POLYGLUTAMINE

作品数:13被引量:11H指数:2
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相关领域:生物学医药卫生更多>>
相关作者:胡红雨林芳秦正红何晓辉贾娜丽更多>>
相关机构:中国科学院上海生命科学研究院苏州大学中国科学技术大学华南理工大学更多>>
相关期刊:《Acta Biochimica et Biophysica Sinica》《Open Journal of Genetics》《Science China(Life Sciences)》《中国组织化学与细胞化学杂志》更多>>
相关基金:国家自然科学基金国家教育部博士点基金更多>>
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Profiling neuroprotective potential of trehalose in animal models of neurodegenerative diseases:a systematic review
《Neural Regeneration Research》2023年第6期1179-1185,共7页Kah Hui Yap Shahrul Azmin Suzana Makpol Hanafi Ahmad Damanhuri Muzaimi Mustapha Jemaima Che Hamzah Norlinah Mohamed Ibrahim 
supported by Dana Impak Perdana Grant(DIP-2019-007)received by NMI from Universiti Kebangsaan Malaysia.
Trehalose,a unique nonreducing crystalline disaccharide,is a potential disease-modifying treatment for neurodegenerative diseases associated with protein misfolding and aggregation due to aging,intrinsic mutations,or ...
关键词:amyotrophic lateral sclerosis autophagy neurodegenerative disease NEUROINFLAMMATION polyglutamine tract protein refolding spinocerebellar ataxia SYNUCLEINOPATHY tau pathology TREHALOSE 
Cell-based therapeutic strategies for treatment of spinocerebellar ataxias:an update被引量:2
《Neural Regeneration Research》2023年第6期1203-1212,共10页Joana Sofia Correia Sara Duarte-Silva António JoséSalgado Patrícia Maciel 
funded by national funds;through the Foundation for Science and Technology (FCT)-project UIDB/50026/2020 and UIDP/50026/2020;by the National Ataxia Foundation (NAF)
Spinocerebellar ataxias are heritable neurodegenerative diseases caused by a cytosine-adenine-guanine expansion,which encodes a long glutamine tract(polyglutamine)in the respective wild-type protein causing misfolding...
关键词:cell transplantation ENGRAFTMENT induced pluripotent stem cells mesenchymal stem cells neural progenitor cells NEUROPROTECTION polyglutamine spinocerebellar ataxias SECRETOME spinocerebellar ataxia stem cell therapy 
Coaggregation of polyglutamine(polyQ)proteins is mediated by polyQ-tract interactions and impairs cellular proteostasis
《Acta Biochimica et Biophysica Sinica》2023年第5期736-748,共13页Jun-Ye Hong Jian-Yang Wang Hong-Wei Yue Xiang-Le Zhang Shu-Xian Zhang Lei-Lei Jiang Hong-Yu Hu 
supported by the grants from the National Natural Science Foundation of China(Nos.31470758 and 31870764).
Nine polyglutamine(polyQ)proteins have already been identified that are considered to be associated with the pathologies of neurodegenerative disorders called polyQ diseases,but whether these polyQ proteins mutually i...
关键词:POLYGLUTAMINE COAGGREGATION protein interaction PROTEOSTASIS SEQUESTRATION 
Synthetic Lethality Induced by Toxic Polyglutamine Tract II: A Survey in Drosophila
《Open Journal of Genetics》2015年第2期58-70,共13页Ping Zhang Daniel Camacho Shashank Vodapally Shanado Williams Kavitha Kannan 
Mutant proteins containing an expanded polyglutamine tract induce cell death and cause neurodegenerative diseases. These toxic proteins interfere with a variety of physiological pathways, but the key interactions betw...
关键词:POLYGLUTAMINE Diseases DROSOPHILA Genetic Screen GMR-Gal4/UAS System Synthetic LETHAL Mutations 
Synthetic Lethality Induced by a Strong <i>Drosophila</i>Enhancer of Expanded Polyglutamine Tract
《Open Journal of Genetics》2014年第4期300-315,共16页Ping Zhang Qiming Wang Hannah Hughes Gino Intrieri 
Proteins containing an expanded polyglutamine tract are neurotoxins. The expanded polyglutamine proteins influence a variety of cellular functions. In Drosophila the GMR-Gal4/UAS expression system has been widely used...
关键词:POLYGLUTAMINE Diseases DROSOPHILA Genetic Screen GMR-Gal4/UAS System Synthetic LETHAL Mutations 
Influence of Species Differences on the Neuropathology of Transgenic Huntington's Disease Animal Models
《Journal of Genetics and Genomics》2012年第6期239-245,共7页Xiao-Jiang Li Shihua Li 
supported by NIH grants NS036232, AG019206,NS041669 for X.J.L. and AG031153 for S.H.L.
Transgenic animal models have revealed much about the pathogenesis of age-dependent neurodegenerative diseases and proved to be a useful tool for uncovering therapeutic targets. Huntington's disease is a well-charact...
关键词:AGING Huntington's disease NEURODEGENERATION Species differences POLYGLUTAMINE Transgenic animals 
Truncated N-terminal huntingtin fragment with expanded-polyglutamine (htt552-100Q) suppresses brain-derived neurotrophic factor transcription in astrocytes
《Acta Biochimica et Biophysica Sinica》2012年第3期249-258,共10页Linhui Wang Fang Lin Jin Wang Junchao Wu Rong Han Lujia Zhu Guoxing Zhang Marian DiFiglia Zhenghong Qin 
Although huntingtin (htt) can be cleaved at many sites by caspases, calpains, and aspartyl proteases, amino acid (aa) 552 was defined as a preferred site for cleavage in human Huntington disease (HD) brains in v...
关键词:Huntington's disease ASTROCYTES brainderived neurotrophic factor HUNTINGTIN TRANSCRIPTION 
Essential sequence of the N-terminal cytoplasmic localization-related domain of huntingtin and its effect on huntingtin aggregates被引量:4
《Science China(Life Sciences)》2011年第4期342-350,共9页YAN YaPing PENG DanTao TIAN Jun CHI JingWei TAN JieQiong YIN XinZhen PU JiaLi XIA Kun ZHANG BaoRong 
supported by the National Natural Science Foundation of China(Grant Nos.30770761 and 30971000)
Huntington's disease (HD) is caused by abnormal CAG repeat expansion in the 5'-end of the Huntingtin (HTT) gene. In addition to the canonical C-terminal full-length huntingtin (htt) nuclear export signal, a cy...
关键词:HUNTINGTIN AGGREGATES cytoplasmic localization related domain mitochondria POLYGLUTAMINE 
Polyglutamine toxicity in non-neuronal cells被引量:1
《Cell Research》2010年第4期400-407,共8页Jennifer W Bradford Shihua Li Xiao-Jiang Li 
The neurodegenerative polyglutamine diseases are caused various disease proteins. Although these mutant proteins are by an expansion of unstable polyglutamine repeats in expressed ubiquitously in neuronal and non-neur...
关键词:POLYGLUTAMINE Huntington's disease NEURODEGENERATION GLIA MISFOLDING AGGREGATION 
Current understanding on the pathogenesis of polyglutamine diseases被引量:2
《Neuroscience Bulletin》2010年第3期247-256,共10页何晓辉 林芳 秦正红 
supported by the grants from the National Natural Science Foundation of China(No.30600197);the Specialized Research Fund for the Doctoral Program of Higher Education of China(No.20050285017)
Polyglutamine (polyQ) diseases are a family of neurodegenerative disorders including Huntington’s disease, spinobulbar muscular atrophy,dentatorubral-pallidoluysian atrophy and several spinocerebellar ataxias.polyQ...
关键词:POLYGLUTAMINE the central nervous system neurodegenerative diseases late-onset disorders UBIQUITIN autophagy 
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