Polyglutamine-expanded ataxin-7 promotes non-cell-autonomous purkinje cell degeneration and displays proteolytic cleavage in ataxic transgenic mice.

@article{Garden2002PolyglutamineexpandedAP,
  title={Polyglutamine-expanded ataxin-7 promotes non-cell-autonomous purkinje cell degeneration and displays proteolytic cleavage in ataxic transgenic mice.},
  author={Gwenn A Garden and Randell T. Libby and Ying-Hui Fu and Yoshito Kinoshita and Jing Huang and Daniel E. Possin and Annette C. Smith and Refugio A. Martinez and Gabriel C. Fine and Sara K Grote and Carol B Ware and David D. Einum and Richard J S Morrison and Louis J. Pt{\'a}cek and Bryce L. Sopher and Albert R La Spada},
  journal={The Journal of neuroscience : the official journal of the Society for Neuroscience},
  year={2002},
  volume={22 12},
  pages={4897-905}
}
Spinocerebellar ataxia (SCA) type 7 is an inherited neurodegenerative disorder caused by expansion of a polyglutamine tract within the ataxin-7 protein. To determine the molecular basis of polyglutamine neurotoxicity in this and other related disorders, we produced SCA7 transgenic mice that express ataxin-7 with 24 or 92 glutamines in all neurons of the CNS, except for Purkinje cells. Transgenic mice expressing ataxin-7 with 92 glutamines (92Q) developed a dramatic neurological phenotype… CONTINUE READING

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