Formation of Neuronal Intranuclear Inclusions Underlies the Neurological Dysfunction in Mice Transgenic for the HD Mutation

@article{Davies1997FormationON,
  title={Formation of Neuronal Intranuclear Inclusions Underlies the Neurological Dysfunction in Mice Transgenic for the HD Mutation},
  author={S. W. Davies and M. Turmaine and B. Cozens and M. DiFiglia and A. Sharp and C. Ross and E. Scherzinger and E. Wanker and L. Mangiarini and G. Bates},
  journal={Cell},
  year={1997},
  volume={90},
  pages={537-548}
}
Huntington's disease (HD) is one of an increasing number of human neurodegenerative disorders caused by a CAG/polyglutamine-repeat expansion. The mutation occurs in a gene of unknown function that is expressed in a wide range of tissues. The molecular mechanism responsible for the delayed onset, selective pattern of neuropathology, and cell death observed in HD has not been described. We have observed that mice transgenic for exon 1 of the human HD gene carrying (CAG)115 to (CAG)156 repeat… Expand

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Nonapoptotic neurodegeneration in a transgenic mouse model of Huntington's disease.
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  • Philosophical transactions of the Royal Society of London. Series B, Biological sciences
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Characterization of a Drosophila model of Huntington's disease
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Selective neuronal degeneration in Huntington's disease.
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