Intranuclear neuronal inclusions in Huntington's disease and dentatorubral and pallidoluysian atrophy: correlation between the density of inclusions and IT15 CAG triplet repeat length.

@article{Becher1998IntranuclearNI,
  title={Intranuclear neuronal inclusions in Huntington's disease and dentatorubral and pallidoluysian atrophy: correlation between the density of inclusions and IT15 CAG triplet repeat length.},
  author={Mark W. Becher and Joyce A Kotzuk and Alan H. Sharp and Stephen William Davies and Gillian P Bates and Donald L. Price and Christopher A. Ross},
  journal={Neurobiology of disease},
  year={1998},
  volume={4 6},
  pages={387-97}
}
Huntington's disease (HD) is caused by CAG triplet repeat expansion in IT15 which leads to polyglutamine stretches in the HD protein product, huntingtin. The pathological hallmark of HD is the degeneration of subsets of neurons, primarily those in the striatum and neocortex. Specific morphological markers of affected cells have not been identified in patients with HD, although a unique itranuclear inclusion was recently reported in neurons of transgenic animals expressing a construct encoding… CONTINUE READING
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