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Treating mouse models of Huntington disease

Sara J Tallaksen-Greene, Roger L Albin

    Neuropsychopharmacology : Official Publication of the American College of Neuropsychopharmacology
    |August 5, 2011
    PubMed
    Abstract

    No abstract available in PubMed .

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    Huntington disease or HD is a progressive, fatal neurodegenerative disorder inherited in an autosomal dominant pattern.PathophysiologyIt is caused by expansion of the CAG trinucleotide repeat in the HTT gene on chromosome 4 (4p16.3), producing an abnormal huntingtin protein with an expanded polyglutamine tract. This misfolded protein disrupts cellular function, leading to neuronal death. Normal alleles have ≤26 repeats, 27–35 are intermediate (risk of expansion), 36–39 show reduced penetrance,...

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