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RNA-binding proteins hnRNP A2/B1 and CUGBP1 suppress fragile X CGG premutation repeat-induced neurodegeneration in a Drosophila model of FXTAS
journal contribution
posted on 2023-06-09, 08:33 authored by Oyinkan Adesakin, Peng Jin, Yunlong Qin, Ranhui Duan, Huijie Liu, Maria de Haro, David Nelson, Juan BotasFragile X-associated tremor/ataxia syndrome (FXTAS) is a recently described neurodegenerative disorder of older adult carriers of premutation alleles (60–200 CGG repeats) in the fragile X mental retardation gene (FMR1). It has been proposed that FXTAS is an RNA-mediated neurodegenerative disease caused by the titration of RNA-binding proteins by the CGG repeats. To test this hypothesis, we utilize a transgenic Drosophila model of FXTAS that expresses a premutation-length repeat (90 CGG repeats) from the 5' UTR of the human FMR1 gene and displays neuronal degeneration. Here, we show that overexpression of RNA-binding proteins hnRNP A2/B1 and CUGBP1 suppresses the phenotype of the CGG transgenic fly. Furthermore, we show that hnRNP A2/B1 directly interacts with riboCGG repeats and that the CUGBP1 protein interacts with the riboCGG repeats via hnRNP A2/B1.
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Publication status
- Published
Journal
NeuronISSN
0896-6273Publisher
ElsevierExternal DOI
Issue
4Volume
55Page range
565-571Department affiliated with
- Neuroscience Publications
Full text available
- No
Peer reviewed?
- Yes
Legacy Posted Date
2017-11-02First Open Access (FOA) Date
2017-11-02First Compliant Deposit (FCD) Date
2017-11-01Usage metrics
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