Analysis of an inducible mouse model for spinocerebellar ataxia type 3
J Boy, T Schmidt, T Leergaard, F Odeh, S Nuber, C Holzmann, S Ibrahim, U Grasshoff, I Schmitt, F Zimmermann, S Prusiner, J Bjaalie, O Rieß · Aktuelle Neurologie · 2005 · Heft 10
Bibliografische Angaben
Zusammenfassung
Spinocerebellar Ataxia Type 3 (SCA3) or Machado-Joseph-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. In order to study the course of the disease we generated an inducible transgenic mouse model using the „Tet-Off-System“ developed by Dr. Bujard (Heidelberg). This system is based on two constructs: The promoter construct controls the expression of the so called tTA (Tetracycline transactivator) gene product. The binding of this protein to a Tetracycline resp…
