Are neurodegerative processes in SCA3 reversible? A study using transgenic mouse models
J Boy, T Schmidt, U Schumann, J Horst, TB Leergaard, F Odeh, S Nuber, S Beck, C Holzmann, S Ibrahim, U Grasshoff, I Schmitt, F Zimmermann, M Seeliger, S Prusiner, JG Bjaalie, O Rieß · Aktuelle Neurologie · 2007 · 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 “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 responde…
