Bergmann glia expression of polyglutamine-expanded ataxin-7 produces neurodegeneration by impairing glutamate transport

@article{Custer2006BergmannGE,
  title={Bergmann glia expression of polyglutamine-expanded ataxin-7 produces neurodegeneration by impairing glutamate transport},
  author={Sara K. Custer and Gwenn A Garden and Nishi Gill and Udo R{\"u}b and Randell T. Libby and Christian Schultz and Stephan J. Guyenet and Thomas Deller and Lesnick E. Westrum and Bryce L. Sopher and Albert R. La Spada},
  journal={Nature Neuroscience},
  year={2006},
  volume={9},
  pages={1302-1311}
}
Non-neuronal cells may be pivotal in neurodegenerative disease, but the mechanistic basis of this effect remains ill-defined. In the polyglutamine disease spinocerebellar ataxia type 7 (SCA7), Purkinje cells undergo non-cell-autonomous degeneration in transgenic mice. We considered the possibility that glial dysfunction leads to Purkinje cell degeneration, and generated mice that express ataxin-7 in Bergmann glia of the cerebellum with the Gfa2 promoter. Bergmann glia–specific expression of… CONTINUE READING
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