ATXN2-CAG42 sequesters PABPC1 into insolubility and induces FBXW8 in cerebellum of old ataxic knock-in mice.

Spinocerebellar Ataxia Type 2 (SCA2) is caused by expansion of a polyglutamine encoding triplet repeat in the human ATXN2 gene beyond (CAG)(31). This is thought to mediate toxic gain-of-function by protein aggregation and to affect RNA processing, resulting in degenerative processes affecting prefer...

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Bibliographic Details
Main Authors: Ewa Damrath, Melanie V Heck, Suzana Gispert, Mekhman Azizov, Joachim Nowock, Carola Seifried, Udo Rüb, Michael Walter, Georg Auburger
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2012-01-01
Series:PLoS Genetics
Online Access:http://europepmc.org/articles/PMC3431311?pdf=render