Article
Delayed spinocerebellar ataxia in transgenic mice expressing mutant ubiquitin.
Neuropathology and applied neurobiology - 1 Feb 2006
Tsirigotis M, Tang M Y, Beyers M, Zhang M, Woulfe J, Gray D A
Abstract excerpt
Spinocerebellar ataxia type 1 (SCA1) is an incurable neurodegenerative disease resulting from loss of Purkinje neurones within the cerebellum. The ubiquitin proteasome pathway (UPP) has been implicated in SCA1 but the role of proteolysis in the disease is still poorly understood. To further investigate this issue in vivo, genetic crosses were performed between an established mouse model of SCA1 and novel strains...
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