Article
Neurodegeneration by polyglutamine Atrophin is not rescued by induction of autophagy.
Cell death and differentiation - 1 Oct 2010
Nisoli I, Chauvin J P, Napoletano F, Calamita P, Zanin V, Fanto M, Charroux B
Abstract excerpt
Polyglutamine pathologies are neurodegenerative diseases that manifest both general polyglutamine toxicity and mutant protein-specific effects. Dentatorubral-pallidoluysian Atrophy (DRPLA) is one of these disorders caused by mutations in the Atrophin-1 protein. We have generated several models for DRPLA in Drosophila and analysed the mechanisms of cellular and organism toxicity. Our genetic and ultrastructural...
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