Abstract:Spinocerebellar ataxia type ATXN7 (SCA7) and other polyglutamine (polyQ) diseases are caused by expansions of polyQ repeats in disease specific proteins. Aggregation of the polyQ proteins resulting in various forms of cellular stress, that could induce the stress granule (SG) response, is believed to be a common pathological mechanism in these disorders. SGs can contribute to cell survival, but have also been suggested to exacerbate disease pathology by seeding protein aggregation. In this study, we show that … Show more
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