Update on huntington’s disease

被引：0
作者：
Sarah B. Berman
J. Timothy Greenamyre
机构：
[1] Pittsburgh Institute for Neurodegenerative Disease,University of Pittsburgh Department of Neurology
来源：
Current Neurology and Neuroscience Reports | 2006年 / 6卷
关键词：
Huntington Disease; Polyglutamine; Celastrol; Tetrabenazine; Mutant Huntingtin;
D O I：
暂无
中图分类号：
学科分类号：
摘要：
Huntington’s disease (HD) is a devastating neurodegenerative disease causing progressive movement disorders, cognitive dysfunction, and behavioral changes. Since the causative mutation of an expanded polyglutamine repeat in the huntingtin gene was identified, significant progress has been achieved in elucidating pathogenic mechanisms. This review summarizes recent developments in evaluating the role of abnormal protein aggregation, transcriptional dysregulation, mitochondrial and bioenergetic dysfunction, excitotoxicity, and abnormal cellular trafficking in the pathogenesis of HD. In addition, although therapeutic options in HD have been limited, progress in developing targeted therapies continues, and these advancements and future directions are reviewed.
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页码：281 / 286
页数：5
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