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Updated: Jun 14, 2026

Growth Assays to Assess Polyglutamine Toxicity in Yeast
Published on: March 5, 2012
[Neurodegenerative polyglutamine expansion diseases: physiopathology and therapeutic strategies]
M Ravache1, G Abou-Sleymane, Y Trottier
1Département de Neurobiologie et Génétique, Institut de Génétique et de Biologie Moléculaire et Cellulaire, CNRS/Inserm, université de Strasbourg, BP 10142, 67404 Illkirch cedex, France.
Abstract:
Polyglutamine expansion diseases are adult-onset inherited neurodegenerative disorders that lead to death 10 to 20 years after the first symptoms. Currently, there is no therapy to fight against these diseases. They include Huntington's disease, spinobulbar muscular atrophy, dentatorubral-pallido-luysian atrophy and six types of spino-cerebellar ataxia. The diseases are caused by a unique mutational mechanism: an expansion of the CAG trinucleotide in the corresponding genes coding for an expanded tract of glutamine in the mutated proteins. Polyglutamine expansion confers to the mutant proteins toxic properties that cause neuronal cell death in brain regions specific to each disease. Thanks to cellular and animal models (fly, fish, mouse and rat) of these diseases, we have considerably improved our understanding of the toxic nature of polyglutamine expansion and the physiopathology, and we are now in position to design and test therapeutic strategies to prevent or delay the disease process.
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