Genes Against Polyglutamine Toxicity

Kazemi-Esfarjani and Benzer of California Institute of Technology report in today's Science the discovery of two genes that protect against eye degeneration resulting from aggregation of polyglutamines in Drosophila. Polyglutamine toxicity is thought to cause Huntington's and several other hereditary neurodegenerative diseases, and understanding genetic mechanisms that block this toxicity may point the way to eventual therapies for these disorders. The authors of the present study developed Drosophila lines with polyglutamine accumulations that caused severe eye degeneration, and then screened for genetic strains that altered the course of the degeneration to uncover the two protective genes.—Hakon Heimer

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Papers

  1. Gan L, Mucke L. Paths of convergence: sirtuins in aging and neurodegeneration. Neuron. 2008 Apr 10;58(1):10-4. PubMed.

Primary Papers

  1. Kazemi-Esfarjani P, Benzer S. Genetic suppression of polyglutamine toxicity in Drosophila. Science. 2000 Mar 10;287(5459):1837-40. PubMed.

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