RNA-binding proteins hnRNP A2/B1 and CUGBP1 suppress fragile X CGG premutation repeat-induced neurodegeneration in a Drosophila model of FXTAS.

Abstract:

:Fragile 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.

journal_name

Neuron

journal_title

Neuron

authors

Sofola OA,Jin P,Qin Y,Duan R,Liu H,de Haro M,Nelson DL,Botas J

doi

10.1016/j.neuron.2007.07.021

subject

Has Abstract

pub_date

2007-08-16 00:00:00

pages

565-71

issue

4

eissn

0896-6273

issn

1097-4199

pii

S0896-6273(07)00542-9

journal_volume

55

pub_type

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