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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 worldwide.As such,our current knowledge of this new disease remains preliminary.This review aims to provide a brief overview of the clinical manifestations,pathogenesis,and treatment strategies for this novel IEI.Data 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 deficiency.Our 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 submission.Results The current signature manifestations of ELF4 deficiency disorder are recurrent and prolonged oral ulcer,abdominal pain,and diarrhea in pediatric males.In some cases,immunodeficiency and autoimmunity can also be prominent.Targeted Sanger sequencing or whole exome sequencing can be used to detect variation in ELF4 gene.Western blotting for ELF4 expression of the patient’s cells can confirm the pathogenic effect of the variant.To fully confirm the pathogenicity of the variant,further functional test is strongly advised.Glucocorticoid and biologics are the mainstream management of ELF4 deficiency disorder.Conclusions Pediatric males presenting with recurring ulcerations in digestive tract epithelium with or without recurrent fever should be suspected of DEX.When atypical presentations are prominent,variations in ELF4 gene should be carefully evaluated functionally due to the complex nature of ELF4 function.Experience 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

Recent Advances in Pd-Catalyzed Reactions Involving the “On-Water” Mechanism

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Chinese Journal of Chemistry 2024年第5期42卷 533-542页

作者： dong Wei Guo-qiang Lin State Key Laboratory of Systems Medicine for Cancer Shanghai Cancer InstituteShanghai Key Laboratory of Systems Regulation and Clinical Translation for CancerRen Ji HospitalShanghai Jiao Tong University School of MedicineShanghai200127 China State Key Laboratory of Chemical Biology Shanghai Institute of Organic ChemistryUniversity of Chinese Academy of SciencesChinese Academy of SciencesShanghai200032 China

As the chemical industry grapples with the need for more eco-friendly practices,the use of water as a reaction medium is gaining attraction in organic transformations.This mini-review delves into Pd-catalyzed reactions that utilize the "on-water" mechanism,spanning from 2019 to late 2023.These reactions are neatly categorized into several types: (A) Catalytic C—H activations,(B) Mizoroki- Heck-type reactions,(C) Suzuki-Miyaura reactions,and (D) Cyclization reactions.By showcasing the potential of water as a sustainable reaction medium for organic transformations,these reactions leave no doubt about the importance of embracing eco-friendly practices in the chemical industry.Key Scientists In 1980,a seminal work by Breslow et al.showed an acceleration of reaction rate in the Diels-Alder reaction.Sharpless and co-workers noted a significant increase in the rate of the [2σ+2σ+2π] cycloaddition of quadricyclane and dimethyl azodicarboxylate (DMAD) when the reaction was conducted in water,as opposed to when it was carried out in organic solvents.The term "on-water" was then coined to describe this phenomenon.This strategy was further expanded to transition-metal catalyzed transformations by Ackermann in 2011.Later,Varma and Leazer disclosed a Pd-catalyzed Mizoroki-Heck type arylation of alkenes with diaryliodonium salts “on-water”.The enantioselective version of "on-water" process was not realized until 2014 by the Zhou group.Later on,the Schaub group described a Pd-catalyzed Suzuki–Miyaura coupling reaction of electron-poor aryl chlorides with water,using only 50 ppm of catalyst loading.Very recently,Liu and Lin extended the "on-water" strategy to Pd-catalyzed double Mizoroki-Heck reactions.This mini-review has focused on Pd-catalyzed reactions involving the “on-water” mechanism.

关键词： Pd-catalyzed “On-water” Green chemistry Aqueous reactions Mechanism Methodology and reactions Mizoroki-Heck reaction Cross-coupling

Brain Cognition Mechanism-Inspired Hierarchical Navigation Method for Mobile Robots

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Journal of Bionic Engineering 2024年第2期21卷 852-865页

作者： qiang Zou Chengdong Wu Ming Cong dong Liu Faculty of Robot Science and Engineering Northeastern UniversityShenyang110819China School of Mechanical Engineering Dalian University of TechnologyDalian116024China

Autonomous navigation is a fundamental problem in robotics.Traditional methods generally build point cloud map or dense feature map in perceptual space;due to lack of cognition and memory formation mechanism,traditional methods exist poor robustness and low cognitive ability.As a new navigation technology that draws inspiration from mammal’s navigation,bionic navigation method can map perceptual information into cognitive space,and have strong autonomy and environment adaptability.To improve the robot’s autonomous navigation ability,this paper proposes a cognitive map-based hierarchical navigation method.First,the mammals’navigation-related grid cells and head direction cells are modeled to provide the robots with location cognition.And then a global path planning strategy based on cognitive map is proposed,which can anticipate one preferred global path to the target with high efficiency and short distance.Moreover,a hierarchical motion controlling method is proposed,with which the target navigation can be divided into several sub-target navigation,and the mobile robot can reach to these sub-targets with high confidence level.Finally,some experiments are implemented,the results show that the proposed path planning method can avoid passing through obstacles and obtain one preferred global path to the target with high efficiency,and the time cost does not increase extremely with the increase of experience nodes number.The motion controlling results show that the mobile robot can arrive at the target successfully only depending on its self-motion information,which is an effective attempt and reflects strong bionic properties.

关键词： Bionic navigation Spatial localization cells Global path planning Hierarchical motion controlling

Ecological effect of the plantation of Sabina vulgaris in the Mu Us Sandy Land,China

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Journal of Arid Land 2024年第1期16卷 14-28页

作者： NAN Weige dong Zhibao ZHOU Zhengchao LI qiang CHEN Guoxiang School of Geography and Tourism Shaanxi Normal UniversityXi'an 710062China Shaanxi Key Laboratory of Ecological Restoration in Shaanbei Mining Area Yulin UniversityYulin 719000China College of Prataculture Gansu Agricultural UniversityLanzhou 730070China

Vegetation restoration through artificial plantation is an effective method to combat desertification,especially in arid and semi-arid areas.This study aimed to explore the ecological effect of the plantation of Sabina vulgaris on soil physical and chemical properties on the southeastern fringe of the Mu Us Sandy Land,China.We collected soil samples from five depth layers(0-20,20-40,40-60,60-80,and 80-100 cm)in the S.vulgaris plantation plots across four plantation ages(4,7,10,and 16 years)in November 2019,and assessed soil physical(soil bulk density,soil porosity,and soil particle size)and chemical(soil organic carbon(SOC),total nitrogen(TN),available nitrogen(AN),available phosphorus(AP),available potassium(AK),cation-exchange capacity(CEC),salinity,p H,and C/N ratio)properties.The results indicated that the soil predominantly consisted of sand particles(94.27%-99.67%),with the remainder being silt and clay.As plantation age increased,silt and very fine sand contents progressively rose.After 16 years of planting,there was a marked reduction in the mean soil particle size.The initial soil fertility was low and declined from 4 to 10 years of planting before witnessing an improvement.Significant positive correlations were observed for the clay,silt,and very fine sand(mean diameter of 0.000-0.100 mm)with SOC,AK,and p H.In contrast,fine sand and medium sand(mean diameter of 0.100-0.500 mm)showed significant negative correlations with these indicators.Our findings ascertain that the plantation of S.vulgaris requires 10 years to effectively act as a windbreak and contribute to sand fixation,and needs 16 years to improve soil physical and chemical properties.Importantly,these improvements were found to be highly beneficial for vegetation restoration in arid and semi-arid areas.This research can offer valuable insights for the protection and restoration of the vegetation ecosystem in the sandy lands in China.

关键词： Sabina vulgaris plantation age soil physical and chemical properties soil particle size soil fertility vegetation restoration Mu Us Sandy Land

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Pathogenesis of chronic enteropathy associated with the SLCO2A1 gene:Hypotheses and conundrums

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World Journal of Gastroenterology 2024年第19期30卷 2505-2511页

作者： Zhi-Xin Xie Yue Li Ai-Ming Yang dong Wu qiang Wang State Key Laboratory of Complex Severe and Rare Diseases Department of GastroenterologyPeking Union Medical College HospitalChinese Academy of Medical SciencesBeijing 100730China Department of Clinical Medicine Peking Union Medical CollegeChinese Academy of Medical SciencesBeijing 100730China

Chronic enteropathy associated with the SLCO2A1 gene(CEAS)is a complex gastroenterological condition characterized by multiple ulcers in the small intestine with chronic bleeding and protein loss.This review explores the potential mechanisms underlying the pathogenesis of CEAS,focusing on the role of SLCO2A1-encoded prostaglandin transporter OATP2A1 and its impact on prostaglandin E2(PGE2)levels.Studies have suggested that elevated PGE2 levels contribute to mucosal damage,inflammation,and disruption of the intestinal barrier.The effects of PGE2 on macrophage activation and Maxi-Cl channel functionality,as well as its interaction with nonsteroidal anti-inflammatory drugs play crucial roles in the progression of CEAS.Understanding the balance between its protective and pro-inflammatory effects and the complex interactions within the gastrointestinal tract can shed light on potential therapeutic targets for CEAS and guide the development of novel,targeted therapies.

关键词： SLCO2A1 Prostaglandin E2 Chronic enteropathy associated with the SLCO2A1 gene Small intestine Macrophage

Analysis of pressure response at an observation well against pressure build-up by early stage of CO_(2)geological storage project

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Journal of Rock Mechanics and Geotechnical Engineering 2024年第2期16卷 470-482页

作者： qiang Sun Kyuro Sasaki Qinxi dong Zhenni Ye Hui Wang Huan Sun School of Civil Engineering and Architecture Hainan UniversityHaikou570228China Faculty of Engineering Kyushu University744 MotookaNishi-kuFukuoka819-0385Japan Key Laboratory of Equipment Safety and Intelligent Technology for Guangzhou Rail Transit System Guangzhou510430China

To ensure a safe and stable CO_(2)storage,pressure responses at an observation well are expected to be an important and useful field monitoring item to estimate the CO_(2)storage behaviors and the aquifer parameters during and after injecting CO_(2),because it can detect whether the injected CO_(2)leaks to the ground surface or the bottom of the sea.In this study,pressure responses were simulated to present design factors such as well location and pressure transmitter of the observation well.Numerical simulations on the pressure response and the time-delay from pressure build-up after CO_(2)injection were conducted by considering aquifer parameters and distance from the CO_(2)injection well to an observation well.The measurement resolution of a pressure transmitter installed in the observation well was presented based on numerical simulation results of the pressure response against pressure build-up at the injection well and CO_(2)plume front propagations.Furthermore,the pressure response at an observation well was estimated by comparing the numerical simulation results with the curve of CO_(2)saturation and relative permeability.It was also suggested that the analytical solution can be used for the analysis of the pressure response tendency using pressure build-up and dimensionless parameters of hydraulic diffusivity.Thus,a criterion was established for selecting a pressure transducer installed at an observation well to monitor the pressure responses with sufficient accuracy and resolution,considering the distance from the injection well and the pressure build-up at the injection well,for future carbon capture and storage(CCS)projects.

关键词： CO_(2)storage Saline aquifer Observation well Pressure response CO_(2)saturation

Irreducible Representations of GL_(n)(C)of Minimal Gelfand-Kirillov Dimension

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Acta Mathematica Sinica,English Series 2024年第3期40卷 639-657页

作者： Zhan qiang BAI Yang Yang CHEN dong Wen LIU Bin Yong SUN School of Mathematical Sciences Soochow UniversitySuzhou 215006P.R.China School of Sciences JiangnanUniversityWui214122P.R.China School of Mathematical Sciences Zhejiang UniversityHangzhou 310058P.R.China Institute for Advanced Study in Mathematics Zhejiang UniversityHangzhou 310058P.R.China

In this article,by studying the Bernstein degrees and Goldie rank polynomials,we es-tablish a comparison between the irreducible representations of G=GL_(n)(C)possessing the minimal Gelfand-Kirillov dimension and those induced from finite-dimensional representations of the maximal parabolic subgroup of G of type(n-1,1).We give the transition matrix between the two bases for the corresponding coherent families.

关键词： Bernstein degree coherent continuation Gelfand-Kirillov dimension

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The mechanisms of thermal solidification agent promoting steam diversion in heavy oil reservoirs

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Petroleum Science 2024年第3期21卷 1902-1914页

作者： Zhan-Xi Pang Qian-Hui Wang qiang Meng Bo Wang dong Liu State Key Laboratory of Petroleum Resources and Engineering China University of Petroleum (Beijing) Liaohe Oilfield Institute CNPC China Henan Oilfield Institute Sinopec China Tianjin Branch of China National Offshore Oil Company (CNOOC) Ltd.

At high cycles of steam huff & puff, oil distribution in reservoirs becomes stronger heterogeneity due to steam channeling. Thermal solidification agent can be used to solve this problem. Its solution is a lowviscosity liquid at normal temperature, but it can be solidified above 80°C. The plugging degree is up to 99% at 250°C. The sweep efficiency reaches 59.2%, which is 7.3% higher than pure steam injection. In addition, simultaneous injection of viscosity reducer and/or nitrogen foams can further enhance oil recovery. The mechanism of this technology depends on its strong plugging ability, which changes the flowing pattern of steam to effectively mobilize remaining oil. Viscosity reducer and nitrogen foams further expand the sweep range and extends the effective period. Therefore, thermal solidification agent can plug steam channeling paths and adjust steam flowing direction to significantly enhance oil recovery at high cycles of steam huff & puff.

关键词： Heavy oil reservoir Steam huff & puff Steam channeling Steam diversion Plugging performance Mechanism analysis

Harmful evaluation of heavy metals from soil layer to the groundwater: Take the Jilin Hunchun Basin as an example

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China Geology 2024年第1期7卷 116-124页

作者： Xiao-dong Guo qiang Liu Hui-Rong Zhang Xu-Fei Shi Chuan-Yu Qin Zhi-qiang Zhang Shenyang Geological Survey Center China Geological SurveyMinistry of Natural ResourcesShenyang 110034China Key Laboratory of Black Land Evolution and Ecological Effects Shenyang 110034China New Energy and Environment College Jilin UniversityChangchun 130026China Jilin Hydrogeological Survey Institute Changchun 130026China

The continuous enrichment of heavy metals in soils has caused potential harm to groundwater.Quantitative methods to evaluate the harm of heavy metals in soil to groundwater are lacked in previous studies.Based on the theory of groundwater circulation and solid-liquid equilibrium,a simple and easy-touse flux model of soil heavy metals migrating to groundwater is constructed.Based on groundwater environmental capacity,an innovative method for evaluating the harm of heavy metals in soil to groundwater is proposed,which has been applied in Hunchun Basin,Jilin Province,China.The results show that the fluxes of soil heavy metals into groundwater in the study area are Zn,Cu,As,Pb,Cd,Ni,and Hg in descending order.The content of heavy metals in groundwater(As,Hg,Cu,Pb,Zn,Ni,and Cd)in most areas has not risen to the threshold of environmental capacity within 10 years.The harm levels of soil heavy metals to groundwater in the most townships soils are at the moderate level or below.This evaluation method can quantify the flux of soil heavy metals into groundwater simply and quickly,determine the residual capacity of groundwater to heavy metals,evaluate the harm level of soil heavy metals to groundwater,provide support for relevant departments to carry out environmental protection of soil and groundwater,and provide a reference to carry out similar studies for related scholars.

关键词： Soil heavy metals As+Hg+Cu+Pb+Zn+Ni+Cd Environmental capacity Groundwater Hazard degree Migration flux model Agricultural geological survey engineering Hunchun Basin Jilin Province

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