Polyglutamine-expanded ataxin-3 causes cerebellar dysfunction of SCA3 transgenic mice by inducing transcriptional dysregulation.

@article{Chou2008PolyglutamineexpandedAC,
  title={Polyglutamine-expanded ataxin-3 causes cerebellar dysfunction of SCA3 transgenic mice by inducing transcriptional dysregulation.},
  author={A L Chou and T. -S. Yeh and Pin Ouyang and Ying-Ling Chen and Si-Ying Chen and Hung-Li Wang},
  journal={Neurobiology of disease},
  year={2008},
  volume={31 1},
  pages={89-101}
}
In the present study, we prepared a SCA3 animal model by generating transgenic mice expressing polyglutamine-expanded ataxin-3-Q79. Ataxin-3-Q79 was expressed in brain areas implicated in SCA3 neurodegeneration, including cerebellum, pontine nucleus and substantia nigra. Ataxin-3-Q79 transgenic mice displayed motor dysfunction with an onset age of 5-6 months, and neurological symptoms deteriorated in the following months. A prominent neuronal loss was not found in the cerebellum of 10 to 11… CONTINUE READING
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