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

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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

Large‑scale transcriptomic and genomic analyses reveal a novel functional gene SERPINB6 for chicken carcass traits

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Journal of Animal Science and Biotechnology 2024年第4期15卷 1466-1481页

作者： Di zhao Ranran Liu Xiaodong Tan Huimin Kang Jie Wang Zheng Ma Haiquan zhao Hai Xiang Zhengfen Zhang Hua Li Guiping zhao Guangdong Provincial Key Laboratory of Animal Molecular Design and Precise Breeding School of Life Science and EngineeringFoshan UniversityFoshanChina State Key Laboratory of Animal Biotech Breeding Institute of Animal SciencesChinese Academy of Agricultural SciencesBeijingChina Guangdong Tinoo’s Foods Group Co. Ltd.QingyuanChina

Background Carcass traits are crucial indicators of meat production ***,the molecular regulatory mechanisms associated with these traits remain *** In this study,we conducted comprehensive transcriptomic and genomic analyses on 399 Tiannong partridge chickens to identify key genes and variants associated with carcass traits and to elucidate the underlying regulatory *** on association analyses with the elastic net(EN)model,we identified 12 candidate genes(AMY1A,AP3B2,CEBPG,EEF2,EIF4EBP1,FGFR1,FOXD3,GOLM1,LOC107052698,PABPC1,SERPINB6 and TBC1D16)for 4 carcass-related traits,namely live weight,dressed weight,eviscerated weight,and breast muscle ***6 was identified as the only overlapping gene by 3 analyses,EN model analysis,weighted gene co-expression network analysis and differential expression ***-level experiments confirmed that SERPINB6 promotes the proliferation of chicken DF1 cells and primary *** expression genome-wide association study and association analysis indicated that rs317934171 is the critical site that enhances SERPINB6 ***,a dual-luciferase reporter assay proved that gga-miR-1615 targets the 3′UTR of *** Collectively,our findings reveal that SERPINB6 serves as a novel gene for chicken carcass traits by promoting fibroblast and myoblast ***,the downstream variant rs317934171 regulates SERPINB6 *** results identify a new target gene and molecular marker for the molecular mechanisms of chicken carcass traits.

关键词： Carcass traits Chicken Genome SERPINB6 Transcriptome

Solar manipulations of perpendicular magnetic anisotropy for flexible spintronics

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Frontiers of physics 2024年第4期19卷 73-79页

作者： Zhexi He Yifan zhao Yujing Du Meng zhao Yuxuan Jiang Ming Liu Ziyao Zhou State Key Laboratory for Manufacturing Systems Engineering Electronic Materials Research LaboratoryKey Laboratory of the Ministry of Education&International Center for Dielectric ResearchSchool of Electronic Science and EngineeringThe International Joint Laboratory for Micro/Nano Manufacturing and Measurement TechnologyXi’an Jiaotong UniversityXi’an 710049China School of Materials Science&Engineering Changzhou UniversityChangzhou 213164China

Flexible electronics/spintronics attracts researchers’attention for their application potential abroad in wearable devices,healthcare,and other *** devices’performance(speed,energy consumption)is highly dependent on manipulating information bits(spin-orientation in flexible spintronics).In this work,we established an organic photovoltaic(OPV)/ZnO/Pt/Co/Pt heterostructure on flexible PET substrates with perpendicular magnetic anisotropy(PMA).Under sunlight illumination,the photoelectrons generated from the OPV layer transfer into the PMA heterostructure,then they reduce the PMA strength by enhancing the interfacial Rashba field *** coercive field(Hc)reduces from 800 Oe to 500 Oe at its maximum,and the magnetization can be switched up and down *** stability of sunlight control of magnetization reversal under various bending conditions is also tested for flexible spintronic ***,the voltage output of sunlight-driven PMA is achieved in our prototype device,exhibiting an excellent angular dependence and opening a door towards solar-driven flexible spintronics with much lower energy consumption.

关键词： interfacial magnetoelectric coupling perpendicular magnetic anisotropy deterministic magnetization reversal photovoltaic control of magnetism

Cellular atlases of ovarian microenvironment alterations by diet and genetically-induced obesity

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Science China(Life Sciences) 2024年第1期67卷 51-66页

作者： Yonghui Jiang Xueying Gao Yue Liu Xueqi Yan Huangcong Shi Rusong zhao Zi-Jiang Chen Fei Gao Han zhao Shigang zhao Center for Reproductive Medicine Shandong UniversityJinan 250012China Research Unit of Gametogenesis and Health of ART-Offspring Chinese Academy of Medical Sciences(No.2021RU001)Jinan 250012China Key Laboratory of Reproductive Endocrinology of Ministry of Education Shandong UniversityJinan 250012China Shandong Key Laboratory of Reproductive Medicine Shandong Provincial Hospital Affiliated to Shandong First Medical UniversityJinan 250012China Shandong Provincial Clinical Research Center for Reproductive Health Jinan 250012China National Research Center of Assisted Reproductive Technology and Reproductive Genetics Shandong UniversityJinan 250012China The Affiliated Suzhou Hospital of Nanjing Medical University Suzhou Municipal HospitalGusu SchoolNanjing Medical UniversitySuzhou 215000China Center for reproductive medicine Ren Ji HospitalSchool of MedicineShanghai Jiao Tong UniversityShanghai 200127China Shanghai Key Laboratory for Assisted Reproduction and Reproductive Genetics Shanghai 200127China State Key Laboratory of Stem Cell and Reproductive Biology Institute of ZoologyChinese Academy of ScienceBeijing 100101China

Obesity,which can arise from genetic or environmental factors,has been shown to cause serious damages to the reproductive *** ovary,as one of the primary regulators of female fertility,is a complex organ comprised of heterogeneous cell types that work together to maintain a normal ovarian microenvironment(OME).Despite its importance,the effect of obesity on the entire ovary remains poorly *** this study,we performed ovary single-cell and nanoscale spatial RNA sequencing to investigate how the OME changed under different kinds of obesity,including high-fat diet(HFD)induced obesity and Leptin ablation induced obesity(OB).Our results demonstrate that OB,but not HFD,dramatically altered the proportion of ovarian granulosa cells,theca-interstitial cells,luteal cells,and endothelial ***,based on the spatial dynamics of follicular development,we defined four subpopulations of granulosa cell and found that obesity drastically disrupted the differentiation of mural granulosa cells from small to large antral ***,HFD enhanced follicle-stimulating hormone(FSH)sensitivity and hormone conversion,while OB caused decreased sensitivity,inadequate steroid hormone conversion,and impaired follicular *** differences can be explained by the differential expression pattern of the transcription factor ***,our study provides a powerful and high-resolution resource for profiling obesity-induced OME and offers insights into the diverse effects of obesity on female reproductive disorders.

关键词： obesity ovarian microenvironment snRNA-seq Stereo-seq Foxo1

Effect of long reaction distance on gas composition from organic-rich shale pyrolysis under high-temperature steam environment

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International Journal of Coal Science & Technology 2024年第3期11卷 102-119页

作者： Lei Wang Rui Zhang Guoying Wang Jing zhao Dong Yang Zhiqin Kang Yangsheng zhao Key Laboratory of In-Situ Property Improving Mining of Ministry of Education Taiyuan University of TechnologyTaiyuan030024China The In-Situ Steam Injection Branch of State Center for Research and Development of Organic-Rich Shale Exploitation Taiyuan University of TechnologyTaiyuan030024China College of Mining Engineering Taiyuan University of TechnologyTaiyuan030024China School of Energy Science and Engineering Henan Polytechnic UniversityJiaozuo454002China

When high-temperature steam is used as a medium to pyrolyze organic-rich shale,water steam not only acts as heat transfer but also participates in the chemical reaction of organic matter pyrolysis,thus affecting the generation law and release characteristics of gas *** this study,based on a long-distance reaction system of organic-rich shale pyrolysis via steam injection,the effects of steam temperature and reaction distance on gas product composition are analyzed in depth and compared with other pyrolysis *** advantages of organic-rich shale pyrolysis via steam injection are then *** volume concentration of hydrogen in the gas product obtained via the steam injection pyrolysis of organic-rich shale is the highest,which is more than 60%.The hydrogen content increases as the reaction distance is extended;however,the rate of increase changes *** the reaction distance from 800 to 4000 mm increases the hydrogen content from 34.91%to 69.68%and from 63.13%to 78.61%when the steam temperature is 500°C and 555°C,***,the higher the heat injection temperature,the smaller the reaction distance required to form a high concentration hydrogen pyrolysis environment(hydrogen concentration>60%).When the steam pyrolysis temperature is increased from 500°C to 555°C,the reaction distance required to form a high concentration of hydrogen is reduced from 3800 to 800 *** with the direct retorting process,the volume concentration of hydrogen obtained from high-temperature steam pyrolysis of organic-rich shale is 8.82 and 10.72 times that of the commonly used Fushun and Kivite furnaces,*** pyrolysis of organic-rich shale via steam injection is a pyrolysis process in a hydrogen-rich environment.

关键词： Steam temperature Pyrolysis gas Hydrogen-rich Reaction distance Direct retorting

Transmit Power Minimization for IRS-Assisted NOMA-UAV Networks

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China Communications 2024年第5期21卷 137-152页

作者： zhao Chen Pang Xiaowei Tang Jie Liu Mingqian zhao Nan Zhang Xiuyin Wang Xianbin School of Information and Communication Engineering Dalian University of TechnologyDalian 116024China School of Electronic and Information Engineering South China University of TechnologyGuangzhou 510641China State Key Laboratory of Integrated Services Networks Xidian UniversityXi’an 710071China Department of Electrical and Computer Engineering Western UniversityLondonON N6A 5B9Canada

The flexibility of unmanned aerial vehicles(UAVs)allows them to be quickly deployed to support ground *** reflecting surface(IRS)can reflect the incident signal and form passive beamforming to enhance the signal in the specific *** by the promising benefits of both technologies,we consider a new scenario in this paper where a UAV uses non-orthogonal multiple access to serve multiple users with *** to their distance to the UAV,the users are divided into the close users and remote *** UAV hovers above the close users due to their higher rate requirement,while the IRS is deployed near the remote users to enhance their received *** aim at minimizing the transmit power of UAV by jointly optimizing the beamforming of UAV and the phase shift of IRS while ensuring the decoding ***,the problem is ***,we decompose it into two sub-problems,including the transmit beamforming optimization and phase shift optimization,which are transformed into second-order cone programming and semidefinite programming,*** propose an iterative algorithm to solve the two sub-problems *** results prove the effectiveness of the proposed scheme in minimizing the transmit power of UAV.

关键词： intelligent reflecting surface nonorthogonal multiple access phase shift optimization transmit beamforming optimization unmanned aerial vehicle

Experimental and Numerical Evaluation of the Cavitation Performances of Self-Excited Oscillating Jets

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Fluid Dynamics & Materials Processing 2024年第8期20卷 1883-1901页

作者： Yuanyuan zhao Fujian zhao Guohui Li Wei Xu Xiuli Wang School of the Environment and Safety Engineering Jiangsu UniversityZhenjiang212013China Research Center of Fluid Machinery Engineering and Technology Jiangsu UniversityZhenjiang212013China GongQing Institute of Science and Technology Jiujiang332020China School of Energy and Power Engineering Jiangsu UniversityZhenjiang212013China

Self-excited oscillating jets(SOJ)are used in several practical *** performances are significantly affected by structural parameters and the target *** this study,a geometric model of the SOJ nozzle accounting for multiple structural parameters is introduced,then the related cavitation performances and the optimal target distance are investigated using a Large-Eddy Simulation(LES)*** are also provided about an experiment,which was conducted to validate the simulation *** analyzing the evolution of the vapor volume fraction at the nozzle outlet,a discussion is presented about the effect of the aforementioned structural parameters on the cavitation performances and the target *** is shown that the distribution of cavitation clouds at the outlet of the SOJ nozzle displays a non-monotonic trend(first increasing,then decreasing).Under working conditions with an inlet pressure of 4 MPa,a SOJ nozzle outlet/inlet diameter ratio(D_(1)/D_(2))of 1.2,and a chamber diameter ratio(D/L)close to 1.8,the nozzle outlet cavitation performance attains a *** optimal structural parameters correspond to the optimal target distance,which is near 50 *** experiments have revealed that the SOJ nozzle with the above parameters displays a good cavitation erosion effect at the target distance of 50 mm,in satisfactory agreement with the numerical simulation results.

关键词： Self-excited oscillating nozzle cavitating jet target distant LES cavitation

Microstructure evolution and mechanical properties of brazing joint for ultra-thin-walled Inconel 718 considering grain size effect and brazing temperature

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Chinese Journal of Aeronautics 2024年第2期37卷 541-556页

作者： Rui zhao Yueshuai SONG Hui KANG Min WAN School of Mechanical Engineering and Automation Beihang UniversityBeijing 100191China Jiangxi Research Institute of Beihang University Jiangxi 330000China

The systematic investigation of the mechanical properties and microstructure evolution process of ultra-thin-walled Inconel 718 capillary brazing joints is of great significance because of the exceptionally high demands on its *** achieve this objective,this study investigates the impact of three distinct brazing temperatures and five typical grain sizes on the brazed joints’mechanical properties and microstructure evolution *** evolution analysis was conducted based on Electron Back Scatter Diffraction(EBSD),Scanning Electron Microscopy(SEM),X-Ray Diffraction(XRD),High-Resolution Transmission Electron Microscopy(HRTEM),and Focused Ion Beam(FIB).Besides,the mechanical properties and fracture behavior were studied based on the uniaxial tension tests and in-situ tension *** findings reveal that the brazing joint’s strength is higher for the fine-grain capillary than the coarse-grain one,primarily due to the formation of a dense branch structure composed of G-phase in the brazing *** effects of grain size,such as pinning and splitting,are amplified at higher brazing ***,micro-cracks initiate around brittle intermetallic compounds and propagate through the eutectic zone,leading to a cleavage fracture *** fracture stress of fine-grain specimens is higher than that of coarse-grain due to the complex micro-crack ***,this study contributes significantly to the literature by highlighting the crucial impact of grain size on the brazing properties of ultra-thin-walled Inconel 718 structures.

关键词： EYWORDS Ultra-thin-walled structure Brazing Inconel 718 Grain size effect Brazing temperature

Land use and cover change and influencing factor analysis in the Shiyang River Basin,China

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Journal of Arid Land 2024年第2期16卷 246-265页

作者： zhao Yaxuan CAO Bo SHA Linwei CHENG Jinquan zhao Xuanru GUAN Weijin PAN Baotian Key Laboratory of Western China's Environmental Systems(Ministry of Education) College of Earth and Environmental SciencesLanzhou UniversityLanzhou 730030China Shiyang River Basin Scientific Observing Station Lanzhou UniversityLanzhou 730030China

Land use and cover change(LUCC)is the most direct manifestation of the interaction between anthropological activities and the natural environment on Earth's surface,with significant impacts on the environment and social *** economic development and climate change have resulted in significant changes in land use and *** Shiyang River Basin,located in the eastern part of the Hexi Corridor in China,has undergone significant climate change and LUCC over the past few *** this study,we used the random forest classification to obtain the land use and cover datasets of the Shiyang River Basin in 1991,1995,2000,2005,2010,2015,and 2020 based on Landsat *** validated the land use and cover data in 2015 from the random forest classification results(this study),the high-resolution dataset of annual global land cover from 2000 to 2015(AGLC-2000-2015),the global 30 m land cover classification with a fine classification system(GLC_FCS30),and the first Landsat-derived annual China Land Cover Dataset(CLCD)against ground-truth classification results to evaluate the accuracy of the classification results in this ***,we explored and compared the spatiotemporal patterns of LUCC in the upper,middle,and lower reaches of the Shiyang River Basin over the past 30 years,and employed the random forest importance ranking method to analyze the influencing factors of LUCC based on natural(evapotranspiration,precipitation,temperature,and surface soil moisture)and anthropogenic(nighttime light,gross domestic product(GDP),and population)*** results indicated that the random forest classification results for land use and cover in the Shiyang River Basin in 2015 outperformed the AGLC-2000-2015,GLC_FCS30,and CLCD datasets in both overall and partial ***,the classification results in this study exhibited a high level of agreement with the ground truth *** 1991 to 2020,the area of bare land exhibited a decreasing trend,with chan

关键词： land use and cover classification land use and cover change(LUCC) climate change random forest accuracy assessment three-dimensional sampling method Shiyang River Basin