BIIB021, a synthetic Hsp90 inhibitor, induces mutant ataxin-1 degradation through the activation of heat shock factor 1
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- 作者：Ying Dinga ; Hiroaki Adachia ; b ; Masahisa Katsunoa ; Kentaro Sahashia ; Naohide Kondoa ; Madoka Iidaa ; Genki Tohnaia ; Hideaki Nakatsujia ; Gen Sobuea ; c ; sobueg@med.nagoya-u.ac.jp" class="auth_mail" title="E-mail the corresponding author
- 关键词：17-AAG ; 17-allylamino-17-demethoxygeldanamycin ; ALP ; autophagy-lysosome pathway ; AR ; androgen receptor ; ATXN1 ; ataxin-1 ; DMSO ; dimethyl sulfoxide ; DRPLA ; dentatorubral pallidoluysian atrophy ; HD ; Huntington&rsquo ; s disease ; HSE ; heat shock element ; HSF1 ; heat shock factor 1 ; HSP ; heat shock proteins ; Hsp90 ; heat shock protein 90 ; N2a ; Neuro2a ; polyQ ; polyglutamine ; RT-PCR ; reverse transcription PCR ; SBMA ; spinal and bulbar muscular atrophy ; SCA1 ; spinocerebellar ataxia type 1 ; UPS ; ubiquitin-pro
- 刊名：Neuroscience
- 出版年：2016
- 出版时间：7 July 2016
- 年：2016
- 卷：327
- 期：Complete
- 页码：20-31
- 全文大小：1602 K
文摘
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BIIB021 suppresses the abnormal accumulation of ATXN1 and its toxicity.
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BIIB021 induces HSF1 activation in the neuronal cell model of SCA1.
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Mutant ATXN1 degradation is dependent on both the proteasome and autophagy systems.
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We first identify HSF1 regulating two degradation systems in a polyQ disease model.