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• W2092371719 endingPage "948" @default.
• W2092371719 startingPage "937" @default.
• W2092371719 abstract "Spinocerebellar ataxia type 1 (SCA1) is an autosomal dominant inherited disorder characterized by degeneration of cerebellar Purkinje cells, spinocerebellar tracts, and selective brainstem neurons owing to the expansion of an unstable CAG trinucleotide repeat. To gain insight into the pathogenesis of the SCA1 mutation and the intergenerational stability of trinucleotide repeats in mice, we have generated transgenic mice expressing the human SCA1 gene with either a normal or an expanded CAG tract. Both transgenes were stable in parent to offspring transmissions. While all six transgenic lines expressing the unexpanded human SCA1 allele had normal Purkinje cells, transgenic animals from five of six lines with the expanded SCA1 allele developed ataxia and Purkinje cell degeneration. These data indicate that expanded CAG repeats expressed in Purkinje cells are sufficient to produce degeneration and ataxia and demonstrate that a mouse model can be established for neurodegeneration caused by CAG repeat expansions." @default.
• W2092371719 created "2016-06-24" @default.
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• W2092371719 date "1995-09-01" @default.
• W2092371719 modified "2023-10-10" @default.
• W2092371719 title "SCA1 transgenic mice: A model for neurodegeneration caused by an expanded CAG trinucleotide repeat" @default.
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• W2092371719 doi "https://doi.org/10.1016/0092-8674(95)90273-2" @default.
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• W2092371719 hasPublicationYear "1995" @default.
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