Polyglutamine-expanded ataxin-7 causes cerebellar dysfunction by inducing transcriptional dysregulation.


In the present study, we prepared an animal model of adult-onset spinocerebellar ataxia type 7 (SCA7) by generating transgenic mice expressing polyglutamine-expanded ataxin-7-Q52. Mutant ataxin-7-Q52 was expressed in brain areas implicated in SCA7 neurodegeneration, including cerebellum and inferior olivary nucleus. Ataxin-7-Q52 transgenic mice exhibited… (More)
DOI: 10.1016/j.neuint.2009.11.003


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