Polyglutamine-expanded ataxin-3 causes cerebellar dysfunction of SCA3 transgenic mice by inducing transcriptional dysregulation

In the present study, we prepared a SCA3 animal model by generating transgenic mice expressing polyglutamine-expanded ataxin-3-Q79. Ataxin-3-Q79 was expressed in brain areas implicated in SCA3 neurodegeneration, including cerebellum, pontine nucleus and substantia nigra. Ataxin-3-Q79 transgenic mice...

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Bibliographic Details
Main Authors: An-Hsun Chou, Tu-Hsueh Yeh, Pin Ouyang, Ying-Ling Chen, Si-Ying Chen, Hung-Li Wang
Format: Article
Language:English
Published: Elsevier 2008-07-01
Series:Neurobiology of Disease
Subjects:
Online Access:http://www.sciencedirect.com/science/article/pii/S0969996108000673