A major role for side-chain polyglutamine hydrogen bonding in irreversible ataxin-3 aggregation.

A major role for side-chain polyglutamine hydrogen bonding in irreversible ataxin-3 aggregation.

The protein ataxin-3 consists of an N-terminal globular Josephin domain (JD) and an unstructured C-terminal region containing a stretch of consecutive glutamines that triggers the neurodegenerative disorder spinocerebellar ataxia type 3, when it is expanded beyond a critical threshold. The disease r...

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Bibliographic Details
Main Authors: Antonino Natalello, Anna Maria Frana, Annalisa Relini, Alessandra Apicella, Gaetano Invernizzi, Carlo Casari, Alessandra Gliozzi, Silvia Maria Doglia, Paolo Tortora, Maria Elena Regonesi
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2011-04-01
Series:PLoS ONE
Online Access:http://europepmc.org/articles/PMC3076451?pdf=render

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http://europepmc.org/articles/PMC3076451?pdf=render

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