Pur α Binds to rCGG Repeats and Modulates Repeat-Mediated Neurodegeneration in a Drosophila Model of Fragile X Tremor/Ataxia Syndrome
نویسندگان
چکیده
Peng Jin,* Ranhui Duan, Abrar Qurashi, Yunlong Qin, Donghua Tian, Tracie C. Rosser, Huijie Liu, Yue Feng, and Stephen T. Warren* 1 Department of Human Genetics 2 Departments of Biochemistry and Pediatrics 3 Department of Pharmacology Emory University School of Medicine, Atlanta, GA 30322, USA 4 These authors contributed equally to this work. *Correspondence: [email protected] (P.J.), [email protected] (S.T.W.) DOI 10.1016/j.neuron.2007.07.020
منابع مشابه
Pur alpha binds to rCGG repeats and modulates repeat-mediated neurodegeneration in a Drosophila model of fragile X tremor/ataxia syndrome.
Fragile X-associated tremor/ataxia syndrome (FXTAS) is a recently recognized neurodegenerative disorder in fragile X premutation carriers with FMR1 alleles containing 55-200 CGG repeats. Previously, we developed a Drosophila model of FXTAS and demonstrated that transcribed premutation repeats alone are sufficient to cause neurodegeneration, suggesting that rCGG-repeat-binding proteins (RBPs) ma...
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Fragile X-associated tremor/ataxia syndrome (FXTAS) is a neurodegenerative disorder seen in Fragile X premutation carriers. Previous studies found that Fragile X rCGG repeats are sufficient to cause neurodegeneration and that the rCGG repeat-binding proteins Pur α and hnRNP A2/B1 can modulate rCGG-mediated neuronal toxicity. To explore the role of Pur α in rCGG-mediated neurodegeneration furthe...
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Fragile X-associated tremor/ataxia syndrome (FXTAS) is a progressive neurodegenerative disorder recognized in fragile X premutation carriers. Using Drosophila, we previously identified elongated non-coding CGG repeats in FMR1 allele as the pathogenic cause of FXTAS. Here, we use this same FXTAS Drosophila model to conduct a chemical screen that reveals small molecules that can ameliorate the to...
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ورودعنوان ژورنال:
- Neuron
دوره 55 شماره
صفحات -
تاریخ انتشار 2007