Therapeutic prospects for the prevention of neurodegeneration Huntington's disease and the polyglutamine repeat disorders

被引:4
作者
Kimura, Yoko
Lee, Wyan-Ching Mimi
Littleton, J. Troy
机构
[1] MIT, Picower Inst Learning & Memory, Dept Biol, Dept Brain & Cognit Sci, Cambridge, MA 02139 USA
[2] Tokyo Metropolitan Inst Med Sci, Lab Frontier Sci, Bunkyo Ku, Tokyo 1138613, Japan
关键词
axonal transport; neurodegeneration; vesicle trafficking; protein aggregation; polyQ; Huntington's disease;
D O I
10.2174/138955707779317867
中图分类号
R914 [药物化学];
学科分类号
100701 ;
摘要
Huntington's disease (HD) is a dominantly inherited neurodegenerative disorder caused by expansion of a polyglutamine (polyQ) tract in the huntingtin protein, resulting in intracellular aggregate formation and neurodegeneration. Biochemical pathways leading from polyQ expansion to disease pathogenesis are largely unknown. Recent approaches using genetic models systems have begun to uncover nuclear and cytoplasmic pathologies that represent potential targets for therapeutic intervention.
引用
收藏
页码:99 / 106
页数:8
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