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Article

N-terminal ataxin-3 causes neurological symptoms with inclusions, endoplasmic reticulum stress and ribosomal dislocation.

Brain : a journal of neurology - 1 Jul 2011

Hübener Jeannette, Vauti Franz, Funke Claudia, Wolburg Hartwig, Ye Yihong, Schmidt Thorsten, Wolburg-Buchholz Karen, Schmitt Ina, Gardyan Adriane, Driessen Stefan, Arnold Hans-Henning, Nguyen Huu Phuc, Riess Olaf

Abstract excerpt

Mutant ataxin-3 is aberrantly folded and proteolytically cleaved in spinocerebellar ataxia type 3. The C-terminal region of the protein includes a polyglutamine stretch that is expanded in spinocerebellar ataxia type 3. Here, we report on the analysis of an ataxin-3 mutant mouse that has been obtained by gene trap integration. The ataxin-3 fusion protein encompasses 259 N-terminal amino acids including the...

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Identifiers

PMID: 21653538
DOI: 10.1093/brain/awr118

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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