Mouse models of human CAG repeat disorders

被引：21

作者：

Burright, EN

Orr, HT

Clark, HB

机构：

[1] UNIV MINNESOTA,DEPT LAB MED & PATHOL,MINNEAPOLIS,MN 55455

[2] UNIV MINNESOTA,DEPT BIOCHEM,MINNEAPOLIS,MN 55455

来源：

BRAIN PATHOLOGY | 1997年 / 7卷 / 03期

关键词：

D O I：

10.1111/j.1750-3639.1997.tb00896.x

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

Expansions of CAG trinucleotide repeats encoding glutamine have been found to be the causative mutations of seven human neurodegenerative diseases, Similarities in the clinical, genetic, and molecular features of these disorders suggest they share a common mechanism of pathogenesis, Recent progress in the generation and characterization of transgenic mice expressing the genes containing expanded repeats associated with spinal and bulbar muscular atrophy (SBMA), spinocerebellar ataxia type 1 (SCA1), Machado-Joseph disease (MJD/SCA3), and Huntington's disease (HD) is beginning to provide insight into the underlying mechanisms of these neurodegenerative disorders.

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收藏

页码：965 / 977

页数：13

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