HDAC inhibitor sodium butyrate reverses transcriptional downregulation and ameliorates ataxic symptoms in a transgenic mouse model of SCA3

HDAC inhibitor sodium butyrate reverses transcriptional downregulation and ameliorates ataxic symptoms in a transgenic mouse model of SCA3

Spinocerebellar ataxia type 3 (SCA3) is an autosomal dominant neurodegenerative disease caused by polyglutamine-expanded ataxin-3. Previously, we prepared a SCA3 animal model by generating transgenic mice expressing disease-causing ataxin-3-Q79. Mutant ataxin-3-Q79 caused cerebellar malfunction of S...

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Bibliographic Details
Main Authors: An-Hsun Chou, Si-Ying Chen, Tu-Hsueh Yeh, Yi-Hsin Weng, Hung-Li Wang
Format: Article
Language:English
Published: Elsevier 2011-02-01
Series:Neurobiology of Disease
Subjects:
Spinocerebellar ataxia type 3
Ataxin-3
Polyglutamine-expanded ataxin-3
SCA3 transgenic mice
Cerebellum
Histone deacetylase
Online Access:http://www.sciencedirect.com/science/article/pii/S0969996110003530

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http://www.sciencedirect.com/science/article/pii/S0969996110003530

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