Gene therapy by proteasome activator, PA28γ, improves motor coordination and proteasome function in Huntington’s disease YAC128 mice
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- 作者：J. Jeona ; &dagger ; ; W. Kima ; &dagger ; ; J. Janga ; O. Isacsonb ; H. Seoa ; hseo@hanyang.ac.kr" class="auth_mail" title="E-mail the corresponding author
- 关键词：AMC ; 7-amido-4-methylcoumarin ; BDNF ; brain-derived neurotrophic factor ; EDTA ; ethylenediaminetetraacetic acid ; GFP ; green fluorescent protein ; HD ; Huntington&rsquo ; s disease ; HRP ; horseradish peroxidase ; LM ; littermate ; MT ; mutant ; NIIs ; neuronal intranuclear inclusions ; PA ; proteasome activator ; PGPH ; peptidyl-glutamyl preferring hydrolytic ; RT-PCR ; reverse transcription polymerase chain reaction ; UPS ; ubiquitin&ndash ; proteasome system ; YAC ; yeast artificial chromosome
- 刊名：Neuroscience
- 出版年：2016
- 出版时间：2 June 2016
- 年：2016
- 卷：324
- 期：Complete
- 页码：20-28
- 全文大小：1970 K
文摘
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We stereotaxically injected lenti-PA28γ virus into the striatum of mutant YAC128 HD transgenic mice and littermate controls.
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YAC128 mice showed significant increase of their peptidyl-glutamyl preferring hydrolytic (PGPH) proteasome activity.
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The number of inclusion bodies was reduced in the striatum of mutant YAC128 mice after lenti-PA28γ gene transfer.
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YAC128 mice showed increased levels of BDNF mRNA, pro-BDNF, and BDNF protein after lenti-PA28γ gene transfer.
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YAC128 mice showed significant improvement of latency to fall on the rota-rod test after lenti-PA28γ gene transfer.
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