FB2026_01 , released March 12, 2026
FB2026_01 , released March 12, 2026
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Reference
Citation
Swanson, M.S., Orr, H.T. (2007). Fragile X tremor/ataxia syndrome: blame the messenger!  Neuron 55(4): 535--537.
FlyBase ID
FBrf0202236
Publication Type
Note
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.
PubMed ID
17698005
PubMed Central ID
DOI
10.1016/j.neuron.2007.07.032
Related Publication(s)
Research paper

Pur alpha binds to rCGG repeats and modulates repeat-mediated neurodegeneration in a Drosophila model of fragile X tremor/ataxia syndrome.
Jin et al., 2007, Neuron 55(4): 556--564 [FBrf0202366]

RNA-Binding proteins hnRNP A2/B1 and CUGBP1 suppress fragile X CGG premutation repeat-induced neurodegeneration in a Drosophila model of FXTAS.
Sofola et al., 2007, Neuron 55(4): 565--571 [FBrf0200897]

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Secondary IDs
    Language of Publication
    English
    Additional Languages of Abstract
    Parent Publication
    Publication Type
    Journal
    Abbreviation
    Neuron
    Title
    Neuron
    Publication Year
    1988-
    ISBN/ISSN
    0896-6273
    Data From Reference