Inducible expression of ataxin-3 in a transgenic mouse model for Spinocerebellar Ataxia Type 3
J. Boy, T. Schmidt, T.B. Leergaard, F. Odeh, S. Nuber, C. Holzmann, S. Ibrahim, I. Schmitt, S. Prusiner, J.G. Bjaalie, O. Rieß, J. Boy · Aktuelle Neurologie · 2006 · Heft 09
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 inducible “Tet-Off-System“. 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 responsive element (TRE) in the…
