Article
A conditional pan-neuronal Drosophila model of spinocerebellar ataxia 7 with a reversible adult phenotype suitable for identifying modifier genes.
The Journal of neuroscience : the official journal of the Society for Neuroscience - 7 Mar 2007
Latouche Morwena, Lasbleiz Christelle, Martin Elodie, Monnier Véronique, Debeir Thomas, Mouatt-Prigent Annick, Muriel Marie-Paule, Morel Lydie, Ruberg Merle, Brice Alexis, Stevanin Giovanni, Tricoire Hérvé
Abstract excerpt
Spinocerebellar ataxia 7 (SCA7) is a neurodegenerative disease caused by a polyglutamine (polyQ) expansion in the ataxin 7 (ATXN7) protein, a member of a multiprotein complex involved in histone acetylation. We have created a conditional Drosophila model of SCA7 in which expression of truncated ATXN7 (ATXN7T) with a pathogenic polyQ expansion is induced in neurons in adult flies. In this model, mutant ATXN7T...
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