Comment
doi: 10.1016/j.neuron.2007.07.032.
Fragile X tremor/ataxia syndrome: blame the messenger!
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- PMID: 17698005
- DOI: 10.1016/j.neuron.2007.07.032
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Comment
Fragile X tremor/ataxia syndrome: blame the messenger!
Neuron.
.
Free article
Abstract
rCGG repeats in premutant alleles of the fragile X gene (FMR1) cause neurodegeneration in Drosophila and are thought to cause fragile X-associated tremor/ataxia syndrome in humans. Two reports in this issue of Neuron (Jin et al. and Sofola et al.) present data indicating a disease mechanism involving disruption of RNA-binding protein function.
Comment on
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Pur alpha binds to rCGG repeats and modulates repeat-mediated neurodegeneration in a Drosophila model of fragile X tremor/ataxia syndrome.Neuron. 2007 Aug 16;55(4):556-64. doi: 10.1016/j.neuron.2007.07.020. Neuron. 2007. PMID: 17698009 Free PMC article.
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RNA-binding proteins hnRNP A2/B1 and CUGBP1 suppress fragile X CGG premutation repeat-induced neurodegeneration in a Drosophila model of FXTAS.Neuron. 2007 Aug 16;55(4):565-71. doi: 10.1016/j.neuron.2007.07.021. Neuron. 2007. PMID: 17698010 Free PMC article.
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