Transgenic animal models for study of the pathogenesis of Huntington’s disease and therapy

@inproceedings{Chang2015TransgenicAM,
  title={Transgenic animal models for study of the pathogenesis of Huntington’s disease and therapy},
  author={Renbao Chang and Xudong Liu and Shihua Li and Xiao-Jiang Li},
  booktitle={Drug design, development and therapy},
  year={2015}
}
Huntington's disease (HD) is caused by a genetic mutation that results in polyglutamine expansion in the N-terminal regions of huntingtin. As a result, this polyQ expansion leads to the misfolding and aggregation of mutant huntingtin as well as age-dependent neurodegeneration. The genetic mutation in HD allows for generating a variety of animal models that express different forms of mutant huntingtin and show differential pathology. Studies of these animal models have provided an important… CONTINUE READING
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Neuronal targets for reducing mutant huntingtin expression to ameliorate disease in a mouse model of Huntington’s disease

  • N Wang, M Gray, XH Lu
  • Nat Med
  • 2014
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