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Protein environment : a crucial triggering factor in Josephin domain aggregation : the role of 2,2,2-trifluoroethanol

Visentin, Cristina ; Navarro, Susanna ; Grasso, Gianvito ; Regonesi, Maria Elena ; Deriu, Marco Agostino ; Tortora, Paolo ; Ventura, Salvador

In: International journal of molecular sciences, 2018, vol. 19, no. 8, p. 2151

The protein ataxin-3 contains a polyglutamine stretch that triggers amyloid aggregation when it is expanded beyond a critical threshold. This results in the onset of the spinocerebellar ataxia type 3. The protein consists of the globular N-terminal Josephin domain and a disordered C-terminal tail where the polyglutamine stretch is located. Expanded ataxin-3 aggregates via a two-stage...