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Fragile X Tremor/Ataxia Syndrome: Blame the Messenger!

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Presentation on theme: "Fragile X Tremor/Ataxia Syndrome: Blame the Messenger!"— Presentation transcript:

1 Fragile X Tremor/Ataxia Syndrome: Blame the Messenger!
Maurice S. Swanson, Harry T. Orr  Neuron  Volume 55, Issue 4, Pages (August 2007) DOI: /j.neuron Copyright © 2007 Elsevier Inc. Terms and Conditions

2 Figure 1 FMR1 mRNA and Its Relationship to FRAXA and FXTAS
FRAXA (lower gray oval) is due to the loss of FMR1 expression and the resulting absence of its gene product FMRP, an RNA-binding protein involved in regulating translation at the synapse. In contrast, FXTAS (upper gray oval) is caused by overexpression of premutant FMR1 mRNA (center), which results in the sequestration of several RNA-binding proteins, including Pur α and the heterodimer hnRNP A2/CUGBP1, which are normally required for transcription, splicing, mRNA trafficking, and translation. Neuron  , DOI: ( /j.neuron ) Copyright © 2007 Elsevier Inc. Terms and Conditions

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