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...
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 |
Similar Items
-
HDAC inhibitor sodium butyrate reverses transcriptional downregulation and ameliorates ataxic symptoms in a transgenic mouse model of SCA3
by: An-Hsun Chou, et al.
Published: (2011-02-01) -
Polyglutamine-expanded ataxin-3 activates mitochondrial apoptotic pathway by upregulating Bax and downregulating Bcl-xL
by: An-Hsun Chou, et al.
Published: (2006-02-01) -
Polyglutamine-induced neurodegeneration in SCA3 is not mitigated by non-expanded ataxin-3: Conclusions from double-transgenic mouse models
by: Jeannette Hübener, et al.
Published: (2010-04-01) -
Ataxin-3 protein modification as a treatment strategy for spinocerebellar ataxia type 3: Removal of the CAG containing exon
by: Melvin M. Evers, et al.
Published: (2013-10-01) -
Tissue-Specific Vulnerability to Apoptosis in Machado-Joseph Disease
by: Ana F. Ferreira, et al.
Published: (2023-05-01)