Destabilization of a non-pathological variant of ataxin-3 results in fibrillogenesis via a partially folded intermediate: a model for misfolding in polyglutamine disease.

Abstract excerpt

Ataxin-3 is a member of the polyglutamine family of proteins, which are associated with at least nine different neurodegenerative diseases. In the disease state, expansion of the polyglutamine tract leads to dysfunction and death of neurons, as well as formation of proteinaceous aggregates known as nuclear inclusions. Intriguingly, both expanded and non-expanded forms of ataxin-3 are observed within these nuclear...

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