1139, 2008-10
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- There are no files associated with this item.
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