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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 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

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Comprehensive study on the spatial distribution of heavy metals and their environmental risks in high-sulfur coal gangue dumps in China

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Journal of Environmental Sciences 2024年第2期136卷 486-497页

作者： Yingbo dong Huan Lu Hai lin School of Energy and Environmental Engineering University of Science and Technology BeijingBeijing 100083China Beijing Key Laboratory on Resource-oriented Treatment of Industrial Pollutants Beijing 100083China

The accumulation of coal gangue(CG)from coal mining is an important source of heavy metals(HMs)in soil.Its spatial distribution and environment risk assessment are extremely important for the management and remediation of HMs.Eighty soil samples were collected from the high-sulfur CG site in northern China and analyzed for six HMs.The results showed that the soil was heavily contaminated by Mn,Cr and Ni based on the Nemerow index,and posed seriously ecological risk depended on the geo-accumulation index,potential ecological risk index and risk assessment code.The semi-variogram model and ordinary kriging interpolation accurately portrayed the spatial distribution of HMs.Fe,Mn,and Cr were distributed by band diffusion,Ni was distributed by core,the distribution of Cu had obvious patchiness and Zn was more uniform.The spatial autocorrelation indicated that all HMs had strong spatial heterogeneity.The BCR sequential extraction was employed to qualify the geochemical fractions of HMs.The data indicated that Fe and Cr were dominated by residual fraction;Cu,Ni and Zn were dominated by reducible and oxidizable fractions;Mn was dominated by reducible and acid-extractable(25.38%-44.67%)fractions.Pearson correlation analysis showed that pH was the main control factor affecting the non-residue fractions of HMs.Therefore,acid production from high sulfur CG reduced soil pH by 2-3,which indirectly promoted the activity of HMs.Finally,the conceptual model of HMs contamination at the CG site was proposed,which can be useful for the development of ecological remediation strategies.

关键词： High-sulfur coal gangue Soil contamination Heavy metals Spatial distribution Geochemical fractions Risk assessment

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Stepwise colloidal lithography toward scalable and various planar chiral metamaterials

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Rare Metals 2024年第2期43卷 723-735页

作者： Xiu Yang Yong Liu Fei-Liang Chen Qian-Qi lin Rohit Chikkaraddy Shan-Shan Huang Shi-lin Xian Yi-dong Hou Jing-Lei du Liang-Ping Xia Chun-Lei du School of Electronic Science and Engineering University of Electronic Science and Technology of ChinaChengdu610054China School of Optoelectronic Science and Engineering University of Electronic Science and Technology of ChinaChengdu610054China Zhuhai Multiscale Optoelectronics Technology Co. Ltd.Zhuhai915060China Hybrid Materials for Opto-Electronics Group Department of Molecules and MaterialsMESA+Institute for NanotechnologyMolecules Center and Center for Brain-Inspired Nano SystemsFaculty of Science and TechnologyUniversity of Twente7500 AEEnschedeThe Netherlands School of Physics and Astronomy University of BirminghamBirminghamB152TTUK College of Physics Sichuan UniversityChengdu610065China Key Laboratory of Micro Nano Optoelectronic Devices and Intelligent Perception Systems Yangtze Normal UniversityChongqing408100China

Chiral metamaterials(CMs)composed by artificial chiral resonators have attracted great attentions in the recent decades due to their strong chiroptical resonance and identifiable interaction with chiral materials,facilitating practical applications in chiral biosensing,chiral emission,and display technology.However,the complex geometry of CMs improves the fabrication difficulty and hinders their scalable fabrication for practical applications,especially in the visible and ultraviolet wavelengths.One potential strategy is the colloidal lithography that enables parallel fabrication for scalable and various planar structures.Here,we demonstrate a stepwise colloidal lithography technique that uses sequential deposition from multiple CMs and expand their variety and complexity.The geometry and optical chirality of building blocks from single deposition are systematically investigated,and their combination enables a significant extension of the range of chiral patterns by multiple-step depositions.This approach resulted in a myriad of complex designs with different characteristic sizes,compositions,and shapes,which are particularly beneficial for the development of nanophotonic materials.In addition,we designed a flexible chiral device based on PDMS,which exhibits a good CD value and excellent stability even after multiple inward and outward bendings.The excellent compatibility to various substrates makes the planar CMs more flexible in practical applications in microfluidic biosensing.

关键词： Chiral metamaterials Colloidal lithography Nanophotonics Metasurfaces Plasmonics Nanofabrications Flexible devices

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Distributed collaborative complete coverage path planning based on hybrid strategy

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Journal of Systems Engineering and Electronics 2024年第2期35卷 463-472页

作者： ZHANG Jia du Xin dong Qichen XIN Bin School of Automation Beijing Institute of TechnologyBeijing 100081China National Key Laboratory of Autonomous Intelligent Unmanned Systems Beijing Institute of TechnologyBeijing 100081China

Collaborative coverage path planning(CCPP) refers to obtaining the shortest paths passing over all places except obstacles in a certain area or space. A multi-unmanned aerial vehicle(UAV) collaborative CCPP algorithm is proposed for the urban rescue search or military search in outdoor environment.due to flexible control of small UAVs, it can be considered that all UAVs fly at the same altitude, that is, they perform search tasks on a two-dimensional plane. Based on the agents’ motion characteristics and environmental information, a mathematical model of CCPP problem is established. The minimum time for UAVs to complete the CCPP is the objective function, and complete coverage constraint, no-fly constraint, collision avoidance constraint, and communication constraint are considered. Four motion strategies and two communication strategies are designed. Then a distributed CCPP algorithm is designed based on hybrid strategies. Simulation results compared with patternbased genetic algorithm(PBGA) and random search method show that the proposed method has stronger real-time performance and better scalability and can complete the complete CCPP task more efficiently and stably.

关键词： multi-agent cooperation unmanned aerial vehicles(UAV) distributed algorithm complete coverage path planning(CCPP)

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Advancing neuroscience through real-time processing of big data:Transition from open-loop to closed-loop paradigms

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Zoological Research 2024年第3期45卷 518-519页

作者： Yu-Fan Wang Jiu-lin du Institute of Neuroscience State Key Laboratory of NeuroscienceCenter for Excellence in Brain Science and Intelligence TechnologyChinese Academy of SciencesShanghai 200031China University of Chinese Academy of Sciences Beijing 100049China School of Life Science and Technology ShanghaiTech UniversityShanghai 200031China

The brain functions as a closed-loop system that continuously generates behavior in response to the external environment and adjusts actions based on the outcomes.Traditional research methodologies in neuroscience,especially those employed in brain imaging experiments,have mainly adopted an open-loop paradigm(Grosenick et al.,2015).Functional neural circuits are analyzed offline and subsequently tested through manipulation of neuronal activities within specific regions or with genetic markers.By establishing a closed-loop research paradigm,functional ensembles can be detected and tested in real time with temporal sequences.These functional ensembles,rather than brain regions or genetically labeled neural populations,serve as fundamental units of neural networks,offering valuable insights into the dissection of neural circuits.The closed-loop research paradigm also enables the capture of high-dimensional activities of internal brain dynamics and precise elucidation of physiological processes such as learning,decision-making,and sleep.

关键词： neural loop Transition

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Na^(+)/K^(+)-ATPase:ion pump,signal transducer,or cytoprotective protein,and novel biological functions

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Neural Regeneration Research 2024年第12期19卷 2684-2697页

作者： Songqiang Huang Wanting dong Xiaoqian lin Jinsong Bian Department of Pharmacology Joint Laboratory of Guangdong-Hong Kong Universities for Vascular Homeostasis and DiseasesSchool of MedicineSouthern University of Science and TechnologyShenzhenGuangdong ProvinceChina Department of Pharmacology School of Basic MedicineTongji Medical CollegeHuazhong University of Science and TechnologyWuhanHubei ProvinceChina

Na^(+)/K^(+)-ATPase is a transmembrane protein that has important roles in the maintenance of electrochemical gradients across cell membranes by transporting three Na^(+)out of and two K^(+)into cells.Additionally,Na^(+)/K^(+)-ATPase participates in Ca^(2+)-signaling transduction and neurotransmitter release by coordinating the ion concentration gradient across the cell membrane.Na^(+)/K^(+)-ATPase works synergistically with multiple ion channels in the cell membrane to form a dynamic network of ion homeostatic regulation and affects cellular communication by regulating chemical signals and the ion balance among different types of cells.Therefo re,it is not surprising that Na^(+)/K^(+)-ATPase dysfunction has emerged as a risk factor for a variety of neurological diseases.However,published studies have so far only elucidated the important roles of Na^(+)/K^(+)-ATPase dysfunction in disease development,and we are lacking detailed mechanisms to clarify how Na^(+)/K^(+)-ATPase affects cell function.Our recent studies revealed that membrane loss of Na^(+)/K^(+)-ATPase is a key mechanism in many neurological disorders,particularly stroke and Parkinson's disease.Stabilization of plasma membrane Na^(+)/K^(+)-ATPase with an antibody is a novel strategy to treat these diseases.For this reason,Na^(+)/K^(+)-ATPase acts not only as a simple ion pump but also as a sensor/regulator or cytoprotective protein,participating in signal transduction such as neuronal autophagy and apoptosis,and glial cell migration.Thus,the present review attempts to summarize the novel biological functions of Na^(+)/K^(+)-ATPase and Na^(+)/K^(+)-ATPase-related pathogenesis.The potential for novel strategies to treat Na^(+)/K^(+)-ATPase-related brain diseases will also be discussed.

关键词： antibody biological functions cellular communication electrochemical gradient ion balance ion channels Na^(+)/K^(+)-ATPase neurological diseases neurotransmitter release signal transduction

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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

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Tetrabromobisphenol A exerts thyroid disrupting effects but has little overt impact on postnatal brain development and neurobehaviors in mice

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Journal of Environmental Sciences 2024年第8期142卷 1-10页

作者： Shilin Song Yuanyuan Li lin Lv Mengqi dong Zhanfen Qin State Key Laboratory of Environmental Chemistry and Ecotoxicology Research Center for Eco-Environmental SciencesChinese Academy of SciencesBeijing 100085China College of Resources and Environment University of Chinese Academy of SciencesBeijing 100049China School of Environment Tsinghua UniversityBeijing 100084China

Tetrabromobisphenol A(TBBPA)is a widely used brominated flame retardant.There is evidence showing that TBBPA can exert thyroid disrupting effects in mammals,but different results were also reported,along with inconsistent reports regarding its neurotoxicity.Here,we investigated thyroid disrupting effects and neurotoxicity of TBBPA(5,50,500μg/(kg·day))to male mice following maternal and direct exposure through drinking water,with the antithyroid drug propylthiouracil(PTU)as the positive control.On postnatal day(PND)15,we expectedly observed severe thyroid compensatory hyperplasia and cerebellar developmental retardation in PTU-treated pups.The highest dose of TBBPA also caused thyroid histological alteration but had no effects on cerebellar development in terms of Purkinje cell morphology and the thickness of the internal granular layer and the molecular layer of the cerebellum.during puberty and adulthood,the thyroid morphological alterations became more pronounced in the TBBPA-treated animals,accompanied by decreased serum thyroid hormone levels.Furthermore,the 50 and 500μg/(kg·day)TBBPA groups showed a significant decrease in the serum level of serotonin,a neurotransmitter associated with anxiety behaviors.Correspondingly,the highest dose group displayed anxiety-like behaviors in the elevated plus-maze test on PND 35,but this neurobehavioral alteration disappeared on PND 56.Moreover,no changes in neurobehavioral parameters tested were found in TBBPAtreated animals at puberty and adulthood.Altogether,all observations show that TBBPA can exert thyroid disrupting effects but has little overt impact on brain development and neurobehaviors in mice,suggesting that thyroid disruption does not necessarily cause overtly adverse neurodevelopmental outcomes.

关键词： Tetrabromobisphenol A Thyroid disruption Brain development Neurobehavior Cerebellum

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A Galaxy Image Augmentation Method Based on Few-shot Learning and Generative Adversarial Networks

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Research in Astronomy and Astrophysics 2024年第3期24卷 180-193页

作者： Yiqi Yao Jinqu Zhang Ping du Shuyu dong School of Artificial Intelligence South China Normal UniversityFoshan 528225China School of Computer Science South China Normal UniversityGuangzhou 510631China Guangdong Construction Vocational Technology Institute Guangzhou 511500China

Galaxy morphology classifications based on machine learning are a typical technique to handle enormous amounts of astronomical observation data,but the key challenge is how to provide enough training data for the machine learning models.Therefore this article proposes an image data augmentation method that combines few-shot learning and generative adversarial networks.The Galaxy10 DECaLs data set is selected for the experiments with consistency,variance,and augmentation effects being evaluated.Three popular networks,including AlexNet,VGG,and ResNet,are used as examples to study the effectiveness of different augmentation methods on galaxy morphology classifications.Experiment results show that the proposed method can generate galaxy images and can be used for expanding the classification model’s training set.According to comparative studies,the best enhancement effect on model performance is obtained by generating a data set that is 0.5–1 time larger than the original data set.Meanwhile,different augmentation strategies have considerably varied effects on different types of galaxies.FSL-GAN achieved the best classification performance on the ResNet network for In-between Round Smooth Galaxies and Unbarred Loose Spiral Galaxies,with F1 Scores of 89.54%and 63.18%,respectively.Experimental comparison reveals that various data augmentation techniques have varied effects on different categories of galaxy morphology and machine learning models.Finally,the best augmentation strategies for each galaxy category are suggested.

关键词： techniques image processing-galaxies structure-galaxies general

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Numerical study on the effective thermal conductivity and thermal tortuosity of porous media with different morphologies

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Science China Technological Sciences 2024年第6期67卷 1685-1694页

作者： du Shen LI dong LI Meng-Jie HE Ya-ling Key Laboratory of Thermo-Fluid Science and Engineering of Ministry of Education School of Energy and Power Engineering Xi’an Jiaotong University

Effective thermal conductivity and thermal tortuosity are crucial parameters for evaluating the effectiveness of heat conduction within porous media. The direct pore-scale numerical simulation method is applied to investigate the heat conduction processes inside porous structures with different morphologies. The thermal conduction performances of idealized porous structures are directly compared with real foams across a wide range of porosity. Real foam structures are reconstructed using X-ray computed tomography and image processing techniques, while Kelvin and Weaire-Phelan structures are generated through periodic unit cell reconstruction. The detailed temperature fields inside the porous structures are determined by solving the heat conduction equation at the pore scale. The results present that the equivalent thermal conductivity of Kelvin and Weaire-Phelan structures is similar to and greater than that of the real foam structure with the same strut porosity. The thermal tortuosity of real foam structure is relatively larger and the heat conduction path becomes straighter by adopting the anisotropic design. The thermal tortuosity of the fluid channels for Kelvin, Weaire-Phelan, and real foam structures is close to one. The thermal conductivity of porous structures with heat transfer fluid increases as the thermal conductivity ratio of fluid to solid becomes larger. A small porosity of porous media leads to a larger equivalent thermal conductivity due to the dominant contribution of porous skeleton in the heat conduction process. Correlations derived from parallel and series models, as well as the Maxwell-Eucken models,provide decent predictions of effective thermal conductivity, with an average error of less than 8% in the entire range of thermal conductivity ratio.

关键词： volumetric solar receiver porous morphology direct pore-scale simulation equivalent thermal conductivity thermal tortuosity empirical correlation

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