Journal
NEURON
Volume 55, Issue 4, Pages 565-571Publisher
CELL PRESS
DOI: 10.1016/j.neuron.2007.07.021
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Funding
- NICHD NIH HHS [P50 HD024064, P30 HD024064, R01 HD038038] Funding Source: Medline
- NIMH NIH HHS [R01 MH076090] Funding Source: Medline
- NINDS NIH HHS [R01 NS042179, R56 NS042179, NS42179, R01 NS042179-05, R01 NS051630] Funding Source: Medline
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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 neuroclegenerative 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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