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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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Thermal safety boundary of lithium-ion battery at different state of charge

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Journal of Energy Chemistry 2024年第4期91卷 59-72页

作者： Hang Wu Siqi chen Yan hong chengshan Xu Yuejiu Zheng Changyong Jin Kaixin chen Yafei He Xuning Feng Xuezhe Wei Haifeng Dai Clean Energy Automotive Engineering Center Tongji UniversityShanghai 201804China College of Mechanical Engineering University of Shanghai for Science and TechnologyShanghai 200093China State Key Laboratory of Automotive Safety and Energy Tsinghua UniversityBeijing 100084China

Thermal runaway(TR)is a critical issue hindering the large-scale application of lithium-ion batteries(LIBs).Understanding the thermal safety behavior of LIBs at the cell and module level under different state of charges(SOCs)has significant implications for reinforcing the thermal safety design of the lithium-ion battery *** study first investigates the thermal safety boundary(TSB)correspondence at the cells and modules level under the guidance of a newly proposed concept,safe electric quantity boundary(SEQB).A reasonable thermal runaway propagation(TRP)judgment indicator,peak heat transfer power(PHTP),is proposed to predict whether TRP ***,a validated 3D model is used to quantitatively clarify the TSB at different SOCs from the perspective of PHTP,TR trigger temperature,SOC,and the full cycle ***,three different TRP transfer modes are *** interconversion relationship of three different TRP modes is investigated from the perspective of *** paper explores the TSB of LIBs under different SOCs at both cell and module levels for the first time,which has great significance in guiding the thermal safety design of battery systems.

关键词： Lithium-ion battery Battery safety Thermal runaway State of charge Numerical analysis

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Study on the clogging mechanism of punching screen in sand control by the punching structure parameters

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Petroleum Science 2024年第1期21卷 609-620页

作者： Fu-cheng Deng Fu-Lin Gui Bai-Tao Fan Lei Wen Sheng-hong chen Ning Gong Yun-chen Xiao Zhi-Hui Xu Yangtze University Jingzhou434023HubeiChina CNOOC Research Institute Ltd. Beijing100028China CNOOC Ener Tech-Drilling&Production Co. Tianjin300457China Tianjin Branch of CNOOC Ltd. Tianjin300459China

As an independent sand control unit or a common protective shell of a high-quality screen,the punching screen is the outermost sand retaining unit of the sand control pipe which is used in geothermal well or oil and gas ***,most screens only consider the influence of the internal sand retaining medium parameters in the sand control performance design while ignoring the influence of the plugging of the punching screen on the overall sand retaining performance of the *** explore the clogging mechanism of the punching screen,this paper established the clogging mechanism calculation model of a single punching screen sand control unit by using the computational fluid mechanics-discrete element method(CFD-DEM)combined *** to the combined motion of particles and fluids,the influence of the internal flow state on particle motion and accumulation was *** results showed that(1)the clogging process of the punching sand control unit is divided into three stages:initial clogging,aggravation of clogging and stability of *** the initial stage of blockage,coarse particles form a loose bridge structure,and blockage often occurs preferentially at the streamline gathering place below chamfering inside the sand control *** the stage of blockage intensification,the particle mass develops into a relatively complete sand bridge,which develops from both ends of the opening to the center of the *** the stable plugging stage,the sand deposits show a“fan shape”and form a“V-shaped”gully inside the punching slot element.(2)Under a certain reservoir particle-size distribution,The slit length and opening height have a large influence on the permeability and blockage rate,while the slit width size has little influence on the permeability and blockage *** microscopic clogging mechanism and its law of the punching screen prevention unit are proposed in this study,which has some field guidance significance for the design of punching screen

关键词： Punching screen Plugging CFD-DEM Size parameter Sand control

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Experimental study of the effects of a multistage pore-throat structure on the seepage characteristics of sandstones in the Beibuwan Basin:Insights into the flooding mode

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Petroleum Science 2024年第2期21卷 1044-1061页

作者： Lei Wang Xiao Lei Qiao-Liang Zhang Guang-Qing Yao Bo sui Xiao-Jun chen Ming-Wei Wang Zhen-Yu Zhou Pan-Rong Wang Xiao-Dong Peng Zhanjiang Branch China National Offshore Oil Corporation(CNOOC)LimitedZhanjiang524057GuangdongChina Faculty of Earth Resources China University of Geosciences(Wuhan)Wuhan430074HubeiChina School of Resources and Safety Engineering Chongqing UniversityChongqing400044China School of Engineering University of ManchesterManchester M139WJUK Hainan Branch China National Offshore Oil Corporation(CNOOC)LimitedHaikou570311HainanChina

To investigate the relationship between grain sizes, seepage capacity, and oil-displacement efficiency in the Liushagang Formation of the Beibuwan Basin, this study identifies the multistage pore-throat structure as a crucial factor through a comparison of oil displacement in microscopic pore-throat experiments. The two-phase flow evaluation method based on the Li-Horne model is utilized to effectively characterize and quantify the seepage characteristics of different reservoirs, closely relating them to the distribution of microscopic pores and throats. It is observed that conglomerate sandstones at different stages exhibit significant heterogeneity and noticeable differences in seepage capacity, highlighting the crucial role played by certain large pore throats in determining seepage capacity and oil displacement efficiency. Furthermore, it was found that the displacement effects of conglomeratic sandstones with strong heterogeneity were inferior to those of conventional homogeneous sandstone, as evidenced by multiple displacement experiments conducted on core samples with varying granularities and flooding systems. Subsequently, core-based experiments on associated gas flooding after water flooding were conducted to address the challenge of achieving satisfactory results in a single displacement mode for reservoirs with significant heterogeneity. The results indicate that the oil recovery rates for associated gas flooding after water flooding increased by 7.3%-16.4% compared with water flooding alone at a gas-oil ratio of approximately 7000 m^(3)/m^(3). Therefore, considering the advantages of gas flooding in terms of seepage capacity, oil exchange ratio, and the potential for two-phase production, gas flooding is recommended as an energy supplement mode for homogeneous reservoirs in the presence of sufficient gas source and appropriate tectonic angle. On the other hand, associated gas flooding after water flooding is suggested to achieve a more favorable develop

关键词： Beibuwan Basin Multistage pore-throat structure Multistage seepage characteristics Microscopic visualization Heterogeneity Gas/water flooding Flooding mode

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Ischemic colitis induced by a platelet-raising capsule: A case report

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World Journal of Clinical Cases 2024年第3期12卷 607-615页

作者： chen-Lu Wang Ze-Kun Si Guo-hong Liu chen chen Hui Zhao Lei Li The Affiliated Hospital of Shandong Second Medical University School of Clinical MedicineShandong Second Medical UniversityWeifang 261000Shandong ProvinceChina Department of Gastroenterology The Affiliated Hospital of Shandong Second Medical UniversityWeifang 261000Shandong ProvinceChina

BACKGROUND Ischemic colitis(IC)is also known as colon ischemia and is caused by colon vascular occlusion or nonocclusion,which results in a reduced blood supply to the colon and is not significant enough to maintain the metabolic function of cells,leading to intestinal wall *** main symptoms include abdominal pain,diarrhea,and bloody *** severe cases,intestinal gangrene,peritonitis,intestinal stenosis and even intestinal obstruction may *** induced by long-term use of certain special drugs is relatively rare in clinical *** article describes the clinical diagnosis and treatment of a typical case and provides a new treatment idea for the treatment of *** SUMMARY The patient was admitted to the hospital with"abdominal pain for half a month and bloody stool with mucous and pus for 3 d"and was diagnosed with"IC".Symptomatic and supportive treatment,such as antibiotics(levofloxacin),acid inhibition and stomach protection,fluid replenishment,and intravenous nutrition,was *** patient's colonic ulcers were considered to be related to the oral administration of platelet(PLT)-raising capsules;the patient was asked to stop PLT-raising drugs for selective review via colonoscopy,and antibiotics and mesalazine enteric-coated tablets were *** the guidance of hematology consultation,60 mg of methylprednisolone was given in combination with PLT infusion to increase the *** treatment,the patient's condition stabilized,the patient’s stool turned yellow,the patient’s symptoms improved,and the patient was allowed to leave the *** PLT-raising capsules can lead to IC,so clinicians should have a full understanding of the application of these drugs in the treatment of various causes of thrombocyt-INTRODUCTION Ischemic colitis(IC)is also known as colon ischemia(CI)and is caused by colon vascular occlusion or nonocclusion,which results in a reduced blood supply to the colon;this process is not significant enough to maintai

关键词： Platelet-raising capsule Ischemic colitis Drug-related Colonic ulcer Case report

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Anesthesia,Anesthetics,and Postoperative Cognitive Dysfunction in Elderly Patients

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Current Medical Science 2024年第2期44卷 291-297页

作者： hong-yu ZHU Jian-li YAN Min ZHANG Tian-yun XU chen chen Zhi-lin WU Department of Anesthesiology Linhe District People's HospitalBayannur 015000China Department of Neurosurgery Xinzhou District People's HospitalWuhan 430408China Department of Anesthesiology Union HospitalTongji Medical CollegeHuazhong University of Science and TechnologyWuhan 430022China

Postoperative cognitive dysfunction(POCD)remains a major issue that worsens the prognosis of elderly surgery *** article reviews the current research on the effect of different anesthesia methods and commonly utilized anesthetics on the incidence of POCD in elderly patients,aiming to provide an understanding of the underlying mechanisms contributing to this condition and facilitate the development of more reasonable anesthesia protocols,ultimately reducing the incidence of POCD in elderly surgery patients.

关键词： anesthesia anesthetics postoperative cognitive dysfunction elderly patients

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Segregation of Mg-6Gd alloy under natural convection:From macro solute distribution to micro dendrite growth

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China Foundry 2024年第5期21卷 613-624页

作者： hong-xu chen Ang Zhang Hao Li Yu Gao Yu-hong Cui Guang-sheng Huang Bin Jiang Fu-sheng Pan National Engineering Research Center for Magnesium Alloys National Key Laboratory of Advanced Casting TechnologiesCollege of Materials Science and EngineeringChongqing UniversityChongqing 400044China

Segregation is a serious defect in alloy ingots which severely deteriorates materials *** segregation defect in Mg-6Gd alloy is studied by coupling macro thermal-solutal-convection transport and micro dendrite *** macroscopic fluid dynamics and mass transfer equations are resolved to forecast the segregation behavior under conditions of continuous temperature variation during the solidification *** numerical model is validated by testing double-diffusive natural convection in a closed square cavity.A phase field model is then applied to simulate the micro dendrite growth,using macro undercooling and liquid flow velocity as boundary *** show that the multiscale segregation behavior,including macro solute distribution and micro dendritic morphology,is strongly dependent on the temperature condition and the liquid convection,which provides guidance for reducing and eliminating the segregation defect.

关键词： magnesium alloy segregation numerical simulation phase-field simulation multiscale

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Profile of *** chen

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Science China(Life Sciences) 2024年第10期67卷 2062-2063页

作者： chen, Songlin Yellow Sea Fisheries Res Inst Qingdao Peoples R China

For decades,*** has been engaged in fish germplasm preservation,sex control and research focusing on molecular breeding for disease *** discovered the first sex-specific molecular marker of fish(chen et al.,2007),deciphered the first fish genome(Chinese tongue sole)in China,revealed the sex determination mechanism in fish(chen et al.,2014),and created molecular sex control *** also discovered the molecular mechanism of metamorphosis in flatfish(Shao et al.,2017)and developed China's first fish disease-resistant breeding gene chip,“YUXIN No.1”.

关键词： technology breeding

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Effect of annealing treatment on the microstructure and mechanical properties of warm-rolled Mg-Zn-Gd-Ca-Mn alloys

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International Journal of Minerals,Metallurgy and Materials 2024年第10期31卷 2208-2220页

作者： Yifan Song Xihai Li Jinliang Xu Kai Zhang Yaozong Mao hong Yan Huiping Li Rongshi chen School of Materials Science and Engineering Shandong University of Science and TechnologyQingdao 266590China Shi-changxu Innovation Center for Advanced Materials Institute of Metal ResearchChinese Academy of SciencesShenyang 110016China Shandong Key Laboratory of Advanced Aluminium Materials and Technology Binzhou Institute of TechnologyBinzhou 256606China

The basal texture of traditional magnesium alloy AZ31 is easy to form and exhibits poor plasticity at room *** address these problems,a multi-micro-alloyed high-plasticity Mg-1.8Zn-0.8Gd-0.1Ca-0.2Mn(wt%)alloy was developed using the unique role of rare earth and Ca solute *** addition,the influence of the annealing process on the grain size,second phase,texture,and mechanical properties of the warm-rolled sheet at room temperature was analyzed with the goal of developing high-plasticity mag-nesium alloy sheets and obtaining optimal thermal-mechanical treatment *** results show that the annealing temperature has a significant effect on the microstructure and properties due to the low alloying content:there are small amounts of larger-sized block and long string phases along the rolling direction(RD),as well as several spherical and rodlike particle phases inside the *** increas-ing annealing temperature,the grain size decreases and then increases,and the morphology,number,and size of the second phase also change *** particle phase within the grains vanishes at 450℃,and the grain size increases *** the full recrystal-lization stage at 300-350℃,the optimum strength-plasticity comprehensive mechanical properties are presented,with yield strengths of 182.1 and 176.9 MPa,tensile strengths of 271.1 and 275.8 MPa in the RD and transverse direction(TD),and elongation values of 27.4%and 32.3%,***,there are still some larger-sized phases in the alloy that influence its mechanical properties,which offers room for improvement.

关键词： Mg-Zn-Gd-Ca-Mn alloy annealing treatment microstructure texture dynamic recrystallization mechanical properties

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Facile preparation of ZIF-8/ZIF-67-derived biomass carbon composites for highly efficient electromagnetic wave absorption

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Chinese Chemical Letters 2024年第9期35卷 376-381页

作者： Xuexia Lin Yihui Zhou Jiafu hong Xiaofeng Wei Bin Liu Chong-chen Wang College of Chemical Engineering Huaqiao UniversityXiamen 361021China Beijing Key Laboratory of Functional Materials for Building Structure and Environment Remediation Beijing University of Civil Engineering and ArchitectureBeijing 100044China College of Materials Science and Engineering Huaqiao UniversityXiamen 361021China

Biomass absorbing materials have received increasing attention for electromagnetic wave(EMW)absorption field absorbing materials due to its low density and high dielectric ***,the biomass EMW absorbing materials often suffer from the insufficient magnetic loss and impedance *** this work,a facile ZIF-8/ZIF-67-derived biomass composites(CoZnO@BPC)was prepared for high-performance EMW absorption based on multi-component micro,nano structures metal particles and xanthoce sorbifolia bunge shells-derived biomass porous carbon(BPC).The dielectric loss and/or magnetic loss abilities of CoZnO@BPC composites were adjusted by changing the mass ratio of Zn^(2+)to Co^(2+)*** the filled amount of 20 wt%,Co Zn O@BPC exhibited excellent EMW absorption with the minimum reflection loss(RL)at 15.84 GHz is-50.2 dB,and the matching thickness is only 1.7 *** adjusting the ZIFs mass ratio,the effective absorption bandwidth(EAB)can be up to 5.92 GHz(from 12.08 GHz to 18 GHz),and the matching thickness is only 1.9 *** results provide a new insight for the economical and efficient preparation of lightweight and advanced microwave absorbing materials.

关键词： Biomass Metal organic framework EMW absorption Dielectric loss Magnetic loss

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