Article
Flanking domain stability modulates the aggregation kinetics of a polyglutamine disease protein.
Protein science : a publication of the Protein Society - 1 Oct 2011
Saunders Helen M, Gilis Dimitri, Rooman Marianne, Dehouck Yves, Robertson Amy L, Bottomley Stephen P
Abstract excerpt
Spinocerebellar Ataxia Type 3 (SCA3) is one of nine polyglutamine (polyQ) diseases that are all characterized by progressive neuronal dysfunction and the presence of neuronal inclusions containing aggregated polyQ protein, suggesting that protein misfolding is a key part of this disease. Ataxin-3, the causative protein of SCA3, contains a globular, structured N-terminal domain (the Josephin domain) and a flexible...
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