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Splice Isoforms of the Polyglutamine Disease Protein Ataxin-3 Exhibit Similar Enzymatic yet Different Aggregation Properties

Protein context clearly influences neurotoxicity in polyglutamine diseases, but the contribution of alternative splicing to this phenomenon has rarely been investigated. Ataxin-3, a deubiquitinating enzyme and the disease protein in SCA3, is alternatively spliced to encode either a C-terminal hydrop...

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Detalles Bibliográficos
Autores principales: Harris, Ginny Marie, Dodelzon, Katerina, Gong, Lijie, Gonzalez-Alegre, Pedro, Paulson, Henry L.
Formato: Texto
Lenguaje:English
Publicado: Public Library of Science 2010
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2965175/
https://www.ncbi.nlm.nih.gov/pubmed/21060878
http://dx.doi.org/10.1371/journal.pone.0013695