RNA-Binding Proteins hnRNP A2/B1 and CUGBP1 Suppress Fragile X CGG Premutation Repeat-Induced Neurodegeneration in a Drosophila Model of FXTAS
Copyright policy )RNA-Binding Proteins hnRNP A2/B1 and CUGBP1 Suppress Fragile X CGG Premutation Repeat-Induced Neurodegeneration in a Drosophila Model of FXTAS
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.
- Emory University United States
- Baylor College of Medicine United States
- Emory University School of Medicine United States
- BAYLOR COLLEGE OF MEDICINE
- EMORY UNIVERSITY
Neuroscience(all), HUMDISEASE, RNA-Binding Proteins, Neurodegenerative Diseases, Eye, MOLNEURO, Animals, Genetically Modified, Disease Models, Animal, Fragile X Mental Retardation Protein, Fragile X Syndrome, Heterogeneous-Nuclear Ribonucleoprotein Group A-B, Microscopy, Electron, Scanning, RNA, Animals, Drosophila Proteins, Immunoprecipitation, Drosophila, Trinucleotide Repeat Expansion, CELF1 Protein
Neuroscience(all), HUMDISEASE, RNA-Binding Proteins, Neurodegenerative Diseases, Eye, MOLNEURO, Animals, Genetically Modified, Disease Models, Animal, Fragile X Mental Retardation Protein, Fragile X Syndrome, Heterogeneous-Nuclear Ribonucleoprotein Group A-B, Microscopy, Electron, Scanning, RNA, Animals, Drosophila Proteins, Immunoprecipitation, Drosophila, Trinucleotide Repeat Expansion, CELF1 Protein
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