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Cerebellar Soluble Mutant Ataxin-3 Level Decreases during Disease Progression in Spinocerebellar Ataxia Type 3 Mice

Spinocerebellar Ataxia Type 3 (SCA3), also known as Machado-Joseph disease, is an autosomal dominantly inherited neurodegenerative disease caused by an expanded polyglutamine stretch in the ataxin-3 protein. A pathological hallmark of the disease is cerebellar and brainstem atrophy, which correlates...

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Detalles Bibliográficos
Autores principales: Nguyen, Huu Phuc, Hübener, Jeannette, Weber, Jonasz Jeremiasz, Grueninger, Stephan, Riess, Olaf, Weiss, Andreas
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2013
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3633920/
https://www.ncbi.nlm.nih.gov/pubmed/23626768
http://dx.doi.org/10.1371/journal.pone.0062043