A novel mouse model for spinocerebellar ataxia type 3 containing 148 polyglutamine repeats
U Bichelmeier, T Schmidt, J Hübener, J Boy, A Preuß, I Schmitt, J Wilbertz, S Ibrahim, F Laccone, O Riess · Aktuelle Neurologie · 2005 · Heft 10
Bibliografische Angaben
Zusammenfassung
Spinocerebellar Ataxia Type 3 (SCA3) or Machado-Disease (MJD) is an autosomal dominantly inherited neurodegenerative disorder caused by the expansion of a CAG stretch in the MJD1 gene encoding a polyglutamine repeat in the respective Ataxin-3 protein. SCA3 therefore belongs to the group of the so called polyglutamine diseases. In order to study the course of the disease we generated transgenic mouse models for this disorder. We used the prion protein (PrP) promotor to control the expression of the full length Ataxin-3 gene containing 70 or 148 CAG repeats, respectively. As controls transgenic mice expressing ata…
