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A new humanized ataxin-3 knock-in mouse model combines the genetic features, pathogenesis of neurons and glia and late disease onset of SCA3/MJD

Spinocerebellar ataxia type 3 (SCA3/MJD) is a neurodegenerative disease triggered by the expansion of CAG repeats in the ATXN3 gene. Here, we report the generation of the first humanized ataxin-3 knock-in mouse model (Ki91), which provides insights into the neuronal and glial pathology of SCA3/MJD....

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Main Authors: Pawel M. Switonski, Wojciech J. Szlachcic, Wlodzimierz J. Krzyzosiak, Maciej Figiel
Format: Artigo
Language:Inglês
Published: Elsevier 2015-01-01
Series:Neurobiology of Disease
Subjects:
Ataxin-3
Mouse
Knock-in
Knockin
SCA3
MJD
Online Access:http://www.sciencedirect.com/science/article/pii/S0969996114002939
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