Cleavage of huntingtin by apopain, a proapoptotic cysteine protease, is modulated by the polyglutamine tract

@article{Goldberg1996CleavageOH,
  title={Cleavage of huntingtin by apopain, a proapoptotic cysteine protease, is modulated by the polyglutamine tract},
  author={Yigal Paul Goldberg and Donald W. Nicholson and Dita M. Rasper and Michael A. Kalchman and Hiroshi B. Koide and Rona K Graham and M. Bromm and Parsa Kazemi-Esfarjani and Nancy A. Thornberry and John P. Vaillancourt and Michael R. Hayden},
  journal={Nature Genetics},
  year={1996},
  volume={13},
  pages={442-449}
}
Apoptosis has recently been recognized as a mode of cell death in Huntington disease (HD). Apopain, a human counterpart of the nematode cysteine protease death–gene product, CED–3, has a key role in proteolytic events leading to apoptosis. Here we show that apoptotic extracts and apopain itself specifically cleave the HD gene product, huntingtin. The rate of cleavage increases with the length of the huntingtin polyglutamine tract, providing an explanation for the gain–of–function associated… CONTINUE READING
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