Article
A novel transgenic rat model for spinocerebellar ataxia type 17 recapitulates neuropathological changes and supplies in vivo imaging biomarkers.
The Journal of neuroscience : the official journal of the Society for Neuroscience - 22 May 2013
Kelp Alexandra, Koeppen Arnulf H, Petrasch-Parwez Elisabeth, Calaminus Carsten, Bauer Claudia, Portal Esteban, Yu-Taeger Libo, Pichler Bernd, Bauer Peter, Riess Olaf, Nguyen Huu Phuc
Abstract excerpt
Spinocerebellar ataxia 17 (SCA17) is an autosomal-dominant, late-onset neurodegenerative disorder caused by an expanded polyglutamine (polyQ) repeat in the TATA-box-binding protein (TBP). To further investigate this devastating disease, we sought to create a first transgenic rat model for SCA17 that carries a full human cDNA fragment of the TBP gene with 64 CAA/CAG repeats (TBPQ64). In line with previous...
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