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PACS gene family-related neurological diseases: limited genotypes and diverse phenotypes

World Journal of Pediatrics 2024年第1期20卷 82-91页

作者： Han zhang Kai Gao Shuang Wang Yue-Hua zhang zhi-Xian Yang Ye Wu Yu-Wu Jiang Department of Pediatrics Peking University First Hospital.No.1 Xi'an Men StreetWest DistrictBeijing 100034China Bejing Key Laboratory of Molecular Diagnosis and Study on Pediatric Genetic Diseases BeijingChina Children Epilepsy Center Peking University First HospitalBeijingChina Key Laboratory for Neuroscience Ministry of Education/National Health and Family Planning CommissionPeking UniversityBeijingChina Center of Epilepsy Beijing Institute for Brain DisordersBeijingChina

Background The PACS gene family has been demonstrated to be related to intracellular vesicular *** phenotypic manifestations caused by the pathogenic variants of PACS include epilepsy,intellectual disability/developmental delay,and malformations,such as facial *** We identified seven new cases with pathogenic or likely pathogenic PACS variants using next-generation *** information obtained from these patients was analyzed along with that obtained from previously reported *** With the inclusion of the newly diagnosed cases in this study,103 cases with PACS gene family-related neurological diseases were reported,of which 43 were PACS2-related cases and the remaining were PACSI-related *** patients had seizures,which have been reported to be effectively controlled by several types of anti-seizure medications(ASMs).The most efficacious and frequently prescribed ASMs included sodium valproate(43.3%,13/30),oxcarbazepine/carbamazepine(26.7%,8/30),and levetiracetam(20%,6/30).Almost all patients had intellectual disability/developmental *** most common pathogenic missense variants were PACSI ***203Trp and PACS2 *** addition,we report a patient carrying a likely pathogenic copy number variation(CNV)(de novo heterozygous deletion of chr14:105821380-106107443,286 kilobase,destroyed part of the furin-binding region domain and the protein structure after it)with more severe and refractory late-onset *** The clinical phenotypes of the different PACS heterozygous missense variants were *** pathogenic variant sites of PACSI and PACS2 were quite limited but located in different regions.A CNV destroying part of the PACS2 gene might also be *** findings may provide an important clue for further functional studies on the pathogenic mechanism of neurological disorders related to the PACS gene family.

关键词： Developmental delay Epilepsy Intellectual disability PACSI PACS2

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Controllable fabrication of FeCoS_(4) nanoparticles/S-doped bowl-shaped hollow carbon as efficient lithium storage anode

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Chinese Journal of Chemical Engineering 2024年第3期67卷 78-88页

作者： Ming Zhou Mengrong Wu Haiwei Yu Xiangjun Zheng kuan Shen Xingmei Guo Yuanjun Liu Fu Cao Hongxing Gu Qinghong Kong Junhao zhang School of Environmental and Chemical Engineering Jiangsu University of Science and TechnologyZhenjiang 212003China Jiangsu Hengshen Co. LtdZhenjiang 212314China School of the Environment and Safety Engineering Jiangsu UniversityZhenjiang 212013China

To address the low conductivity and easy agglomeration of transition metal sulfide nanoparticles,FeCoS_(4) nanoparticles embedded in S-doped hollow carbon(FeCoS_(4)@S-HC)composites were successfully fabricated through a combination of hydrothermal processes and sulfidation *** unique bowlshaped FeCoS_(4)/S-HC composites exhibit excellent structural stability with a high specific surface area of 303.7 m^(2)·g^(-1) and a pore volume of 0.93 cm^(3)·g^(-1).When applied as anode material for lithium-ion batteries,the FeCoS_(4)@S-HC anode exhibits efficient lithium storage with high reversible specific capacity(970.2 mA·h·g^(-1) at 100 mA·g^(-1))and enhanced cycling stability(574 mA·h·g^(-1) at 0.2 A·g^(-1) after 350 cycles,a capacity retention of 84%).The excellent lithium storage is attributed to the fact that the bimetallic FeCoS_(4) nanoparticles with abundant active sites can accelerate the electrochemical reaction kinetics,and the bowl-shaped S-HC structure can provide a stable mechanical structure to suppress volume expansion.

关键词： Bimetallic sulfides Bowl-shaped hollow carbon S doping Buffering volume expand Lithium-ion batteries

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Dynamic coupling of ferrihydrite transformation and associated arsenic desorption/redistribution mediated by sulfate-reducing bacteria

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Journal of Environmental Sciences 2024年第1期135卷 39-50页

作者： Wenjing Zhou Huiyan Zhu Shiwen Hu Bowei zhang Kun Gao zhi Dang Chongxuan Liu State Environmental Protection Key Laboratory of Integrated Surface Water-Groundwater Pollution Control School of Environmental Science and EngineeringSouthern University of Science and TechnologyShenzhen 518055China The Key Lab of Pollution Control and Ecosystem Restoration in Industry Clusters Ministry of EducationSouth China University of TechnologyGuangzhou 510006China

Sulfate-reducing bacteria play an important role in the geochemistry of iron(oxyhydr)oxide and arsenic(As)in natural environments;however,the associated reaction processes are yet to be fully *** this study,batch experiments coupled with geochemical,spectroscopic,microscopic,and thermodynamic analyses were conducted to investigate the dynamic coupling of ferrihydrite transformation and the associated As desorption/redistribution mediated by Desulfovibrio vulgaris(***).The results indicated that *** could induce ferrihydrite transformation via S^(2-)-driven and direct reduction *** the absence of SO_(4)^(2-),*** directly reduced ferrihydrite,and As desorption and re-sorption occurred simultaneously during the partial transformation of ferrihydrite to *** increase in SO_(4)^(2-)loading promoted the S^(2-)-driven reduction of ferrihydrite and accelerated the subsequent mineralogical *** the low and medium SO_(4)^(2-)treatments,ferrihydrite was completely transformed to a mixture of magnetite and mackinawite,which increased the fraction of As in the residual phase and stabilized *** the high SO_(4)^(2-)treatment,although the replacement of ferrihydrite by only mackinawite also increased the fraction of As in the residual phase,22.1%of the total As was released into the solution due to the poor adsorption affinity of As to mackinawite and the conversion of As^(5+)to As^(3+).The mechanisms of ferrihydrite reduction,mineralogy transformation,and As mobilization and redistribution mediated by sulfate-reducing bacteria are closely related to the surrounding SO_(4)^(2-)*** results advance our understanding of the biogeochemical behavior of Fe,S,and As,and are helpful for the risk assessment and remediation of As contamination.

关键词： Sulfate-reducing bacteria Arsenic Ferrihydrite Transformation Redistribution

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A Multiscale Reliability-Based Design Optimization Method for Carbon-Fiber-Reinforced Composite Drive Shafts

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Computer Modeling in Engineering & Sciences 2024年第8期140卷 1975-1996页

作者： Huile zhang Shikang Li Yurui Wu Pengpeng zhi Wei Wang Zhonglai Wang Yangtze Delta Region Institute(Huzhou) University of Electronic Science and Technology of ChinaHuzhou313001China Huzhou Key Laboratory of Green Energy Materials and Battery Cascade Utilization Huzhou CollegeHuzhou313000China SAIC Motor Corporation Limited Passenger Vehicle Co. Shanghai201804China School ofMechanical and Electrical Engineering University of Electronic Science and Technology of ChinaChengdu611731China

Carbon fiber composites,characterized by their high specific strength and low weight,are becoming increasingly crucial in automotive ***,current research primarily emphasizes layer count and orientation,often neglecting the potential of microstructural design,constraints in the layup process,and performance *** study,therefore,introduces a multiscale reliability-based design optimization method for carbon fiber-reinforced plastic(CFRP)drive ***,parametric modeling of the microscale cell was performed,and its elastic performance parameters were predicted using two homogenization methods,examining the impact of fluctuations in microscale cell parameters on composite material performance.A finite element model of the CFRP drive shaft was then constructed,achieving parameter transfer between microscale and macroscale through Python *** enabled an investigation into the influence of both micro and macro design parameters on the CFRP drive shaft’s *** Multi-Objective Particle Swarm Optimization(MOPSO)algorithm was enhanced for particle generation and updating strategies,facilitating the resolution of multi-objective reliability optimization problems,including composite material layup process *** studies demonstrated that this approach leads to over 30%weight reduction in CFRP drive shafts compared to metallic counterparts while satisfying reliability requirements and offering insights for the lightweight design of other vehicle components.

关键词： Multiscale reliability-based design optimization carbon-fabric-reinforced composite drive shaft

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Consideration on immunotherapy of liver metastases of malignant tumors

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World Journal of Gastrointestinal Surgery 2024年第8期16卷 2374-2381页

作者： Chuang Jiang zhi-Hong zhang Jia-Xin Li Division of Liver Surgery Department of General Surgery and Laboratory of Liver Surgeryand State Key Laboratory of Biotherapy and Collaborative Innovation Center of BiotherapyWest China HospitalSichuan UniversityChengdu 610041Sichuan ProvinceChina Department of General Surgery Dafang County People's HospitalBijie 551600Guizhou ProvinceChina

In this editorial,we comment on the article“Analysis of the impact of immuno-therapy efficacy and safety in patients with gastric cancer and liver metastasis”by Liu et al that was published in the recent issue of the World Journal of Gastroin-testinal *** has prompted us to think and summarize some thoughts on immunotherapy for malignant tumor liver *** plays a crucial role in the treatment of malignant tumors;however,the presence of liver metastases in advanced tumors may impact its *** patients with liver metastases can still benefit from immunotherapy,multiple clinical studies have indicated that,compared to other sites of metastasis,liver metastases may diminish the effectiveness of *** efficacy of immune checkpoint inhibitors in patients with liver metastases often fails to reach the ideal level,primarily due to the liver metastases exploiting the host's peripheral immune to-lerance mechanisms to promote systemic CD8(+)T cell exhaustion,resulting in a systemic immune-tolerant *** article aims to summarize the reasons for the decreased efficacy of immunotherapy following liver metastasis in various malignant tumors and propose potential clinical strategies for manage-ment.

关键词： Liver metastasis Immunotherapy Immune tolerance Cancer Treatment

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Guest Editorial:Special issue on trustworthy machine learning for behavioural and social computing

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CAAI Transactions on Intelligence Technology 2024年第3期9卷 541-543页

作者： zhi-Hui Zhan Jianxin Li Xuyun zhang Deepak Puthal Nankai University TianjinChina Deakin University BurwoodVictoriaAustralia Macquarie University SydneyNew South WalesAustralia Khalifa University Abu DhabiUAE

Machine learning has been extensively applied in behavioural and social computing,encompassing a spectrum of applications such as social network analysis,click stream analysis,recommendation of points of interest,and sentiment *** datasets pertinent to these applications are inherently linked to human behaviour and societal dynamics,posing a risk of disclosing personal or sensitive information if mishandled or subjected to attacks.

关键词： computing handle learning

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Senegenin suppresses hepatocellular carcinoma by regulating O-GlcNAcylation

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World Journal of Gastrointestinal Oncology 2024年第9期16卷 3994-4005页

作者： Xiang zhang Li-Qiong Wang zhi-Yong Liu Department of Traditional Chinese Medicine Qingpu Branch of Zhongshan Hospital Affiliated to Fudan UniversityShanghai 201700China Department of Hepatology Longhua Hospital Affiliated to Shanghai University of Traditional Chinese MedicineShanghai 200032China

BACKGROUND Based on current knowledge,hepatocellular carcinoma(HCC)is a condition with numerous etiologies and risk ***,the pathogenesis of HCC remains *** To investigate the roles of senegenin and O-GlcNAcylation in the growth and metastasis of *** The levels of O-linked N-acetylglucosamine transferase(OGT)and O-GlcNAcylation in HCC cells and tissues were detected using western blot *** effects of senegenin and O-GlcNAcylation on the proliferation of HCC cells were investigated in vitro using cell counting kit-8 and clonogenic *** potential effects of senegenin and O-GlcNAcylation on HCC metastasis were examined using the transwell migration assay.O-GlcNAcylation levels were altered via drug treatment and lentiviral infection,and western blot analysis was used to detect proteins involved in various *** Western blot analysis revealed that OGT and O-GlcNAcylation levels were significantly elevated in HCC tissues and cells.O-GlcNAcylation levels in HCC cells were significantly altered by drug treatment and lentiviral *** increase in the glycosylation level was linked to enhanced proliferation,invasiveness,clonogenicity,and metastatic potential of cancer cells.O-GlcNAcylation induced by senegenin was found to slow the proliferation and migration of HCC *** levels of proteins involved in nuclear factor-kappa B(NF-κB)and c-Jun N-terminal kinase(JNK)pathways,which are associated with endoplasmic reticulum stress,were *** Senegenin lowers O-GlcNAcylation levels,decreases OGT expression,and inhibits cancer cell growth and metastasis by regulating proteins involved in NF-κB and JNK pathways.

关键词： Senegenin O-GlcNAcylation Liver cancer Pathogenesis Metastasis

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Bayesian network-based survival prediction model for patients having undergone post-transjugular intrahepatic portosystemic shunt for portal hypertension

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World Journal of Gastroenterology 2024年第13期30卷 1859-1870页

作者： Rong Chen Ling Luo Yun-zhi zhang Zhen Liu An-Lin Liu Yi-Wen zhang Department of Infectious Diseases Second Affiliated Hospital of Chongqing Medical UniversityChongqing 400016China

BACKGROUND Portal hypertension(PHT),primarily induced by cirrhosis,manifests severe symptoms impacting patient *** transjugular intrahepatic portosystemic shunt(TIPS)is a critical intervention for managing PHT,it carries risks like hepatic encephalopathy,thus affecting patient survival *** our knowledge,existing prognostic models for post-TIPS survival in patients with PHT fail to account for the interplay among and collective impact of various prognostic factors on ***,the development of an innovative modeling approach is essential to address this *** To develop and validate a Bayesian network(BN)-based survival prediction model for patients with cirrhosis-induced PHT having undergone *** The clinical data of 393 patients with cirrhosis-induced PHT who underwent TIPS surgery at the Second Affiliated Hospital of Chongqing Medical University between January 2015 and May 2022 were retrospectively *** were selected using Cox and least absolute shrinkage and selection operator regression methods,and a BN-based model was established and evaluated to predict survival in patients having undergone TIPS surgery for *** Variable selection revealed the following as key factors impacting survival:age,ascites,hypertension,indications for TIPS,postoperative portal vein pressure(post-PVP),aspartate aminotransferase,alkaline phosphatase,total bilirubin,prealbumin,the Child-Pugh grade,and the model for end-stage liver disease(MELD)*** on the above-mentioned variables,a BN-based 2-year survival prognostic prediction model was constructed,which identified the following factors to be directly linked to the survival time:age,ascites,indications for TIPS,concurrent hypertension,post-PVP,the Child-Pugh grade,and the MELD *** Bayesian information criterion was 3589.04,and 10-fold cross-validation indicated an average log-likelihood loss of 5.55 with a standard deviation of *** model’s acc

关键词： Bayesian network Cirrhosis Portal hypertension Transjugular intrahepatic portosystemic shunt Survival prediction model

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Re-evaluating the nano-sized inorganic protective layer on Cu current collector for anode free lithium metal batteries

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Chinese Chemical Letters 2024年第7期35卷 452-455页

作者： Shuo zhang Haitao Liao zhi-Qun Liu Chong Yan Jia-Qi Huang School of Materials Science&Engineering Beijing Institute of TechnologyBeijing 100081China Advanced Research Institute of Multidisciplinary Science Beijing Institute of TechnologyBeijing 100081China Wuxi Yiwen Microelectronics Technology Co. Ltd.Wuxi 214000China Shanxi Research Institute for Clean Energy Tsinghua UniversityTaiyuan 030032China

Anode free lithium metal batteries(AF-LMBs)have conspicuous advantages both in energy density and the compatibility of battery manufacturing ***,the limited cycle life of AF-LMBs is a crucial factor hindering its practical *** or nitride artificial inorganic solid electrolyte interphase(SEI)has been found as an effective method to prolong the lifespan of ***,by investigating the impact of nano-sized inorganic gradient layers(LiF or Li3N)on initial Li deposition behavior,we notice that the Li^(+) diffusion barrier and the deposition morphology are highly depended on the thickness of inorganic *** protective layers cause larger overpotential as well as more aggregated Li^(+) *** study reveals that the ideal SEI should be synthesized thin and uniformly enough and uncontrollable artificial SEI can cause damage to the lifespan of AF-LMBs.

关键词： Anode free lithium metal battery Solid electrolyte interphase Nano-sized protective layer Lithium fluoride Li deposition behavior

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Predicting dynamic compressive strength of frozen-thawed rocks by characteristic impedance and data-driven methods

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Journal of Rock Mechanics and Geotechnical Engineering 2024年第7期16卷 2591-2606页

作者： Shengtao Zhou Zong-Xian zhang Xuedong Luo Yifan Huang zhi Yu Xiaowei Yang Faculty of Engineering China University of GeosciencesWuhan430074China Oulu Mining School University of OuluOulu90570Finland Zijin School of Geology and Mining Fuzhou UniversityFuzhou350116China Department of Earth Science&Engineering Imperial College LondonLondonSW72AZUK

In cold regions,the dynamic compressive strength(DCS)of rock damaged by freeze-thaw weathering significantly influences the stability of rock ***,testing the dynamic strength under freeze-thaw weathering conditions is often both time-consuming and ***,this study considers the effect of characteristic impedance on DCS and aims to quickly determine the DCS of frozen-thawed rocks through the application of machine-learning ***,a database of DCS for frozen-thawed rocks,comprising 216 rock specimens,was *** external load parameters(freeze-thaw cycle number,confining pressure,and impact pressure)and two rock parameters(characteristic impedance and porosity)were selected as input variables,with DCS as the predicted *** research optimized the kernel scale,penalty factor,and insensitive loss coefficient of the support vector regression(SVR)model using five swarm intelligent optimization algorithms,leading to the development of five hybrid *** addition,a statistical DCS prediction equation using multiple linear regression techniques was *** performance of the prediction models was comprehensively evaluated using two error indexes and two trend indexes.A sensitivity analysis based on the cosine amplitude method has also been *** results demonstrate that the proposed hybrid SVR-based models consistently provided accurate DCS *** these models,the SVR model optimized with the chameleon swarm algorithm exhibited the best performance,with metrics indicating its effectiveness,including root mean square error(RMSE)﹦3.9675,mean absolute error(MAE)﹦2.9673,coefficient of determination(R^(2))﹦0.98631,and variance accounted for(VAF)﹦*** suggests that the chameleon swarm algorithm yielded the most optimal results for enhancing SVR ***,impact pressure and characteristic impedance emerged as the two most influential parameters in DCS *** research is

关键词： Freeze-thaw cycle Characteristic impedance Dynamic compressive strength Machine learning Support vector regression

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