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Current understanding of ELF4 deficiency:a novel inborn error of immunity

World Journal of Pediatrics 2024年第5期20卷 444-450页

作者： Hong-Qiang Du xiao-dong zhao Department of Rheumatology&Immunology National Clinical Research Center for Child Health and DisordersMinistry of Education Key Laboratory of Child Development and DisordersChongqing Key Laboratory of Child Rare Diseases in Infection and ImmunityChildren’s Hospital of Chongqing Medical UniversityZhongshan Er Road 136Yuzhong DistrictChongqingChina

Background ELF4 deficiency has been recently recognized as a novel disorder within the spectrum of inborn errors of immunity(IEIs),specifically categorized as a“disease of immune dysregulation.”Cases of this condition,reported by our team and others,are very limited *** such,our current knowledge of this new disease remains *** review aims to provide a brief overview of the clinical manifestations,pathogenesis,and treatment strategies for this novel *** sources A comprehensive review was conducted after an extensive literature search in the PubMed/Medline database and websites concerning transcriptional factor ELF4 and reports concerning patients with ELF4 *** search strategy was“ELF4 OR ETS-related transcription factor Elf-4 OR EL4-like factor 4 OR myeloid Elf-1-like factor”as of the time of manuscript *** The current signature manifestations of ELF4 deficiency disorder are recurrent and prolonged oral ulcer,abdominal pain,and diarrhea in pediatric *** some cases,immunodeficiency and autoimmunity can also be *** Sanger sequencing or whole exome sequencing can be used to detect variation in ELF4 *** blotting for ELF4 expression of the patient’s cells can confirm the pathogenic effect of the *** fully confirm the pathogenicity of the variant,further functional test is strongly *** and biologics are the mainstream management of ELF4 deficiency *** Pediatric males presenting with recurring ulcerations in digestive tract epithelium with or without recurrent fever should be suspected of *** atypical presentations are prominent,variations in ELF4 gene should be carefully evaluated functionally due to the complex nature of ELF4 *** of treating DEX includes use of glucocorticoid and biologics and more precise treatment needs more patients to identify and further mechanistic study.

关键词： ELF4 transcription factor Immune dysregulation Inborn errors of immunity Mechanism Recurrent infections

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Research on the Dynamic Compressibility of Polyurethane Microcellular Elastomer and its Application for Impact Resistance

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Chinese Journal of Polymer Science 2024年第8期42卷 1185-1197页

作者： Zhi-Ying zhao Hao Jiang xiao-dong Li Xu-dong Zhang Xing Su Mei-Shuai Zou School of Materials Science and Engineering Beijing Institute of TechnologyBeijing 100081China

The packaging materials with cushioning performance are used to prevent the internal contents from being damaged by the impact and vibration of external *** polyurethane microcellular elastomers(PUMEs)can absorb energy through cell collapse and molecular chain *** this study,PUMEs with different densities were investigated by scanning electron microscopy,dynamic mechanical analysis and dynamic compression *** exhibited significant im pact resistance and the maximum peak stress attenuation ratio reached 73.33%.The protective equipment was made by PUME with the optimal density of 600 kg/m^(3),and then the acceleration sensing device installed with the same protective equipment fell from a height of 3,5 and 10 m to evaluate the energy-absorbing property and reusability of *** results showed that PUMEs equipment reduced the peak acceleration of the device by 93.84%,with a maximum deviation of 9%between actual test and simulation,and shortened the impact time of first landing by 57.39%.In addition,the equipment PUMEs equipment could effectively reduce the stress on the protected items.

关键词： Polyurethane microcellular elastomer Impact velocity Stress attenuation ratio Impact resistance

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Resilient control of flexible hypersonic vehicles against unmatched distributed faults

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Chinese Journal of Aeronautics 2024年第5期37卷 60-70页

作者： dong zhao Wenjing REN School of Artificial Intelligence Anhui UniversityHefei 230601China Key Laboratory of System Control and Information Processing Ministry of EducationShanghai Jiao Tong UniversityShanghai 200240China School of Electrical Engineering and Automation Hefei University of TechnologyHefei 230009China

This paper studies a robust fault compensation and vibration suppression problem of flexible hypersonic *** controlled plant is represented by a cascade system composed of a nonlinear Ordinary Differential Equation(ODE)and an Euler-Bernoulli Beam Equation(EBBE),in which the vibration dynamics is coupled with the rigid dynamics and suffers from distributed faults.A state differential transformation is introduced to transfer distributed faults to an EBBE boundary and a longitudinal dynamics is refined by utilizing T-S fuzzy IF-THEN rules.A novel T-S fuzzy based fault-tolerant control algorithm is developed and related stability conditions are *** robust exponential stability and well-posedness are proved by using the modified l_(0)-semigroup based Lyapunov direct approach.A simulation study on the longitudinal dynamics of flexible hypersonic vehicles effectively verifies the validity of the developed theoretical results.

关键词： Flexible hypersonic vehicles ODE-EBBE cascade Robust exponential stability Unmatched distributed fault compensation Vibration suppression

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The extraction of effective components and an antioxidant activity study of Tulipa edulis

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Food Science and Human Wellness 2024年第1期13卷 276-286页

作者： Doudou Zhang dong xiao Tingting Yin Shuangzhi zhao Olena Zhur Xun xiao Hailun He Leilei Chen Beijing Advanced Innovation Center for Food Nutrition and Human Health Beijing Technology and Business UniversityBeijing 102488China School of Life Sciences Central South UniversityChangsha 410013China State Key Laboratory of Coal Resources and Safe Mining University of Mining and TechnologyXuzhou 221116China Institute of Agro-Food Science and Technology Shandong Academy of Agricultural SciencesJinan 250100China

Plant extracts from natural sources are an excellent choice for food additives and natural *** this study,the active components of Tulipa edulis were extracted and analysed,and their antioxidant capacity was ***,the crude extract mixture was separated and purified using a Sephadex LH-20 gel,and the antioxidant activity of the purified products was *** umbilical vein endothelial human umbilical vein endothelial cells(HUVEC)cells were treated with 35 mmol/L glucose to construct a model of oxidative ***,the cells were treated with the active component to observe whether the products of *** could have a good protective effect on HUVEC cells induced by *** analysis was also performed on HUVEC cells after same treatment to explore the possible mechanism of the component F2 protecting HUVEC cells from oxidative stress induced by high *** results showed that component F2 obtained from *** has strong antioxidant ***,F2 can play a strong antioxidant protective role in HUVEC ***,the gene expression of heme oxygenase 1(HO-1),γ-glutamyl cysteine ligase catalytic subunit(GCLC)and NAD(P)H quinone oxidoreductase-1(NQO1)in HUVEC cells was up-regulated after treated with *** study provides reference value for the further development and application of *** and the d evelopment of functional food.

关键词： Tulipa edulis Oxidative stress Purification Antioxidant

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Recent advances in vacancy engineering for reliable lithiumsulfur batteries

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Rare Metals 2024年第2期43卷 455-477页

作者： Wen-dong Liu xiao Tang Jun-An Feng Chao-Yue Zhang Hao Liu Chuan Shi xiao-Xian zhao Jian-Jun Song College of Physics School of Chemistry and Chemical EngineeringQingdao UniversityQingdao266071China Faculty of Science Centre for Clean Energy TechnologySchool of Mathematical and Physical SciencesUniversity of Technology SydneySydneyNSW2007Australia Department of Chemistry College of ScienceHebei Agricultural UniversityBaoding071001China

The high-energy-density and low-cost features endow lithium-sulfur batteries with broad application ***,many drawbacks,especially the detrimental shuttle effect,have hindered the further development of *** response,a lot of new structures have been applied to suppress the shuttle effect and promote the development of ***,vacancy engineering has gained the attention of researchers due to its unique electronic *** review aims to analyze the application of vacancy engineering in ***,the electrochemistry of LSBs has been systematically discussed and the existing challenges as well as improvement tactics of LSBs have also been ***,the preparation methods and characterization technologies of various vacancies are summarized,including oxygen vacancies,sulfur vacancies,selenium vacancies,other anion vacancies,cation vacancies,*** latest applications of vacancy engineering in LSBs are also summarized in this ***,some prospects and insights for further investigation and practical application of vacancy engineering in LSBs are put forward.

关键词： Lithium-sulfur batteries(LSBs) Defects Vacancy engineering Electronic structure Shuttle effect

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On Minor Left Prime Factorization Problem for Multivariate Polynomial Matrices

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Journal of Systems Science & Complexity 2024年第3期37卷 1295-1307页

作者： LU dong WANG Dingkang xiao Fanghui School of Mathematics Southwest Jiaotong UniversityChengdu 610031China KLMM Academy of Mathematics and Systems ScienceChinese Academy of SciencesBeijing 100190China School of Mathematical Sciences University of Chinese Academy of SciencesBeijing 100049China MOE-LCSM School of Mathematics and StatisticsHunan Normal UniversityChangsha 410081China

A new necessary and sufficient condition for the existence of minor left prime factorizations of multivariate polynomial matrices without full row rank is *** key idea is to establish a relationship between a matrix and any of its full row rank *** on the new result,the authors propose an algorithm for factorizing matrices and have implemented it on the computer algebra system *** examples are given to illustrate the effectiveness of the algorithm,and experimental data shows that the algorithm is efficient.

关键词： Free modules Grobner bases minor left prime(MLP) multivariate polynomial matrices polynomial matrix factorizations

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Coupled interactive effects of ammonia and hydrogen additions on ethylene diffusion flames: A detailed kinetic study

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Science China(Technological Sciences) 2024年第3期67卷 930-948页

作者： xiao JiaYi XU TingTing SHU ZhiMei DENG QiGe zhao Xuan YING YaoYao LI TianJiao LIU dong MIIT Key Laboratory of Thermal Control of Electronic Equipment School of Energy and Power EngineeringNanjing University of Science and TechnologyNanjing 210094China Advanced Combustion Laboratory School of Energy and Power EngineeringNanjing University of Science and TechnologyNanjing210094China

In order to create effective combustion technologies and fuels with low or no carbon emissions,the research was conducted to assess the coupled interactive effects of NH_(3) and H_(2) additions on ethylene counterflow diffusion flames from a kinetic *** effects of the NH_(3)/H_(2) combination on flame temperatures,major species,key radicals,important intermediate species,representative oxygenated species and NO_xwere *** results of the study utilizing fictitious inert NH_(3) and/or H_(2) revealed the chemical effects of the two *** was found that the NH_(3)/H_(2) coupled effects had a more effective inhibitory effect on soot precursors than the effects of corresponding sum of single NH_(3) or H_(2) *** production of soot precursors was promoted by the coupled chemical effects of NH_(3) and H_(2),but the coupled dilution and thermal effects were observed to have a greater impact,resulting in a decrease of the mole fractions of soot *** for the interaction of NH_(3) and H_(2) effects,the presence of H_(2) decreased the chemical effects of NH_(3) on the augmentation of C_(2)H_(2),A1,A2,and CH_(3)CHO mole *** NH_(3) addition alleviated the H_(2) chemical effects on increasing C_(2)H_(2),C_(3)H_(3),A1 and A2 ***,the NH_(3)chemical effects on C_(3)H_(3),OH and CH_(3)CHO were enhanced when H_(2) was *** presence of NH_(3) augmented the chemical effects of H_(2) on the growth of OH mole ***,the H_(2) chemical effects hindered the production of NO and NO_(2) in the presence of NH_(3).

关键词： NH_(3)/H_(2)addition kinetic analysis coupled interactive effects chemical effects

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Toward Improved Accuracy in Quasi-Static Elastography Using Deep Learning

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Computer Modeling in Engineering & Sciences 2024年第4期139卷 911-935页

作者： Yue Mei Jianwei Deng dongmei zhao Changjiang xiao Tianhang Wang Li dong Xuefeng Zhu Department of Engineering Mechanics State Key Laboratory of Structural Analysis for Industrial EquipmentDalian University of TechnologyDalian116023China International Research Center for Computational Mechanics Dalian University of TechnologyDalian116023China DUT-BSU Joint Institute Dalian University of TechnologyDalian116023China Department of Automotive Engineering Tongji UniversityShanghai201804China *** SeattleWA98109USA School of Automotive Engineering Dalian University of TechnologyDalian116023China

Elastography is a non-invasive medical imaging technique to map the spatial variation of elastic properties of soft *** quality of reconstruction results in elastography is highly sensitive to the noise induced by imaging measurements and *** address this issue,we propose a deep learning(DL)model based on conditional Generative Adversarial Networks(cGANs)to improve the quality of nonhomogeneous shear modulus *** train this model,we generated a synthetic displacement field with finite element simulation under known nonhomogeneous shear modulus *** the simulated and experimental displacement fields are used to validate the proposed *** reconstructed results demonstrate that the DL model with synthetic training data is able to improve the quality of the reconstruction compared with the well-established optimization ***,we emphasize that our DL model is only trained on synthetic *** might provide a way to alleviate the challenge of obtaining clinical or experimental data in ***,this work addresses several fatal issues in applying the DL technique into elastography,and the proposed method has shown great potential in improving the accuracy of the disease diagnosis in clinical medicine.

关键词： Nonhomogeneous elastic property distribution reconstruction deep learning finite element method inverse problem elastography conditional generative adversarial network

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Two-dimensional bifunctional electrocatalyst(Mo-NiFe-LDH)with multilevel structure for highly efficient overall water splitting

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Rare Metals 2024年第6期43卷 2613-2622页

作者： Bao-Jie Zhang Bin Chang Shi-Peng Qiu Gang zhao xiao Wang Xi-Jin Xu Lan Mu Wen-Bo Liao xiao-Jing dong School of Physics and Technology University of JinanJinan 250000China KAUST Catalysis Center(KCC) King Abdullah University of Science and Technology(KAUST)Thuwal 23955-6900Kingdomof SaudiArabia School of Chemical Engineering and Technology Tianjin UniversityTianjin 300072China School of Physics and Physical Engineering Qufu Normal UniversityQufu273100China

To effectively address energy challenges,it is crucial to explore efficient and stable bifunctional nonprecious metal *** this study,a Mo-doped nickeliron layered double hydroxide with flower-cluster architecture was successfully prepared by a one-step hydrothermal method,which demonstrated a good water splitting *** an appropriate amount of Mo doping,some lattice distortions in the material provided reactive sites for the adsorption and conversion of intermediates,thus optimising the charge distribution of the ***,the multidimensional void structures formed after doping had a larger specific surface area and accelerated the penetration of the electrolyte,which significantly improved the activity of the catalyst in alkaline *** 10 mA·cm^(-2),the hydrogen and oxygen evolution overpotentials of Mo-doped nickel-iron double hydroxides(Mo-NiFe LDH/NF-0.2)were 167 and 220 mV,respectively,with an excellent durability up to 24 *** the Mo-NiFe LDH/NF-0,2 catalyst was used as the cathode and anode of an electrolytic cell,the catalyst achieved a current density of 10 mA·cm^(-2)at an applied voltage of 1.643 *** study provides a novel approach for designing excellent bifunctional electrocatalysts containing nonprecious metals.

关键词： Bifunctional electrocatalysts 2D multilevel structure Overall water splitting Mo-doped Ni-Fe LDH

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Direct numerical simulation of shock wave/boundary layer interaction controlled by steady microjet in a compression ramp

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Chinese Journal of Aeronautics 2024年第10期37卷 83-102页

作者： Ruoye xiao dong SUN Jian YU School of Aeronautic Science and Engineering Beihang UniversityBeijing 100191China State Key Laboratory of Aerodynamics Mianyang 621000China

Shock wave/boundary layer interaction in a 24°turning angle of the compression ramp at Mach number 2.9 controlled by steady microjet is investigated using direct numerical *** different jet spacings which are termed as sparse,moderate and dense are considered,and the induced vortex system and shock structures are compared.A moderate jet spacing configuration is found to generate counter-rotating vortex pairs that transport high-momentum fluid towards the vicinity of wall and strengthen the boundary layer to resist separation,reducing the separation *** dense jet spacing configuration creates a larger momentum deficit region,reducing the friction downstream of the *** of pressure and pressure gradient reveals that dense jet spacing configuration reduces the intensity of separation *** impact of varying jet spacings on the turbulent kinetic energy transport mechanism is also investigated by decomposing the budget terms in the transport ***,the spectral characteristics of the separation region are studied using power spectral density and dynamic mode decomposition methods,revealing that moderate jet spacing configuration suppresses low-frequency fluctuations in the separation region.

关键词： Shock wave/boundary layer interaction Compression ramp Steady microjet Different jet spacings Direct numerical simulation

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