Ataxin-3 binds VCP/p97 and regulates retrotranslocation of ERAD substrates.

@article{Zhong2006Ataxin3BV,
  title={Ataxin-3 binds VCP/p97 and regulates retrotranslocation of ERAD substrates.},
  author={Xiaoyan Zhong and Randall Pittman},
  journal={Human molecular genetics},
  year={2006},
  volume={15 16},
  pages={
          2409-20
        }
}
Expansion of a polyglutamine tract in ataxin-3 (AT3) results in spinocerebellar ataxia type 3/Machado-Joseph disease, one of the nine polyglutamine neurodegenerative diseases. Understanding the normal functions of AT3 as well as its function in the context of expansion of the polyglutamine tract is critical for understanding the disease process. AT3 is a deubiquitylating enzyme with limited information on its cellular functions. We find that transfecting cells with AT3 increases cellular levels… CONTINUE READING

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