Article
Self-assembly and conformational heterogeneity of the AXH domain of ataxin-1: an unusual example of a chameleon fold.
Biophysical journal - 19 Mar 2013
de Chiara Cesira, Rees Martin, Menon Raj P, Pauwels Kris, Lawrence Ceri, Konarev Petr V, Svergun Dmitri I, Martin Stephen R, Chen Yu Wai, Pastore Annalisa
Abstract excerpt
Ataxin-1 is a human protein responsible for spinocerebellar ataxia type 1, a hereditary disease associated with protein aggregation and misfolding. Essential for ataxin-1 aggregation is the anomalous expansion of a polyglutamine tract near the protein N-terminus, but the sequence-wise distant AXH domain modulates and contributes to the process. The AXH domain is also involved in the nonpathologic functions of the...
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