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E674Q(Shanghai APP mutant),a novel amyloid precursor protein mutation,in familial late-onset Alzheimer's disease

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Genes & Diseases 2024年第2期11卷 1022-1034页

作者： Yongfang zhang Xinyi Xie Boyu Chen Lina Pan Jianping Li Wanbing Wang Jintao Wang Ran Tang Qiang Huang Xiaofen Chen Rujing Ren zhentao zhang Wei Fu Gang Wang Department of Neurology Ruijin Hospital Affiliated to Shanghai Jiao Tong University School of MedicineShanghai 200025China Department of Pharmacology and Chemical Biology Shanghai Jiao Tong University School of MedicineShanghai 200025China Department of Medicinal Chemistry School of PharmacyFudan UniversityShanghai 201203China Department of Neurology Renmin Hospital of Wuhan UniversityWuhanHubei 430060China Fujian Provincial Key Laboratory of Neurodegenerative Disease and Aging Research Institute of NeuroscienceSchool of MedicineXiamen UniversityXiamenFujian 361102China Shenzhen Research Institute of Xiamen University ShenzhenGuangdong 518063China

Identified as the pathogenic genes of Alzheimer's disease(AD),APP,PSEN1,and PSEN2 mainly lead to early-onset AD,whose course is more aggressive,and atypical symptoms are more common than sporadic ***,a novel missense mutation,APP E674Q(also named“Shanghai APP”),was detected in a Chinese index patient with typical late-onset AD(LOAD)who developed memory decline in his *** results from neuroimaging were consistent with AD,where widespread amyloidβdeposition was demonstrated in 18 F-florbetapir Positron Emission Tomography(PET).APP E674Q is close to theβ-secretase cleavage site and the well-studied Swedish APP mutation(KM670/671NL),which was predicted to be pathogenic in *** dynamics simulation indicated that the E674Q mutation resulted in a rearrangement of the interaction mode between APP and BACE1 and that the E674Q mutation was more prone to cleavage by *** in vitro results suggested that the E674Q mutation was pathogenic by facilitating the BACE1-mediated processing of APP and the production of Aβ.Furthermore,we applied an adeno-associated virus(AAV)-mediated transfer of the human E674Q mutant APP gene to the hippocampi of two-month-old C57Bl/6 J ***-E674Q-injected mice exhibited impaired learning behavior and increased pathological burden in the brain,implying that the E674Q mutation had a pathogenicity that bore a comparison with the classical Swedish ***,we report a strong amyloidogenic effect of the E674Q substitution in *** our knowledge,E674Q is the only pathogenic mutation within the amyloid processing sequence causing LOAD.

关键词： Alzheimer's disease Amyloid beta APP mutation E674Q Late onset

记得云间第一歌——“杨荫浏中国音乐学研究基金”纪实

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中国音乐学 2024年第2期 5-11页

作者：张振涛南京艺术学院音乐学院

20世纪90年代中国艺术研究院音乐研究所设立了一项旨在为传统音乐研究提供出版资助的基金,“杨荫浏中国音乐学研究基金”资助了《杨荫浏全集》《20世纪中国音乐期刊目录汇编》《琴学60年论文集》等一系列学术著作和工具书的出版。虽然... 详细信息

20世纪90年代中国艺术研究院音乐研究所设立了一项旨在为传统音乐研究提供出版资助的基金,“杨荫浏中国音乐学研究基金”资助了《杨荫浏全集》《20世纪中国音乐期刊目录汇编》《琴学60年论文集》等一系列学术著作和工具书的出版。虽然因不符合相关政策而撤销,但其中反映的国家研究机构的转型,使得这宗掺杂了新旧体制分离的阵痛故事,有了社会学意义。保存下来的“捐款薄”,提供了一份音乐社会学样本,成为颇具历史含义的档案,证明了音乐学一度面临的困境与试图改变困境的种种尝试。

关键词：基金捐款薄机构转型学术出版

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手赋——李斯特纪念馆脞录

人民音乐 2024年第4期 81-86页

作者：张振涛南京艺术学院

一、世界上跑得最快的手。匈牙利布达佩斯“李斯特纪念馆”的大堂中,摆放着两架相向行列的大三角钢琴,这种摆放位置不禁让人想起电影《肖邦与李斯特》中两人初见的情景。李斯特笑容灿烂,满身阳光,见到就让人乐翻。他还没顾得上看肖邦一... 详细信息

一、世界上跑得最快的手。匈牙利布达佩斯“李斯特纪念馆”的大堂中,摆放着两架相向行列的大三角钢琴,这种摆放位置不禁让人想起电影《肖邦与李斯特》中两人初见的情景。李斯特笑容灿烂,满身阳光,见到就让人乐翻。他还没顾得上看肖邦一眼,就注意到谱架上摆放的新作,立马坐下来就弹。两人背对背,琴对琴,各自坐在一架钢琴前。

关键词：李斯特三角钢琴摆放位置纪念馆肖邦背对背

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他们怎么有那么多“表情”

人民音乐 2024年第7期 62-64页

作者：张振涛南京艺术学院中国艺术研究院

一、音乐家的行话音乐圈流行着一套术语,多到再稍稍借助一点肢体语言就能让全世界的音乐家沟通的程度。欧洲指挥不懂中文,能在中国乐团排练;中国演奏家不懂英文,能与欧洲乐团合作。甚至中国音乐家找不着恰当的词形容“散板”时,直接说Ru... 详细信息

一、音乐家的行话音乐圈流行着一套术语,多到再稍稍借助一点肢体语言就能让全世界的音乐家沟通的程度。欧洲指挥不懂中文,能在中国乐团排练;中国演奏家不懂英文,能与欧洲乐团合作。甚至中国音乐家找不着恰当的词形容“散板”时,直接说Rubato(自由节奏),两边也能心领神会。有了行话,音乐家像得了便宜似的,省了许多翻译的麻烦。混杂着拉丁文、意大利文、法文、德文、英文以及从日语倒腾过来的术语,使音乐圈成为世界上能够超越国界顺利交流的少数学科之一。

关键词：中国音乐家自由节奏 Rubato 意大利文肢体语言散板行话术语

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GTF2H4 regulates partial EndMT via NF-κB activation through NCOA3 phosphorylation in ischemic diseases

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The Innovation 2024年第2期5卷 27-41页

作者： Zheyan Fang Gang Zhao Shuang Zhao Xueting Yu Runyang Feng You-en zhang Haomin Li Lei Huang Zhenyang Guo zhentao zhang Mukaddas Abdurahman Hangnan Hong Peng Li Bing Wu Jinhang Zhu Xin Zhong Dong Huang Hao Lu Xin Zhao Zhaoyang Chen Wenbin zhang Junjie Guo Hongchao Zheng Yue He Shengying Qin Haojie Lu Yun Zhao Xiangdong Wang Junbo Ge Hua Li Department of Cardiology Zhongshan HospitalFudan UniversityShanghai Institute of Cardiovascular DiseasesShanghai 200032China Department of Cardiology Zhongshan HospitalFudan UniversityShanghai 200032China Department of Medical Examination Shanghai Xuhui District Central HospitalShanghai 200031China Department of Cardiology and Institute of Clinical Medicine Renmin HospitalHubei University of MedicineShiyan 442000China Clinical Data Center Children’s HospitalZhejiang University School of MedicineNational Clinical Research Center for Child HealthHangzhou 310052China Department of Molecular Celland Cancer BiologyUniversity of Massachusetts Chan Medical SchoolWorcesterMA 01605USA Bio-X Institute Key Laboratory for The Genetics of Developmental and Neuropsychiatric DisordersShanghai Jiao Tong UniversityShanghai 200030China Department of Cardiology Heart Center of Fujian ProvinceFujian Medical University Union HospitalFuzhou 350001China Department of Cardiology Sir Run Run Shaw Hospitalaffiliated with Zhejiang University School of MedicineHangzhou 310020China Department of Cardiology The Affiliated Hospital of Qingdao UniversityQingdao 266003China Department of Cardiology Shanghai Xuhui District Central HospitalShanghai 200031China Department of Cardiology Shanghai Eighth People’s HospitalShanghai 200235China Department of Chemistry and Institutes of Biomedical Sciences Fudan UniversityShanghai 200032China State Key Laboratory of Cell Biology Shanghai Institute of Biochemistry and Cell BiologyCenter for Excellence in Molecular Cell ScienceChinese Academy of SciencesUniversity of Chinese Academy of SciencesShanghai 200031China School of Life Science and Technology Shanghai Tech University100 Haike RoadShanghai 201210China Key Laboratory of Systems Health Science of Zhejiang Province School of Life ScienceHangzhou Institute for Advanced StudyUniversity of Chinese Academy of SciencesHangzhou 310024China Department of Pulmonary and Critical Care Medicine Zhongshan Hospital

Partial endothelial-to-mesenchymal transition(EndMT)is an intermediate phenotype observed in endothelial cells(ECs)undergoing a transition toward a mesenchymal state to support neovascularization during(patho)physiological ***,we investigated the occurrence of partial EndMT in ECs under hypoxic/ischemic conditions and identified general transcription factor IIH subunit 4(GTF2H4)as a positive regulator of this *** addition,we discovered that GTF2H4 collaborates with its target protein excision repair cross-complementation group 3(ERCC3)to co-regulate partial ***,by using phosphorylation proteomics and site-directed mutagenesis,we demonstrated that GTF2H4 was involved in the phosphorylation of receptor coactivator 3(NCOA3)at serine 1330,which promoted the interaction between NCOA3 and p65,resulting in the transcriptional activation of NF-κB and the NF-kB/Snail signaling axis during partial *** vivo experiments confirmed that GTF2H4 significantly promoted partial EndMT and angiogenesis after ischemic ***,our findings reveal that targeting GTF2H4 is promising for tissue repair and offers potential opportunities for treating hypoxic/ischemic diseases.

关键词： ischemic activation GTF

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围岩水中玻璃体关键元素动态浸出特性研究

原子能科学技术 2024年第2期58卷 289-295页

作者：孙琦张振涛王辰宇晁昊杜晓慧张银刘畅中国原子能科学研究院退役治理工程技术中心北京102413

为了获得地下水侵入深部围岩裂隙所致高放玻璃固化体核素释出与迁移参数的变化规律,本研究以高放玻璃固化体中核素在石英石水岩饱和水中的迁移行为为研究对象,开展了动态浸出试验,重点分析研究了元素B、Re和Si在浸出液中的浓度变化以及... 详细信息

为了获得地下水侵入深部围岩裂隙所致高放玻璃固化体核素释出与迁移参数的变化规律,本研究以高放玻璃固化体中核素在石英石水岩饱和水中的迁移行为为研究对象,开展了动态浸出试验,重点分析研究了元素B、Re和Si在浸出液中的浓度变化以及玻璃的腐蚀情况。结果显示,动态浸出试验150 d后,离子浓度均随反应时间的增加而增大。反应温度为90℃时,地质处置库中流速为40 L/(m^(2)·a)的石英石水岩饱和水中,玻璃柱的腐蚀速率为540 nm/a,3000年后,长1350 mm、直径440 mm、质量400 kg、表面积17 m^(2)的玻璃柱腐蚀深度为1.62 mm,B、Re和Si分别释出元素总含量的12.24%、10.41%和0.18%。

关键词：裂隙充填物高放玻璃固化体北山预选区元素释出

永磁同步风力发电系统的最大功率跟踪模糊分数阶控制

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现代电力 2024年第2期41卷 230-239页

作者：姜礼洁王晓燕苏杰张镇韬华北电力大学控制与计算机工程学院河北省保定市071003 河北省发电过程仿真与优化控制技术创新中心(华北电力大学) 河北省保定市071003

在“双碳”背景下,风电作为零碳电力和新能源发电的主力军,在助力社会全面绿色低碳转型方面发挥了关键性作用。在保证发电稳定的前提下实现风能的最大化利用,提升风力发电系统发电量至为重要。文中针对永磁同一步风力发电系统的最大功... 详细信息

在“双碳”背景下,风电作为零碳电力和新能源发电的主力军,在助力社会全面绿色低碳转型方面发挥了关键性作用。在保证发电稳定的前提下实现风能的最大化利用,提升风力发电系统发电量至为重要。文中针对永磁同一步风力发电系统的最大功率跟踪(maximum power point tracking, MPPT)问题进行研究。首先建立了永磁同步风力发电系统的机理仿真模型,用两电平双PWM全功率换流器连接风力发电机与电网。然后基于以上模型,分别设计了整数阶PI控制器、分数阶PI"控制器、模糊分数阶PP控制器以实现MPPT控制。最后对以上控制策略进行了仿真研究。结果表明,无论在阶跃风速还是随机风速下,模糊分数阶PU控制器相较于其他两种均具有更出色的MPPT性能与更强的鲁棒性。

关键词：永磁同步风力发电系统两电平双PWM全功率换流器模糊分数阶控制器最大功率跟踪发电稳定

C/EBPβ/AEP Signaling Drives Alzheimer's Disease Pathogenesis

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Neuroscience Bulletin 2023年第7期39卷 1173-1185页

作者： Jing Xiong zhentao zhang Keqiang Ye Department of Neurology Renmin Hospital of Wuhan UniversityWuhan 430060China Faculty of Life and Health Sciences Shenzhen Institute of Advanced Technology(SIAT)Shenzhen 518034China

Alzheimer’s disease(AD)is the most common type of *** two-thirds of patients with AD are *** reason for the higher susceptibility to AD onset in women is ***,hormone changes during the menopausal transition are known to be associated with *** recently,we reported that follicle-stimulating hormone(FSH)promotes AD pathology and enhances cognitive dysfunctions via activating the CCAAT-enhancer-binding protein(C/EBPβ)/asparagine endopeptidase(AEP)*** review summarizes our current understanding of the crucial role of the C/EBPβ/AEP pathway in driving AD pathogenesis by cleaving multiple critical AD players,including APP and Tau,explaining the roles and the mechanisms of FSH in increasing the susceptibility to AD in postmenopausal *** FSH-C/EBPβ/AEP pathway may serve as a novel therapeutic target for the treatment of AD.

关键词： Alzheimer's disease C/EBPβ Asparagine endopeptidase Pathogenesis Follicle stimulating hormone

Experimental Study on Hydrodynamic Behavior of Falling Film over Vertical Tube

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Journal of Thermal Science 2023年第4期32卷 1512-1522页

作者： YUE Yunkai zhang zhentao zhang Hailun YANG Junling LI Xiaoqiong zhang Yu Key Laboratory of Cryogenics Technical Institute of Physics and ChemistryChinese Academy of SciencesBeijing100190China University of Chinese Academy of Sciences Beijing100049China Key Laboratory of Technology on Space Energy Conversion Technical Institute of Physics and ChemistryChinese Academy of ScienceBeijing100190China

Falling film configurations play an important role in characterizing the heat transfer due to changes in hydrodynamic *** purpose of this study is to establish a novel film distributor to investigate the hydrodynamic behavior of the falling film on the vertical *** falling film thickness and flow patterns on the vertical tube were analyzed at a feed water temperature of 30℃ for film Re ranged from 53 to 4544 and the heat fluxes ranged from 1.33 to 49.45 kW/m^(2).The correlation between the average falling film thickness and the film Re was fitted;the maximum deviation between the experimental data and the predicted values was 7.58%.Additionally,the film thickness changed sharply when the heat flux increased to a certain *** the further increase of the heat flux,dry patches appeared on the surface of the experimental *** was Marangoni effect on vertical tube and the falling film thickness and flow patterns were significantly affected by *** interval value of the critical heat flux with film Re was *** with the porous film distributor reported in the literature,the critical heat flux of the new film distributor increased by 3.72%-56.95%.

关键词： falling film film distributor Marangoni effect film thickness