Tissue Transglutaminase Selectively Modifies Proteins Associated with Truncated Mutant Huntingtin in Intact Cells

@article{Chun2001TissueTS,
  title={Tissue Transglutaminase Selectively Modifies Proteins Associated with Truncated Mutant Huntingtin in Intact Cells},
  author={Wanjoo Chun and Mathieu J Lesort and Janusz Tucholski and Peter W Faber and Gail V W Johnson},
  journal={Neurobiology of Disease},
  year={2001},
  volume={8},
  pages={391-404}
}
The cause of Huntington's disease (HD) is a pathological expansion of the polyglutamine domain within the N-terminal region of huntingtin. Neuronal intranuclear inclusions and cytoplasmic aggregates composed of the mutant huntingtin within certain neuronal populations are a characteristic hallmark of HD. However, how the expanded polyglutamine repeats of mutant huntingtin cause HD is not known. Because in vitro expanded polyglutamine repeats are excellent glutaminyl-donor substrates of tissue… CONTINUE READING
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