Proteasome function is inhibited by polyglutamine-expanded ataxin-1, the SCA1 gene product.

@article{Park2005ProteasomeFI,
  title={Proteasome function is inhibited by polyglutamine-expanded ataxin-1, the SCA1 gene product.},
  author={Yongjae Park and Sunghoi Hong and Sung-Jo Kim and Seongman Kang},
  journal={Molecules and cells},
  year={2005},
  volume={19 1},
  pages={23-30}
}
Spinocerebellar ataxia type 1 (SCA1) is an autosomal-dominant neurodegenerative disorder caused by expansion of the polyglutamine tract in the SCA1 gene product, ataxin-1. Using d2EGFP, a short-lived enhanced green fluorescent protein, we investigated whether polyglutamine-expanded ataxin-1 affects the function of the proteasome, a cellular multicatalytic protease that degrades most misfolded proteins and regulatory proteins. In Western blot analysis and immunofluorescence experiments, d2EGFP… CONTINUE READING

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