Purkinje cell expression of a mutant allele of SCA1 in transgenic mice leads to disparate effects on motor behaviors, followed by a progressive cerebellar dysfunction and histological alterations.

@article{Clark1997PurkinjeCE,
  title={Purkinje cell expression of a mutant allele of SCA1 in transgenic mice leads to disparate effects on motor behaviors, followed by a progressive cerebellar dysfunction and histological alterations.},
  author={H. Brent Clark and Eric N. Burright and Wael S. Yunis and Stephanie Larson and Christine L. Wilcox and Barbara L. Hartman and Antoni Matilla and Huda Y Zoghbi and Harry T. Orr},
  journal={The Journal of neuroscience : the official journal of the Society for Neuroscience},
  year={1997},
  volume={17 19},
  pages={
          7385-95
        }
}
Spinocerebellar ataxia type 1 (SCA1) is an autosomal dominant neurological disorder caused by the expansion of a CAG repeat encoding a polyglutamine tract. Work presented here describes the behavioral and neuropathological course seen in mutant SCA1 transgenic mice. Behavioral tests indicate that at 5 weeks of age mutant mice have an impaired performance on the rotating rod in the absence of deficits in balance and coordination. In contrast, these mutant SCA1 mice have an increased initial… CONTINUE READING

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