| Literature DB >> 25451224 |
Fengzhen Huang1, Li Zhang2, Zhe Long2, Zhao Chen2, Xuan Hou2, Chunrong Wang2, Huirong Peng2, Junling Wang2, Jiada Li3, Ranhui Duan3, Kun Xia3, De-Maw Chuang4, Beisha Tang5, Hong Jiang6.
Abstract
MicroRNAs (miRNAs) have been reported to play significant roles in the pathogenesis of various polyQ diseases. This study aims to investigate the regulation of ATXN3 gene expression by miRNA. We found that miR-25 reduced both wild-type and polyQ-expanded mutant ataxin-3 protein levels by interacting with the 3'UTR of ATXN3 mRNA. miR-25 also increased cell viability, decreased early apoptosis, and downregulated the accumulation of mutant ataxin-3 protein aggregates in SCA3/MJD cells. These novel results shed light on the potential role of miR-25 in the pathogenesis of SCA3/MJD, and provide a possible therapeutic intervention for this disorder.Entities:
Keywords: ATXN3; Cytotoxicity; Gene expression regulation; Neuronal intranuclear inclusion; Spinocerebellar ataxia type 3/Machado–Joseph disease; miR-25
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Year: 2014 PMID： 25451224 PMCID： PMC6370487 DOI： 10.1016/j.febslet.2014.11.013
Source DB: PubMed Journal: FEBS Lett ISSN： 0014-5793 Impact factor: 4.124