Polyglutamine neurodegeneration: protein misfolding revisited

  title={Polyglutamine neurodegeneration: protein misfolding revisited},
  author={Aislinn Joanmarie Williams and Henry L. Paulson},
  journal={Trends in Neurosciences},
Polyglutamine diseases are a major cause of neurodegeneration worldwide. Recent studies highlight the importance of protein quality control mechanisms in regulating polyglutamine-induced toxicity. Here we discuss a model of disease pathogenesis that integrates current understanding of the role of protein folding in polyglutamine disease with emerging evidence that alterations in native protein interactions contribute to toxicity. We also incorporate new findings on other age-related… CONTINUE READING


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