Article
Enhanced molecular mobility of ordinarily structured regions drives polyglutamine disease.
The Journal of biological chemistry - 2 Oct 2015
Lupton Christopher J, Steer David L, Wintrode Patrick L, Bottomley Stephen P, Hughes Victoria A, Ellisdon Andrew M
Abstract excerpt
Polyglutamine expansion is a hallmark of nine neurodegenerative diseases, with protein aggregation intrinsically linked to disease progression. Although polyglutamine expansion accelerates protein aggregation, the misfolding process is frequently instigated by flanking domains. For example, polyglutamine expansion in ataxin-3 allosterically triggers the aggregation of the catalytic Josephin domain. The molecular...
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