A transgenic mouse model of spinocerebellar ataxia type 3 resembling late disease onset and gender-specific instability of CAG repeats

A transgenic mouse model of spinocerebellar ataxia type 3 resembling late disease onset and gender-specific instability of CAG repeats

Spinocerebellar ataxia type 3 (SCA3), or Machado–Joseph disease (MJD), is caused by the expansion of a polyglutamine repeat in the ataxin-3 protein. We generated a mouse model of SCA3 expressing ataxin-3 with 148 CAG repeats under the control of the huntingtin promoter, resulting in ubiquitous expre...

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Bibliographic Details
Main Authors: Jana Boy, Thorsten Schmidt, Ulrike Schumann, Ute Grasshoff, Samy Unser, Carsten Holzmann, Ina Schmitt, Tim Karl, Franco Laccone, Hartwig Wolburg, Saleh Ibrahim, Olaf Riess
Format: Article
Language:English
Published: Elsevier 2010-02-01
Series:Neurobiology of Disease
Subjects:
Spinocerebellar ataxia type 3
Machado–Joseph disease
SCA3
MJD
Polyglutamine
Intranuclear inclusion bodies
Online Access:http://www.sciencedirect.com/science/article/pii/S0969996109002204

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