Genetic and pharmacological suppression of polyglutamine-dependent neuronal dysfunction in Caenorhabditis elegans

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• 作者：J. Alex Parker (1)
  Sébastien Holbert (1)
  Emmanuel Lambert (1)
  Salima Abderrahmane (1)
  Christian Néri (1)
  
• 关键词：Polyglutamine expansion ; neuronal dysfunction ; C. elegans ; genetic suppression ; drug screening
• 刊名：Journal of Molecular Neuroscience
• 出版年：2004
• 出版时间：February 2004
• 年：2004
• 卷：23
• 期：1-2
• 页码：61-67
• 全文大小：107KB
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• 作者单位：J. Alex Parker (1)
  Sébastien Holbert (1)
  Emmanuel Lambert (1)
  Salima Abderrahmane (1)
  Christian Néri (1)
  
  1. Laboratory of Genomic Biology, Inserm Avenir Group, Centre Paul Broca, 75014, Paris, France
  

文摘

The identification of disease genes for several neurodegenerative illnesses has allowed for the development of disease models in experimental organisms. We discuss our approach to studying Huntington’s disease, the best characterized of the polyglutamine (polyQ) expansion disorders. We have developed a system in Caenorhabditis elegans to study the effects of (polyQ)-dependent neuronal dysfunction at the resolution of two neurons in screening for genetic and pharmacological suppression. Our data suggest that C. elegans might be instructive in searching for targets and active compounds against polyglutamine neuronal toxicity.