Article
SCA1 transgenic mice: a model for neurodegeneration caused by an expanded CAG trinucleotide repeat.
Cell - 22 Sept 1995
Burright E N, Clark H B, Servadio A, Matilla T, Feddersen R M, Yunis W S, Duvick L A, Zoghbi H Y, Orr H T
Abstract excerpt
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...
Topics
- Animals
- Ataxin-1
- Ataxins
- Base Sequence
- Cerebellum
- Disease Models, Animal
- Gene Expression
- Immunohistochemistry
- Mice
- Mice, Transgenic
- Molecular Sequence Data