Ectopic expression of the striatal-enriched GTPase Rhes elicits cerebellar degeneration and an ataxia phenotype in Huntington's disease
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- 作者：Supriya Swarnkar ; Youjun Chen ; William M. Pryor ; Neelam Shahani ; Damon T. Page ; Srinivasa Subramaniam ; ssubrama@scripps.edu" class="auth_mail" title="E-mail the corresponding author
- 关键词：Cerebellar pathology ; Ectopic expression ; Soluble Huntingtin ; Neuronal death ; Stereotaxy ; Knock-in HD mice ; Transgenic HD mice ; Rhes ; Huntington's disease ; Neurodegeneration ; Ataxia
- 刊名：Neurobiology of Disease
- 出版年：2015
- 出版时间：October 2015
- 年：2015
- 卷：82
- 期：Complete
- 页码：66-77
- 全文大小：1816 K
文摘
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Rhes deletion in N171-82Q mice protects against HD-related behavioral and anatomical deficits.
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Cerebellar Rhes expression in N171-82Q mice produces ataxia-like phenotype.
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Reintroducing Rhes into the striatum of Rhes−/−/Hdh150Q/150Q mice accelerates motor deficit.