Huntington’s disease: from huntingtin function and dysfunction to therapeutic strategies

@article{BorrellPags2006HuntingtonsDF,
  title={Huntington’s disease: from huntingtin function and dysfunction to therapeutic strategies},
  author={Maria Borrell-Pag{\`e}s and Diana Zala and Sandrine Humbert and Fr{\'e}d{\'e}ric Saudou},
  journal={Cellular and Molecular Life Sciences CMLS},
  year={2006},
  volume={63},
  pages={2642-2660}
}
Huntington’s disease (HD) is a neurodegenerative disorder that usually starts in middle age and is characterized by involuntary movements (chorea), personality changes and dementia, leading to death within 10–20 years. The defective gene in HD contains a trinucleotide CAG repeat expansion within its coding region that expresses a polyglutamine repeat in the protein huntingtin. Together with the characteristic formation of aggregates in HD, aberrant protein interactions and several post… CONTINUE READING

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