Huntington and its Role in Neuronal Degeneration

@article{Li2004HuntingtonAI,
  title={Huntington and its Role in Neuronal Degeneration},
  author={Shi-hua Li and Xiao-Jiang Li},
  journal={The Neuroscientist},
  year={2004},
  volume={10},
  pages={467 - 475}
}
Huntington’s disease results from a polyglutamine expansion in the N-terminal region of huntingtin (htt). This abnormality causes protein aggregation and leads to neurotoxicity. Despite its widespread expression in the brain and body, mutant htt causes selective neurodegeneration in Huntington’s disease patient brains. However, Huntington’s disease mouse models expressing mutant htt do not have obvious neurodegeneration despite significant neurological symptoms. Most Huntington’s disease mouse… Expand
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