HUNTINGTIN

作品数：29被引量：50H指数：4
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相关领域：医药卫生生物学更多>>
相关作者：秦正红顾振纶王晔林芳吴腾腾更多>>
相关机构：苏州大学复旦大学华中科技大学中国科学院大学更多>>
相关期刊：《Science China(Life Sciences)》《Acta Pharmacologica Sinica》《中华医学信息导报》《Neural Regeneration Research》更多>>
相关基金：国家自然科学基金国家教育部博士点基金中国博士后科学基金国家高技术研究发展计划更多>>
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Therapeutic advances in neural regeneration for Huntington’s disease被引量：2
《Neural Regeneration Research》2024年第9期1991-1997,共7页Francesco D’Egidio Vanessa Castelli Giorgia Lombardozzi Fabrizio Ammannito Annamaria Cimini Michele d’Angelo 
Huntington’s disease is a neurodegenerative disease caused by the expansion mutation of a cytosine-adenine-guanine triplet in the exon 1 of the HTT gene which is responsible for the production of the huntingtin (Htt)...
关键词：cell therapy EXOSOMES extracellular vesicles HUNTINGTIN Huntington’s disease medium spiny neurons neurodegenerative disease NEUROGENESIS neuronal loss stem cells 
Elevated plasma and CSF neurofilament light chain concentrations are stabilized in response to mutant huntingtin lowering in the brains of Huntington’s disease mice
《Translational Neurodegeneration》2024年第1期329-350,共22页Nicholas S.Caron Lauren M.Byrne Fanny L.Lemarié Jeffrey N.Bone Amirah E.-E.Aly Seunghyun Ko Christine Anderson Lorenzo L.Casal Austin M.Hill David J.Hawellek Peter McColgan Edward J.Wild Blair R.Leavitt Michael R.Hayden 
Support for N.S.C.and L.M.B.provided by the Huntington’s Disease Society of America Berman/Topper HD Career Development Fellowship.Project operational support for E.J.W.,B.R.L.,and M.R.H.provided by F.Hoffmann-La Roche.Additional operational support for N.S.C.and M.R.H.provided by a Bev Hartig Huntington’s Disease Foundation grant.Funding sources were not involved in the design of experiments,collection and measurement of samples,or analysis and interpretation of the data presented herein.
Background Therapeutic approaches aimed at lowering toxic mutant huntingtin(mHTT)levels in the brain can reverse disease phenotypes in animal models of Huntington’s disease(HD)and are currently being evaluated in cli...
关键词：Neurofilament light chain Response biomarker Huntington’s disease Huntingtin lowering Antisense oligonucleotide Cerebrospinal fluid PLASMA BIOFLUIDS 
Investigational treatments for neurodegenerative diseases caused by inheritance of gene mutations:lessons from recent clinical trials
《Neural Regeneration Research》2023年第8期1679-1683,共5页Bruno P.Imbimbo Viviana Triaca Camillo Imbimbo Robert Nisticò 
We reviewed recent major clinical trials with investigational drugs for the treatment of subjects with neurodegenerative diseases caused by inheritance of gene mutations or associated with genetic risk factors.Specifi...
关键词：Alzheimer's disease amyotrophic lateral sclerosis amyloid precursor protein GLUCOCEREBROSIDASE HUNTINGTIN Huntington's disease Parkinson's disease presenilin 1 presenilin 2 superoxide dismutase 1 
Altered microRNA expression in animal models of Huntington’s disease and potential therapeutic strategies被引量：1
《Neural Regeneration Research》2021年第11期2159-2169,共11页Bridget Martinez Philip V.Peplow 
A review of recent animal models of Huntington’s disease showed many microRNAs had altered expression levels in the striatum and cerebral cortex,and which were mostly downregulated.Among the altered microRNAs were mi...
关键词：animal model cerebral cortex HUNTINGTIN Huntington’s disease MICRORNA NEURODEGENERATION STRIATUM therapeutic strategies 
Oral administration of the cannabigerol derivative VCE-003.2 promotes subventricular zone neurogenesis and protects against mutant huntingtin-induced neurodegeneration
《Translational Neurodegeneration》2019年第1期111-125,共15页José Aguareles Juan Paraíso-Luna Belén Palomares Raquel Bajo-Grañeras Carmen Navarrete Andrea Ruiz-Calvo Daniel García-Rincón Elena García-Taboada Manuel Guzmán Eduardo Muñoz Ismael Galve-Roperh 
This work was supported by the MINECO grant RTC-2015-3364 to EM and IGR cofounded by the European Development Regional Fund in the Framework of the Operative Program“Reinforcement of research,technological development and innovation”;IGR was also supported by grant PI15-00310 and PI18-00941 cofinanced by the European Development Regional Fund“A way to achieve Europe”;EM by the MINECO grant SAF2017-87701-R;JA and JPL were supported by FPI and FPU program fellowship(Ministerio de Educación,Cultura y Deporte)and DGR by Fundación Tatiana de Guzmán el Bueno.BP is a predoctoral fellow supported by the i-PFIS program,Instituto de Salud CarlosⅢ(IFI15/00022,European Social Fund“Investing in your future”).
Background:The administration of certain cannabinoids provides neuroprotection in models of neurodegenerative diseases by acting through various cellular and molecular mechanisms.Many cannabinoid actions in the nervou...
关键词：CANNABINOID HUNTINGTIN NEURODEGENERATION NEUROGENESIS PPAR 
Using Huntingtin Knock-In Minipigs to Fill the Gap Between Mouse Models and Patients with Huntington's Disease
《Neuroscience Bulletin》2018年第5期870-872,共3页Xiangqian Liu Ting Peng He Li 
Huntington＇s disease （HD） is an inherited autosomal dominant neurodegenerative disease characterized by pro- gressive motor deficits, cognitive decline, and psychiatric symptoms. It is caused by a pathological expa...
关键词：HD In Using Huntingtin Knock-In Minipigs to Fill the Gap Between Mouse Models and Patients with Huntington’s Disease 
Onjisaponin B derived from Radix Polygalae enhances autophagy and accelerates the degradation of mutant α-Synuclein and Huntingtin in PC-12 cells
《中国药理学与毒理学杂志》2017年第10期1003-1004,共2页An-guo WU Betty Yuen-Kwan LAW Jian-min WU Da-lian QIN 
supported by Macao Foundation(Project code:0212);FDCT grant from the Science and Technology Development Fund of Macao(Project code:076/2011/A3)
OBJECTIVE To investigate the autophagic effect of the compounds from the Chinese medicinal herbs,Radix polygalae as a potential neuroprotective agent that enhances the clearance of mutant Huntingtin andα-synuclein in...
关键词：AUTOPHAGY Radix polygalae onjisaponin B AMPK MTOR Α-SYNUCLEIN HUNTINGTIN PC-12 
RNAi mechanisms in Huntington’s disease therapy:siRNA versus shRNA被引量：5
《Translational Neurodegeneration》2017年第1期300-309,共10页Sebastian Aguiar Bram van der Gaag Francesco Albert Bosco Cortese 
Huntington’s Disease(HD)is a genetically dominant trinucleotide repeat disorder resulting from CAG repeats within the Huntingtin(HTT)gene exceeding a normal range(>36 CAGs).Symptoms of the disease manifest in middle ...
关键词：RNAI SHRNA SIRNA Huntington’s disease Off-target effects HUNTINGTIN SILENCING 
A high-throughput-compatible assay to measure the degradation of endogenous Huntingtin proteins被引量：1
《Acta Pharmacologica Sinica》2016年第10期1307-1314,共8页Peng WU Ming-xing LU Xiao-tian CUI He-qing YANG Shen-liang YU Jian-bin ZHU Xiao-li SUN Boxun LU 
Acknowledgements The study was supported by the National High Technology Research and Development Program of China （2014AA02502） and the National Natural Science Foundation of China （NSFC 31371421 and 31422024）.
Aim： The accumulation of disease-causing proteins is a common hallmark of many neurodegenerative disorders. Measuring the degradation of such proteins using high-throughput-compatible assays is highly desired for the...
关键词：click chemistry SPAAC HIGH-THROUGHPUT PULSE-CHASE protein degradation Huntington＇s disease PolyQ 
Estrogen/Huntingtin:a novel pathway involved in neuroprotection
《Neural Regeneration Research》2016年第3期402-403,共2页Maria Teresa Nuzzo Maria Marino 
supported by Ministero dell’Istruzione,dell’Università e della Ricerca of Italy (PRIN 20109MXHMR_001);Associazione Italiana Ricerca sul Cancro (AIRC,IG#15221)
Neurodegenerative diseases（NDs）include more than 600 disease entities that are characterized by loss of specific neurons located in anatomically related functional areas which progressively lead to motor and cogniti...
关键词：programmed mitochondrial pathogenesis progressively aggregation oxidative abnormal neuronal caspase physiological 
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