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• W2025078843 startingPage "387" @default.
• W2025078843 abstract "Ataxin-3 belongs to the family of polyglutamine proteins, which are associated with nine different neurodegenerative disorders. Relatively little is known about the structural and functional properties of ataxin-3, and only recently have these aspects of the protein begun to be explored. We have performed a preliminary investigation into the conserved N-terminal domain of ataxin-3, termed Josephin. We show that Josephin is a monomeric domain which folds into a globular conformation and possesses ubiquitin protease activity. In addition, we demonstrate that the presence of the polyglutamine region of the protein does not alter the structure of the protein. However, its presence destabilizes the Josephin domain. The implications of these data in the pathogenesis of polyglutamine repeat proteins are discussed." @default.
• W2025078843 created "2016-06-24" @default.
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• W2025078843 date "2004-09-01" @default.
• W2025078843 modified "2023-09-28" @default.
• W2025078843 title "Structural and functional analysis of the Josephin domain of the polyglutamine protein ataxin-3" @default.
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• W2025078843 doi "https://doi.org/10.1016/j.bbrc.2004.07.131" @default.
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