Article
Reduction of Purkinje cell pathology in SCA1 transgenic mice by p53 deletion.
Neurobiology of disease - 1 Dec 2001
Shahbazian M D, Orr H T, Zoghbi H Y
Abstract excerpt
The expansion of a polyglutamine tract in the ataxin-1 protein beyond a critical threshold causes spinocerebellar ataxia type 1 (SCA1). To investigate the mechanism of neuronal degeneration in SCA1, we analyzed the phenotype of an SCA1 transgenic mouse model in the absence of p53, an important regulator of cell death. p53 deficiency did not affect the early features of SCA1 mice such as impaired motor...
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