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Both gain-and loss-of-function variants of KCNA1 are associated with paroxysmal kinesigenic dyskinesia

Journal of Genetics and Genomics 2024年第8期51卷 801-810页

作者： Wan-Bing sun jing-Xin fu Yu-Lan Chen Hong-fu Li Zhi-Ying Wu Dian-fu Chen Department of Medical Genetics and Center for Rare Diseases and Department of Neurologyand Zhejiang Key Laboratory of Rare Diseases for Precision Medicine and Clinical Translation in Second Affiliated HospitalZhejiang University School of MedicineHangzhouZhejiang 310009China Nanhu Brain-computer Interface Institute HangzhouZhejiang 314050China MOE Frontier Science Center for Brain Science and Brain-machine Integration School of Brain Science and Brain MedicineZhejiang UniversityHangzhouZhejiang 310012China

KCNA1 is the coding gene for Kv1.1 voltage-gated potassium-channelα*** variants of KCNA1 have been reported to manifest as paroxysmal kinesigenic dyskinesia(PKD),but the correlation between them remains unclear due to the phenotypic complexity of KCNA1 variants as well as the rarity of PKD *** the whole exome sequencing followed by Sanger sequencing,we screen for potential pathogenic KCNA1 variants in patients clinically diagnosed with paroxysmal movement disorders and identify three previously unreported missense variants of KCNA1 in three unrelated Chinese *** proband of one family(c.496G>A,p.A166T)manifests as episodic ataxia type 1,and the other two(c.877G>A,p.V293I and c.1112C>A,p.T371A)manifest as *** pathogenicity of these variants is confirmed by functional studies,suggesting that p.A166T and p.T371A cause a loss-of-function of the channel,while p.V293I leads to a gain-of-function with the property of voltage-dependent gating and activation kinetic *** reviewing the locations of PKD-manifested KCNA1 variants in Kv1.1 protein,we find that these variants tend to cluster around the pore domain,which is similar to ***,our study strengthens the correlation between KCNA1 variants and PKD and provides more information on genotype–phenotype correlations of KCNA1 channelopathy.

关键词： Paroxysmal kinesigenic dyskinesia KCNA1 Loss-of-function Gain-of-function Channelopathy Episodicataxiatype1

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Light-responsive Self-Immolative L-glutamic Acid-based Polyester Nanoparticles for Controlled Drug Release via Passerini Three-Component Polymerization

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Chinese Journal of Polymer Science 2024年第5期42卷 570-578,I0005页

作者： Xiao-Fei sun Xu Zhang Shu-Ping Song Ya-Qun Yao Yan Zhang Cheng-Liang Wang jing-Jiang sun Qing-fu Wang Key Laboratory of Rubber-plastics Ministry of EducationSchool of Polymer Science and EngineeringQingdao University of Science and TechnologyQingdao266042China Qingdao Research Academy of Environmental Science Qingdao266012China

L-glutamic acid(LA)is a bio-based,non-toxic,environmentally friendly material derived from *** present study reports the application of Passerini three-component polymerization(P-3CP)for the straightforward preparation of LA-based light-responsive polyesters(PLTDs)under mild *** with molar masses up to 8500 g/mol and high yields exceeding 90%are *** chemical structures and light-responsive self-immolative behavior of PLTDs are comprehensively characterized by employing ultraviolet-visible(UV-Vis)spectroscopy,size exclusion chromatography(SEC),nuclear magnetic resonance(NMR)spectroscopy,and liquid chromatography mass spectrometry(LC-MS).Meanwhile,monodisperse PLTD-based doxorubicin-loaded nanoparticles(PLTD-DOX-NP)(size=193 nm,PDI=0.018)are formulated by nanoprecipitation *** light-induced depolymerization,the PLTD-DOX-NP undergoes rapid decomposition,resulting in a burst release of 80%cargo within 13 ***,according to biological toxicity tests,the PLTD-NP possesses adequate biosafety,both before and after ***,the incorporation of P-3CP with biorenewable LA-based monomer adheres to the principles of green chemistry,significantly simplifying the synthetic pathway of light-responsive polymers.

关键词： L-glutamic acid Passerini three-component polymerization Light-responsive Polyester Nanoparticles

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Recursive filtering of multi-rate cyber-physical systems with unknown inputs under adaptive event-triggered mechanisms

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Frontiers of Information Technology & Electronic Engineering 2024年第2期25卷 250-259页

作者： Ying sun Miaomiao fu jingyang MAO Guoliang WEI Business School University of Shanghai for Science and TechnologyShanghai200093China Shanghai Caoyang Vocational School Shanghai200333China School of Electrical and Electronic Engineering Shanghai Institute of TechnologyShanghai201418China School of Intelligent Emergency Management University of Shanghai for Science and TechnologyShanghai200093China

Cyber-physical systems(CPSs)take on the characteristics of both multiple rates of information collection and processing and the dependency on information *** purpose of this paper is to develop a joint recursive filtering scheme that estimates both unknown inputs and system states for multi-rate CPSs with unknown *** cyberspace,the information transmission between the local joint filter and the sensors is governed by an adaptive event-triggered ***,the desired parameters of joint filters are determined by a set of algebraic matrix equations in a recursive way,and a sufficient condition verifying the boundedness of filtering error covariance is found by resorting to some algebraic operation.A state fusion estimation scheme that uses local state estimation is proposed based on the covariance intersection(CI)based fusion ***,an illustrative example demonstrates the effectiveness of the proposed adaptive event-triggered recursive filtering algorithm.

关键词： Cyber-physical systems Multi-rate Joint recursive filtering Adaptive event-triggered mechanisms Unknown inputs

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Reputation-based joint optimization of user satisfaction and resource utilization in a computing force network

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Frontiers of Information Technology & Electronic Engineering 2024年第5期25卷 685-700页

作者： Yuexia fu jing WANG Lu LU Qinqin TANG Sheng ZHANG China Mobile Research Institute Beijing 100053China Purple Mountain Laboratories Nanjing 211111China China Mobile Communications Corporation Beijing 100033China

Under the development of computing and network convergence,considering the computing and network resources of multiple providers as a whole in a computing force network(CFN)has gradually become a new ***,since each computing and network resource provider(CNRP)considers only its own interest and competes with other CNRPs,introducing multiple CNRPs will result in a lack of trust and difficulty in unified *** addition,concurrent users have different requirements,so there is an urgent need to study how to optimally match users and CNRPs on a many-to-many basis,to improve user satisfaction and ensure the utilization of limited *** this paper,we adopt a reputation model based on the beta distribution function to measure the credibility of CNRPs and propose a performance-based reputation update ***,we formalize the problem into a constrained multi-objective optimization problem and find feasible solutions using a modified fast and elitist non-dominated sorting genetic algorithm(NSGA-II).We conduct extensive simulations to evaluate the proposed *** results demonstrate that the proposed model and the problem formulation are valid,and the NSGA-II is effective and can find the Pareto set of CFN,which increases user satisfaction and resource ***,a set of solutions provided by the Pareto set give us more choices of the many-to-many matching of users and CNRPs according to the actual situation.

关键词： Computing force network Resource scheduling Performance-based reputation User satisfaction

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Significantly Enhanced Melt Memory Effect of Metallocene-made Isotactic Polypropylene Containing Talc

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Chinese Journal of Polymer Science 2024年第2期42卷 213-222,I0008页

作者： Hong-Wen sun fu-Shan Wang Yan Gao fu-Qing Wei Jia-Chun Feng State Key Laboratory of Molecular Engineering of Polymers Department of Macromolecular ScienceFudan UniversityShanghai200433China Lanzhou Petrochemical Corporation of PetroChina Lanzhou730060China Lanzhou Petrochemical Research Center Petrochemical Research Institute of PetroChinaLanzhou730060China

The melt memory effect is a widely observed phenomenon in semi-crystalline polymers. In practical applications, various additives are usually introduced into polymers, which may affect their melt memory behavior. In this work, the effect of talc on the melt memory effect of metallocene-made isotactic polypropylene(M-PP) was investigated in detail by using the differential scanning calorimetry. The results indicated that the introduction of talc significantly strengthened the melt memory effect of M-PP. Specifically, the upper limit temperature of Domain II increased from 161 ℃ to 174 ℃, resulting in a substantial widening of the temperature range of Domain IIa from 1 ℃ to 14 ℃. Analysis of the crystal orientation of the M-PP containing talc cooled from various Ts suggested that the remarkably enhanced melt memory effect could be ascribed to the stabilization of oriented nuclei facilitated by talc. This stabilizing effect was likely attributable to the prefreezing effect or the sorption interaction between talc and the M-PP chains.

关键词： Metallocene-made isotactic polypropylene Talc Melt memory effect Crystal orientation

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Global trend and future landscape of intestinal microcirculation research from 2000 to 2021:A scientometric study

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World Journal of Gastroenterology 2023年第9期29卷 1523-1535页

作者： sun-jing fu Meng-Ting Xu Bing Wang Bing-Wei Li Hao Ling Yuan Li Qin Wang Xue-Ting Liu Xiao-Yan Zhang Ai-Ling Li Ming-Ming Liu Institute of Microcirculation Key Laboratory of MicrocirculationMinistry of HealthChinese Academy of Medical Sciences&Peking Union Medical CollegeBeijing 100005China Department of Radiology The Affiliated Changsha Central HospitalHengyang Medical SchoolUniversity of South ChinaChangsha 410004Hunan ProvinceChina Diabetes Research Center Chinese Academy of Medical Sciences&Peking Union Medical CollegeBeijing 100005China

BACKGROUND The intestinal microcirculation functions in food absorption and metabolic substance *** evidence indicates that intestinal microcirculatory dysfunction is a significant source of multiple gastrointestinal *** date,there has not been a scientometric analysis of intestinal microcirculatory *** To investigate the current status,development trends,and frontiers of intestinal microcirculatory research based on bibliometric *** VOSviewer and CiteSpace 6.1.R2 were used to identify the overall characteristics and knowledge map of intestinal microcirculatory research based on the core literature published from 2000 to 2021 in the Web of Science *** characteristics of each article,country of origin,institution,journal,cocitations,and other information were analyzed and *** There were 1364 publications enrolled in the bibliometric analysis,exhibiting an upward trend from 2000 to 2021 with increased participation *** United States and Dalhousie University took the lead among countries and institutions,*** was the most prolific journal,and Nature Reviews Microbiology Clinical had the most *** topical hotspots and frontiers in intestinal microcirculatory research were centered on the pathological processes of functional impairment of intestinal microvessels,diverse intestinal illnesses,and clinical *** Our study highlights insights into trends of the published research on the intestinal microcirculation and offers serviceable guidance to researchers by summarizing the prolific areas in intestinal disease research to date.

关键词： Intestinal microcirculation Bibliometric analysis CiteSpace VOSviewer Visualization

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Design of low-alloying and high-performance solid solution-strengthened copper alloys with element substitution for sustainable development

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International Journal of Minerals,Metallurgy and Materials 2024年第5期31卷 826-832页

作者： Jiaqiang Li Hongtao Zhang jingtai sun Huadong fu Jianxin Xie Beijing Advanced Innovation Center for Materials Genome Engineering University of Science and Technology BeijingBeijing 100083China Key Laboratory for Advanced Materials Processing(MOE) University of Science and Technology BeijingBeijing 100083China Beijing Laboratory of Metallic Materials and Processing for Modern Transportation University of Science and Technology BeijingBeijing 100083China Liaoning Academy of Materials Shenyang 110004China

Solid solution-strengthened copper alloys have the advantages of a simple composition and manufacturing process,high mechanical and electrical comprehensive performances,and low cost;thus,they are widely used in high-speed rail contact wires,electronic component connectors,and other *** the contradiction between low alloying and high performance is an important challenge in the development of solid solution-strengthened copper *** the typical solid solution-strengthened alloy Cu-4Zn-1Sn as the research object,we proposed using the element In to replace Zn and Sn to achieve low alloying in this *** new alloys,Cu-1.5Zn-1Sn-0.4In and Cu-1.5Zn-0.9Sn-0.6In,were designed and *** total weight percentage content of alloying elements decreased by 43%and 41%,respectively,while the product of ultimate tensile strength(UTS)and electrical conductivity(EC)of the annealed state increased by 14%and 15%.After cold rolling with a 90%reduction,the UTS of the two new alloys reached 576 and 627MPa,respectively,the EC was 44.9%IACS and 42.0%IACS,and the product of UTS and EC(UTS×EC)was 97%and 99%higher than that of the annealed state *** dislocations proliferated greatly in cold-rolled alloys,and the strengthening effects of dislocations reached 332 and 356 MPa,respectively,which is the main reason for the considerable improvement in mechanical properties.

关键词： element substitution copper alloy solid solution strengthening microstructure and performance

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Investigation of Maxima Assumptions in Modelling Tropical Cyclone- Induced Hazards in the South China Sea

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China Ocean Engineering 2024年第3期38卷 491-504页

作者： WEN Ze-guo WANG fu-ming WAN jing YANG Fan School of Civil Engineering Sun Yat-sen University&Southern Marine Science and Engineering Guangdong Laboratory(Zhuhai)Zhuhai 519082China Guangdong Academy of Safety Science and Technology Guangzhou 510060China Guangdong Key Laboratory of Oceanic Civil Engineering Guangzhou 510275China Advanced Energy Science and Technology Guangdong Laboratory(Yangjiang Offshore Wind Energy Laboratory) Yangjiang 529500China

The present study aims to examine the suitability of two commonly used assumptions that simplify modelling metoceanconditions for designing offshore wind turbines in the South China Sea (SCS). The first assumption assumes thatjoint N-year extreme wind and wave events can be independently estimated and subsequently combined. The secondone assumes peak wind and waves can be modelled as occurring simultaneously during a tropical cyclone (TC) *** better understand the potential TC activity, a set of 10000 years synthetic TC events are generated. The wind fieldmodel and the Mike 21 spectral wave model are employed to model the TC-induced hazards. Subsequently, theeffect of the assumptions is evaluated by analyzing the peak structural response of both monopile and semisubmersibleoffshore wind turbines during TC events. The results demonstrate that the examined assumptions are generally *** assessing the implications of these assumptions, valuable insights are obtained, which can inform andimprove the modelling of TC-induced hazards in the SCS region.

关键词： tropical cyclone numerical wave modelling hazards offshore wind turbines structural response

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Role of insulin in pancreatic microcirculatory oxygen profile and bioenergetics

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World Journal of Diabetes 2022年第9期13卷 765-775页

作者： Bing-Wei Li Yuan Li Xu Zhang sun-jing fu Bing Wang Xiao-Yan Zhang Xue-Ting Liu Qin Wang Ai-Ling Li Ming-Ming Liu Institute of Microcirculation Diabetes Research CenterChinese Academy of Medical Sciences&Peking Union Medical CollegeBeijing 100005China Laboratory of Electron Microscopy Ultrastructural Pathology CenterPeking University First HospitalBeijing 100005China

BACKGROUND The pancreatic islet microcirculation adapts its metabolism to cope with limited oxygen availability and nutrient *** diabetes,the balance between oxygen delivery and consumption is *** has been proven to exert complex actions promoting the maintenance of homeostasis of the pancreas under *** To test the hypothesis that insulin administration can improve the integrated pancreatic microcirculatory oxygen profile and *** The pancreatic microcirculatory partial oxygen pressure(PO_(2)),relative hemoglobin(rHb)and hemoglobin oxygen saturation(SO_(2))were evaluated in nondiabetic,type 1 diabetes mellitus(T1DM),and insulin-treated mice.A threedimensional framework was generated to visualize the microcirculatory oxygen *** changes in the microvasculature were examined using transmission electron *** Extracellular Flux Analyzer was used to detect the real-time changes in bioenergetics by measuring the oxygen consumption rate and extracellular acidification rate in islet microvascular endothelial cells(IMECs).RESULTS Significantly lower PO_(2),rHb,and SO_(2) values were observed in T1DM mice than in nondiabetic *** administration ameliorated the streptozotocin-induced decreases in these microcirculatory oxygen parameters and improved the mitochondrial ultrastructural abnormalities in *** profiling revealed that the IMECs did not have spare respiratory ***-treated IMECs exhibited significantly greater basal respiration than glucotoxicity-exposed IMECs(P<0.05).An energy map revealed increased energetic metabolism in insulin-treated IMECs,with significantly increased ATP production,non-mitochondrial respiration,and oxidative metabolism(all P<0.05).Significant negative correlations were revealed between microcirculatory SO_(2) and bioenergetic *** Glucotoxicity deteriorates the integrated pancreatic microcirculatory oxygen profil

关键词： Diabetes mellitus Glucotoxicity Endothelial cells Microcirculation Mitochondria Bioenergetics

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Initial precursor reaction mechanism of CVD-HfC coating based on density functional theory

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Chinese Journal of Aeronautics 2024年第7期37卷 511-521页

作者： Yunzhou LIN Yulei ZHANG Yanqin fu Jian ZHANG jing’an KONG Ruicong CHEN State Key Laboratory of Solidification Processing Carbon/Carbon Composites Research CenterNorthwestern Polytechnical UniversityXi’an 710072China Henan Key Laboratory of High Performance Carbon Fiber Reinforced Composites Carbon Matrix Composites Research InstituteHenan Academy of SciencesZhengzhou 450046China

Recently,the preparation of ultra-high temperature HfC ceramic coating has gained significant attention,particularly through the application of the HfCl_(4)-CH_(4)-H_(2)-Ar system via Chemical Vapor Deposition(CVD),which has been found widely applied to C/C ***,an analysis of the reactions that occur in the initial stage of the CVD-HfC coating process is presented using Density functional Theory(DFT)and Transition State Theory(TST)at the B3LYP/Lanl2DZ *** results reveal that HfCl4 can only cleave to produce hypochlorite,which will further react with methyl to synthesize intermediates to form *** to the analysis of the energy barrier and reaction constant,HfCl preferentially reacts with methyl groups to form complex adsorptive intermediates at 1573 *** a C—Hf bond production energy of 212.8 kcal/mol(1 kcal=4.18 kJ),the reaction rate constant of HfCl+CH is calculated to be 2.15×10^(-18) cm^(3)/s at 1573 ***,both the simulation and experimental results exhibit that the upward trend of reaction rate constants with temperature is also consistent with the deposition rate,indicating that the growth curve of the reaction rate constants tends to flatten *** proposed reaction model of the precursor’s decomposition and reconstruction during deposition process has significant implication for the process guidance.

关键词： Carbon composite HfC coating Chemical vapor deposition Density functional theory Precursor reaction mechanism

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