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A novel structural variant of RET causes familial Hirschsprung's disease via nonsense-mediated mRNA decay

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Genes & Diseases 2024年第1期11卷 7-10页

作者： qi wang Siyu pu Bo Xiang Jing Chen Department of Pediatric Surgery and Laboratory of Pediatric Surgery Rare Diseases CenterInstitute of Immunology and InflammationFrontiers Science Center for Disease-Related Molecular NetworkWest China HospitalSichuan UniversityChengduSichuan 610041China

Hirschsprung's disease(HsCR)is the most common type of developmental gastrointestinal malformation causing intestinal obstruction in children,with an incidence of 1/5000 in live births.1 it is characterized by the absence of intrinsic ganglion cells in the myenteric and submucosal plexuses of the distal gastrointestinal tract,leading to the dysregulation of smooth muscle contraction/relaxation and resulting in intestinal *** on the length of the aganglionic intestine,HSCR can be categorized into short-segmental HSCR(S-HSCR),which affects up to the sigmoid colon from the anus,and long-segmental HSCR(LHSCR),which affects beyond the sigmoid colon.1 In some rare conditions,aganglionic lesions can affect the full length of the colon or even the whole intestine.

关键词： intestine colon relaxation

Effects of two silicone resin coatings on performance of FeSiAl magnetic powder cores

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Journal of Iron and Steel Research International 2024年第5期31卷 1279-1288页

作者： Jia-qi Liu Zheng-qu Zhu pu wang Yi-fan Li Jing Pang Jia-quan Zhang School of Metallurgical and Ecological Engineering University of Science and Technology BeijingBeijing100083China qingdao Yunlu Advanced Materials Technology Co. Ltd.Qingdao266232ShandongChina

Two silicon resins with excellent thermal stability,JH1123 and JH7102,are used as the insulated agents and binders for the gas-atomized FeSiAl powder,and corresponding magnetic powder cores(MPCs)are *** insulation capability and application prospects of the two silicon resins are evaluated by comparing the magnetic properties of the coated powder and *** scanning electron microscopy,energy dispersive X-ray spectroscopy and Fourier transform infrared spectroscopy results show that uniform insulation layers are both formed on the powder ***1123 has stronger binding ability,and the JH1123-coated powder exhibits severe agglomeration,with d50(average particle size)approximately twice that of the JH7102-coated *** as-prepared MPCs exhibit outstanding soft magnetic ***,the permeability of FeSiAl@JH1123 is up to 74.0,which is 35.5%higher than that of FeSiAl@JH7102 because JH1123 can further improve the density of the *** for FeSiAl@JH7102,it has better direct current bias and lower core loss of 716.9 mW cm^(−3) at 20 mT and 1000 kHz due to its lower coercivity and greater anti-magnetic saturation ability.A comprehensive comparison shows that FeSiAl@JH1123 is suitable for medium and high frequency applications,while FeSiAl@JH7102 is more suitable for high frequency *** indicates that the use of JH1123 and JH7102 silicon resins for binding and insulated coating not only simplifies the preparation process of MPCs,but also enables the controlled production of MPCs for different applications.

关键词： FeSiAl Insulated coating Magnetic powder core Permeability Core loss

Real-time Energy Management Method for Electric-hydrogen Hybrid Energy Storage Microgrids Based on DP-MPC

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CSEE Journal of Power and Energy Systems 2024年第1期10卷 324-336页

作者： qi Li Xueli Zou Yuchen pu Weirong Chen School of Electrical Engineering Southwest Jiaotong UniversityChengdu 611756China

With the increasing presence of intermittent energy resources in microgrids,it is difficult to precisely predict the output of renewable resources and their load *** order to realize the economical operations of the system,an energy management method based on a model predictive control(MPC)and dynamic programming(DP)algorithm is *** method can reasonably distribute the energy of the battery,fuel cell,electrolyzer and external grid,and maximize the output of the distributed power supply while ensuring the power balance and cost optimization of the *** on an ultra-shortterm forecast,the output power of the photovoltaic array and the demand power of the system load are *** offline global optimization of traditional dynamic programming is replaced by the repeated rolling optimization in a limited period of time to obtain power values of each unit in the energy storage *** with the traditional DP,MILP-MPC and the logic based real-time management method,the proposed energy management method is proved to be feasible and effective.

关键词： Electric-hydrogen hybrid energy economic energy management improved dynamic programming microgrid model predictive control

Spontaneous gallbladder perforation and colon fistula in hypertriglyceridemia-related severe acute pancreatitis:A case report

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World Journal of Clinical Cases 2022年第17期10卷 5846-5853页

作者： qi-pu wang Yi-Jun Chen Mei-Xing Sun Jia-Yuan Dai Jian Cao qiang Xu Guan-Nan Zhang Sheng-Yu Zhang Department of Gastroenterology Peking Union Medical College HospitalBeijing 100730China School of Medicine Tsinghua UniversityBeijing 100084China Department of Emergency Medicine Peking Union Medical College HospitalBeijing 100730China Department of Radiology Peking Union Medical College HospitalBeijing 100730China Department of General Surgery Peking Union Medical College HospitalBeijing 100730China

BACKGROUND Gallbladder perforation and gastrointestinal fistula are rare but serious complications of severe acute pancreatitis(SAP).However,neither spontaneous gallbladder perforation nor cholecysto-colonic fistula has been reported in acalculous acute pancreatitis *** SUMMARY A 31-year-old male presenting with epigastric pain was diagnosed with hypertriglyceridemia-related *** suffered from multiorgan failure and was able to leave the intensive care unit on day *** percutaneous drainage tubes were placed for profound exudation in the peripancreatic region and left paracolic *** developed spontaneous gallbladder perforation with symptoms of fever and right upper quadrant pain 1 mo after SAP onset and was stabilized by percutaneous *** infection appeared 1 mo later and was treated with antibiotics but without satisfactory *** multiple colon fistulas,including a cholecysto-colonic fistula and a descending colon fistula,emerged 3 mo after the onset of ***-assisted peripancreatic debridement and ileostomy were carried out *** fistulas achieved spontaneous closure 7 mo later,and the patient recovered after cholecystectomy and ileostomy *** presume that the causes of gallbladder perforation are poor bile drainage due to external pressure,pancreatic enzyme erosion,and *** possible causes of colon fistulas are pancreatic enzymes or infected necrosis erosion,ischemia,and iatrogenic *** to our experience,localized gallbladder perforation can be stabilized by percutaneous *** debridement and proximal colostomy followed by cholecystectomy are feasible and valid treatment options for cholecysto-colonic *** Gallbladder perforation and cholecysto-colonic fistula should be considered in acalculous SAP patients.

关键词： Acalculous severe acute pancreatitis Gallbladder perforation Cholecysto-colonic fistula Percutaneous drainage Cholecystectomy Case report

Neuroprotective Effect and Mechanism of Tanreqing Injection on Ischemic Stroke:Insights from Network Pharmacology and in vivo Experiments

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Chinese Journal of Integrative Medicine 2024年第8期30卷 713-720页

作者： LI Zhong-hao pu Xiao-qi LI Sha-sha DONG Xiao-ke ZHANG Guo-qiang wang Yu LIU Jin-min Department of Emergency China-Japan Friendship HospitalBeijing100029China Department of Neurology Beijing University of Chinese Medicine Dongfang HospitalBeijing100078China Department of Radiology China-Japan Friendship HospitalBeijing100029China Graduate School Beijing University of Chinese MedicineBeijing100029China

Objective To explore the neuroprotective effects and mechanism of Tanreqing Injection(TRQ)on treating ischemic stroke based on network pharmacology and in vivo experimental *** The chemical compounds of TRQ were retrieved based on published data,with targets retrieved from pubChem,Therapeutic Target Database and *** visualization and analysis were performed using Cytoscape,with protein-protein interaction networks derived from the STRING *** analysis was performed using Kyoto Encyclopedia of Genes Genomes pathway and Gene Ontology *** in vivo experiments,the middle cerebral artery occlusion(MCAO)model was *** volume was determined by 2,3,5-triphenyltetrazolium hydrochloride staining and protein expressions were analyzed by Western *** docking was performed to predict ligand-receptor *** We screened 81 chemical compounds in TRQ and retrieved their therapeutic *** the targets,116 were therapeutic targets for *** enrichment analysis showed that the apelin signaling pathway was a key pathway for ischemic ***,in in vivo experiment we found that administering with intraperitoneal injection of 2.5 mL/kg TRQ every 6 h could significantly reduce the infarct volume of MCAO rats(P<0.05).In addition,protein levels of the apelin receptor(APJ)/phosphatidylinositol 3-kinase(PI3K)/protein kinase B(AKT)pathway were increased by TRQ(P<0.05).In addition,41 chemical compounds in TRQ could bind to *** The neuroprotective effect of TRQ may be related to the APJ/PI3K/AKT signaling ***,further studies are needed to confirm the findings.

关键词： Tanreqing Injection Chinese medicine ischemic stroke middle cerebral artery occlusion quantitative estimate of drug-likeness

Two-stage Scheduling for Island CPHH IES Considering Plateau Climate

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CSEE Journal of Power and Energy Systems 2024年第4期10卷 1775-1786页

作者： Yuchen pu qi Li Yibin qiu Xueli Zou Weirong Chen School of Electrical Engineering Southwest Jiaotong UniversityChengdu 611756China

In this paper,an island integrated energy system(IES)combining power,heat and hydrogen(CPHH)is built,and optimal power-heat-hydrogen scheduling is studied under actual plateau meteorological *** the comprehensive demands of residents,an IES including renewable energy sources(RESs),fuel cell,electrolyzer,electric boiler,thermal energy storage device and new energy vehicles is established and *** the first stage of operation,according to the prediction of RESs and demands,day-ahead optimization is carried out which aims to minimize operation cost,while electric vehicles(EVs)are considered as special demand response(DR)*** the second stage of operation,ultra-short-term prediction is implemented to provide prediction data for model predictive control(MPC),realizing real-time *** as a CPHH system,the fuel cell and electrolyzer collaborate to meet various needs of the plateau residential area with high *** addition,the method and IES structure proposed in this paper is compared with other options.

关键词： CPHH feul cell hydrogen island IES scheduling

Copper-catalyzed asymmetric dearomatizing amination of 2-naphthols:Csp^(2)–N coupling via 1,3-reductive elimination

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Science China Chemistry 2024年第8期67卷 2694-2700页

作者： Aying Yihuo Maoping pu Zheng Tan Jibang Liao Jiuqi Tan qi-Lin Zhou Xiaohua Liu Xiaoming Feng Key Laboratory of Green Chemistry&Technology Ministry of EducationCollege of ChemistrySichuan UniversityChengdu 610064China Key Laboratory and Institute of Elemento-Organic Chemistry College of Chemistry Frontiers Science Centerfor New Organic MatterNankai UniversityTianjin300071China

An efficient catalytic asymmetric dearomatizing amination of 2-naphthols and phenols catalyzed by N,N′-dioxide-copper(I)complex as a chiral catalyst was presented.A variety of optically activeβ-naphthalenone compounds with a nitrogen-containing quaternary carbon stereocenter were obtained with high yield and enantioselectivity under mild reaction *** studies indicated that this Csp^(2)–N dearomatizing coupling proceeds via 1,3-reductive elimination of phenolate-Cu~Ⅲ-amino intermediate in five-membered ring transition *** origin of enantioselectivity has also been elucidated based on density functional theory calculations.

关键词： asymmetric catalysis amination dearomatization copper catalysis coupling reaction

Enhanced potency of an IgM-like nanobody targeting conserved epitope in SARS-CoV-2 spike N-terminal domain

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Signal Transduction and Targeted Therapy 2024年第6期9卷 2726-2737页

作者： Bo Liu Honghui Liu pu Han Xiaoyun wang Chunmei wang Xinxin Yan Wenwen Lei Ke Xu Jianjie Zhou Jianxun qi Ruiwen Fan Guizhen Wu Wen-xia Tian George F.Gao qihui wang College of Veterinary Medicine Shanxi Agricultural University030801 JinzhongChina CAS Key Laboratory of Pathogen Microbiology and Immunology Institute of MicrobiologyChinese Academy of Sciences(CAS)100101 BeijingChina School of Life Sciences Yunnan University650504 KunmingYunnan ProvinceChina NHC Key Laboratory of Biosafety National Institute for Viral Disease Control and PreventionChinese Center for Disease Control and Prevention(China CDC)102206 BeijingChina Savaid Medical School University of Chinese Academy of Sciences101408 BeijingChina

Almost all the neutralizing antibodies targeting the receptor-binding domain(RBD)of spike(S)protein show weakened or lost efficacy against severe acute respiratory syndrome coronavirus 2(SARS-CoV-2)emerged or emerging variants,such as Omicron and its *** suggests that highly conserved epitopes are crucial for the development of neutralizing ***,we present one nanobody,N235,displaying broad neutralization against the SARS-CoV-2 prototype and multiple variants,including the newly emerged Omicron and its ***-electron microscopy demonstrates N235 binds a novel,conserved,cryptic epitope in the N-terminal domain(NTD)of the S protein,which interferes with the RBD in the neighboring S *** neutralization mechanism interpreted via flow cytometry and Western blot shows that N235 appears to induce the S1 subunit shedding from the trimeric S ***,a nano-IgM construct(MN235),engineered by fusing N235 with the human IgM Fc region,displays prevention via inducing S1 shedding and cross-linking virus *** to N235,MN235 exhibits varied enhancement in neutralization against pseudotyped and authentic viruses in *** intranasal administration of MN235 in low doses can effectively prevent the infection of Omicron sub-variant BA.1 and XBB in vivo,suggesting that it can be developed as a promising prophylactic antibody to cope with the ongoing and future infection.

关键词： doses prevention conserved

Crystal structures of RNA-dependent RNA polymerases from Jingmen tick virus and Alongshan virus

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hLife 2024年第1期2卷 18-31页

作者： Zhenyang Liu qi Peng pu Han Lu Kuai Jianxun qi Yi Shi CAS Key Laboratory of Pathogen Microbiology and Immunology Institute of MicrobiologyChinese Academy of SciencesBeijingChina Beijing Life Science Academy BeijingChina International Institute of Vaccine Research and Innovation Savaid Medical SchoolUniversity of Chinese Academy of SciencesBeijingChina Center for Influenza Research and Early-warning CAS-TWAS Center of Excellence for Emerging Infectious DiseaseChinese Academy of SciencesBeijingChina Research Unit of Adaptive Evolution and Control of Emerging Viruses Chinese Academy of Medical SciencesBeijingChina

Jingmenviruses are a group of flavi-like viruses with segmented genome and have been found in various types of hosts,including humans,cattle,monkeys,bats,rodents,sheep,ticks,mosquitoes and ***,including the Jingmen tick virus(JMTV)and Alongshan virus(ALSV),have been associated with febrile illness and flu-like symptoms in *** polymerase plays critical roles in genome replication and transcription and is an ideal target for antiviral ***,we determined the crystal structures of RNA-dependent RNA polymerase(RdRp)domains of JMTV and ALSV at 2.6Åand 3.2Åresolutions,*** overall structures of JMTV and ALSV RdRp domains are similar to those from the typical unsegmented viruses in Flaviviridae family,especially the Flavivirus *** and ALSV RdRps can be divided into three subdomains and the catalytical Motif A-G are conserved like the typical flaviviruses,whereas the zinc-binding pockets are absent from the JMTV and ALSV *** 50-ends of jingmenvirus genomes are varied in length and sequence,and a highly conserved 8-nucleotide element located on the tip of stem loop A was identified and shown to be required for binding with RdRp and performing de novo replication *** findings provide important structural insights into RdRp of segmented flavivirus and reveal the key region of virus genome responsible for replication initiation,which would promote molecular understanding of segmented flavivirus replication and the structure-based design of antiviral drugs against flaviviruses.

关键词： Jingmenviruses X-rays RNA-dependent RNA polymerase replication