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Reference Report
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| Citation | Qurashi, A., Liu, H., Ray, L., Nelson, D.L., Duan, R., Jin, P. (2012). Chemical screen reveals small molecules suppressing fragile X premutation rCGG repeat-mediated neurodegeneration in Drosophila. Hum. Mol. Genet. 21(9): 2068--2075. (Export to RIS) | ||
| FlyBase ID | FBrf0217893 | ||
| Publication Type | Research paper | ||
| PubMed ID | 22298836 | ||
| PubMed Abstract | 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 toxic effects of fragile X premutation ribo-CGG (rCGG) repeats, among them several known phospholipase A(2) (PLA(2)) inhibitors. We show that specific inhibition of PLA(2) activity could mitigate the neuronal deficits caused by fragile X premutation rCGG repeats, including lethality and locomotion deficits. Furthermore, through a genetic screen, we identified a PLA(2) Drosophila ortholog that specifically modulates rCGG repeat-mediated neuronal toxicity. Our results demonstrate the utility of Drosophila models for unbiased small molecule screens and point to PLA(2) as a possible therapeutic target to treat FXTAS. | ||
| DOI | 10.1093/hmg/dds024 | ||
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| Language of Publication | English | ||
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| Publication Type | Journal | ||
| Abbreviation | Hum. Mol. Genet. | ||
| Title | Human Molecular Genetics | ||
| Publication Year | 1992- | ||
| ISBN/ISSN | 0964-6906 | ||
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Data from Reference
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| Genes (16)
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