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Transgenic Rat Models of Huntington's Disease

João Casaca Carreira1, Ali Jahanshahi, Dagmar Zeef

  • 1Departments of Neuroscience and Neurosurgery, Maastricht University Medical Center, Maastricht, The Netherlands.

Current Topics in Behavioral Neurosciences
|September 10, 2013
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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,...