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Genetic Suppression of Polyglutamine Toxicity in Drosophila

Science  10 Mar 2000:
Vol. 287, Issue 5459, pp. 1837-1840
DOI: 10.1126/science.287.5459.1837

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Abstract

A Drosophila model for Huntington's and other polyglutamine diseases was used to screen for genetic factors modifying the degeneration caused by expression of polyglutamine in the eye. Among 7000 P-element insertions, several suppressor strains were isolated, two of which led to the discovery of the suppressor genes described here. The predicted product of one, dHDJ1, is homologous to human heat shock protein 40/HDJ1. That of the second, dTPR2, is homologous to the human tetratricopeptide repeat protein 2. Each of these molecules contains a chaperone-related J domain. Their suppression of polyglutamine toxicity was verified in transgenic flies.

  • * To whom correspondence should be addressed. E-mail: parsa{at}its.caltech.edu

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