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Rare loss-of-function variants in FLNB cause non-syndromic orofacial clefts

Journal of Genetics and Genomics 2024年第2期51卷 222-229页

作者： Wenbin Huang Shiying Zhang Jiuxiang Lin Yi ding nan Jiang Jieni Zhang Huaxiang zhao Feng Chen Department of Orthodontics Peking University School and Hospital of StomatologyBeijing100081China Key Laboratory of Shaanxi Province for Craniofacial Precision Medicine Research College of StomatologyXi’an Jiaotong UniversityXi’anShaanxi710004China Guangdong Provincial High-level Clinical Key Specialty Guangdong Province Engineering Research Center of Oral Disease Diagnosis and TreatmentDepartment of OrthodonticsStomatological CenterPeking University Shenzhen HospitalShenzhen Peking University-The Hong Kong University of Science and Technology Medical CenterShenzhenGuangdong 518036China Department of Physiology and Pathophysiology School of Basic Medical SciencesXi'an Jiaotong UniversityXi'anShaanxi710049China National Center for Stomatology National Clinical Research Center for Oral DiseasesNational Engineering Laboratory for Digital and Material Technology of StomatologyBeijing Key Laboratory for Digital StomatologyResearch Center of Engineering and Technology for Computerized Dentistry Ministry of HealthNMPA Key Laboratory for Dental MaterialsBeijing100101China Central Laboratory Peking University School and Hospital of StomatologyBeijing100081China

Orofacial clefts (OFCs) are the most common congenital craniofacial disorders, of which the etiology is closely related to rare coding variants. Filamin B (FLNB) is an actin-binding protein implicated in bone formation. FLNB mutations have been identified in several types of syndromic OFCs and previous studies suggest a role of FLNB in the onset of non-syndromic OFCs (NSOFCs). Here, we report two rare heterozygous variants (p.P441T and p.G565R) in FLNB in two unrelated hereditary families with NSOFCs. Bioinformatics analysis suggests that both variants may disrupt the function of FLNB. In mammalian cells, p.P441T and p.G565R variants are less potent to induce cell stretches than wild type FLNB, suggesting that they are loss-of-function mutations. Immunohistochemistry analysis demonstrates that FLNB is abundantly expressed during palatal development. Importantly, Flnb^(−/−) embryos display cleft palates and previously defined skeletal defects. Taken together, our findings reveal that FLNB is required for development of palates in mice and FLNB is a bona fide causal gene for NSOFCs in humans.

关键词： Or ofacial clefts FLNB Loss-of-function mutati on Cleft palate Filamin B Actin flannent Knockout mouse

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Solution-processed rare-earth thulium oxide with high permittivity for low-voltage transistor and inverters applications

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Journal of Rare Earths 2024年第8期42卷 1604-1609,I0007页

作者： Daiming Liu Fei Wang Yongtao Zhang Ya'nan ding Shandong Engineering Laboratory for Preparation and Application of High-performance Carbon-Materials College of Electromechanical EngineeringQingdao University of Science&TechnologyQingdao266061China College of Electronic and Information Engineering Shandong University of Science and TechnologyQingdao266590China

The use of high-permittivity(high-k) thin films as gate dielectrics is essential in the development of lowpower electronics,In this work,rare-earth thulium oxide(Tm_(2)O_(3)) thin films were prepared by a facile solution process and annealed at various temperatures from 400 to 700℃.The evolution of the physical and dielectric properties of Tm_(2)O_(3) thin film with annealing temperature was *** is demonstrated that the Tm_(2)O_(3) thin film annealed at 600℃ exhibits the optimal performance,including a low leakage current of 3×10^(-10) A/cm^(2),a large areal capacitance of 250 nF/cm^(2) at 100 Hz,and a high permittivity value of *** Tm_(2)O_(3) thin film as a gate insulator was integrated into the thin film transistor(TFT) employing In_(2)O_(3)-based semiconducting *** In_(2)O_(3) TFT with 600℃-annealed Tm_(2)O_(3) dielectric exhibits the superior perfo rmance,with a high I_(on)/I_(off) of 1.65×10^(7),a small subthre shold swing(SS) value of 0.2 V/dec,a V_(TH) of+1.8 V,and a mobility of 1.68 cm^(2)/(V·s).Furthermore,an inverter constructed by connecting the TFT with a resistor exhibits full-swing *** work provides a facile and appealing method for preparing the high-k Tm_(2)O_(3) thin films as alternative gate dielectrics with the potential for use in low-power electronics and logic circuit applications.

关键词： Rare earths Tm_(2)O_(3) Dielectric Transistor Inverter

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A viscoelastic metamaterial beam for integrated vibration isolation and energy harvesting

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Applied Mathematics and Mechanics(English Edition) 2024年第7期45卷 1243-1260页

作者： Long zhao Zeqi LU Hu ding Liqun CHEN Shanghai Institute of Applied Mathematics and Mechanics Shanghai Key Laboratory of Mechanics in Energy EngineeringShanghai Frontier Science Center of MechanoinformaticsSchool of Mechanics and Engineering ScienceShanghai UniversityShanghai200072China School of Microelectronics Shanghai UniversityShanghai201800China

Locally resonant metamaterials have low-frequency band gaps and the capability of converging vibratory energy in the band gaps at resonant *** has been demonstrated by several researchers that the dissipatioin of vibratory energy within the band gap can be improved by using viscoelastic *** paper designs an integrated viscoelastic metamaterial for energy harvesting and vibration *** viscoelastic metamaterial is achieved by a viscoelastic beam periodically arrayed with spatial ball-pendulum nonlinear energy *** nonlinear resonator with an energy harvesting function is achieved by placing a free-rolling magnetic ball in a spherical cavity with an additional induction *** dynamic equations of viscoelastic metamaterials under transverse excitation are established,and the energy harvesting and vibration isolation characteristics within the dispersion relation of viscoelastic metamaterials are *** results show that the vibrations of the main body of the viscoelastic metamaterial beam are significantly suppressed in the frequency range of the local resonance band *** the same time,the elastic waves are limited in the nonlinear resonator with an energy harvesting function,which improves the energy ***,an experimental platform of viscoelastic metamaterial vibration is established for validation purposes.

关键词： viscoelastic metamaterial nonlinear vibration vibration isolation energy harvesting

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SsdchA is a novel secretory cellobiohydrolase driving pathogenicity in Sclerotinia sclerotiorum

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The Crop Journal 2024年第2期12卷 493-502页

作者： Yangui Chen Yijuan ding Siqi zhao nan Yang zhaohui Wu Ping Zhang Hongmei Liao Mengquan Dong Yang Yu Huafang Wan Wei Qian Integrative Science Center of Germplasm Creation in Western China(Chongqing)Science City College of Agronomy and BiotechnologySouthwest UniversityChongqing 400715China Academy of Agricultural Sciences Southwest UniversityChongqing 400715China Engineering Research Center of South Upland Agriculture Ministry of EducationChongqing 400715China College of Plant Protection Southwest UniversityChongqing 400715China

The necrotrophic fungus, Sclerotinia sclerotiorum, employs an array of cell wall-degrading enzymes(CWDEs), including cellulase, to dismantle host cell walls. However, the molecular mechanisms through which S. sclerotiorum degrades cellulose remain elusive. Here, we unveil a novel secretory cellobiohydrolase, SsdchA, characterized by a signal peptide and a Glyco_hydro_7(GH7) domain. SsdchA exhibits a robust expression of during early infection stages. Interestingly, colony morphology and growth rates remain unaffected across the wild-type, SsdchA deletion strains and SsdchA overexpression strains on potato dextrose agar(PDA) medium. Nevertheless, the pathogenicity and cellobiohydrolase activity decreased in the SsdchA deletion strains, but enhanced in the SsdchA overexpression strains. Moreover,the heterologous expression of SsdchA in Arabidopsis thaliana leads to reduced cellulose content and heightened susceptibility to S. sclerotiorum. Collectively, our data underscore the pivotal role of the novel cellobiohydrolase SsdchA in the pathogenicity of S. sclerotiorum.

关键词： Cellobiohydrolase Cellulose Pathogenicity Sclerotinia sclerotiorum Secretory protein SsdchA

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Li-Rich Organosulfur Cathode with Boosted Kinetics for High-Energy Lithium-Sulfur Batteries

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Energy & Environmental Materials 2024年第4期7卷 118-125页

作者： Ting Ma Jiaojiao Deng Yuxiao Lin Qinghua Liang Liang Hu Xiaohu Wang Jun Liu Xinsheng zhao Yinwei Li ding nan Xiaoliang Yu College of Chemistry and Chemical Engineering Inner Mongolia University Inner Mongolia Key Laboratory of Graphite and Graphene for Energy Storage and Coating School of Materials Science and Engineering Inner Mongolia University of Technology Shenzhen Key Laboratory on Power Battery Safety and Shenzhen Geim Graphene Center Tsinghua Shenzhen International Graduate School (SIGS) School of Physics and Electronic Engineering Jiangsu Normal University Key Laboratory of Rare Earth Ganjiang Innovation Academy Chinese Academy of Sciences Department of Mechanical Engineering Research Institute for Smart EnergyThe Hong Kong Polytechnic University

Organosulfur materials containing sulfur-sulfur bonds are an emerging class of high-capacity cathodes for lithium ***,it remains a great challenge to achieve rapid conversion reaction kinetics at practical testing conditions of high cathode mass loading and low electrolyte *** this study,a Li-rich pyrolyzed polyacrylonitrile/selenium disulfide（pPAN/Se2S3） composite cathode is synthesized by deep lithiation to address the above challenges.

关键词： Electrolytes

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Numerical analysis of geosynthetic-reinforced embankment performance under moving loads

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Journal of Rock Mechanics and Geotechnical Engineering 2024年第2期16卷 682-696页

作者： Xuanming ding Jinqiao zhao Qiang Ou Jianfei Liu College of Civil Engineering Chongqing UniversityChongqing400045China Key Laboratory of New Technology for Construction of Cities in Mountain Area Chongqing UniversityChongqing400045China China United Engineering Corporation Limited Hangzhou310052China

The performance of geosynthetic-reinforced embankments under traffic moving loads is always a hotspot in the geotechnical engineering field.A three-dimensional(3D)model of a geosynthetic-reinforced embankment without drainage consolidation was established using the finite element software *** this model,the traffic loads were simulated by two moving loads of rectangular pattern,and their amplitude,range,and moving speed were realized by a Fortran *** embankment fill was simulated by an equivalent linear viscoelastic model,which can reflect its *** geogrid was simulated by the truss element,and the geocell was simulated by the membrane *** elements were utilized to weaken the boundary effect caused by the model geometry at the *** of the established numerical model was conducted by comparing the predicted deformations in the cross-section of the geosynthetic-reinforced embankment with those from the existing *** this basis,the dynamic stress and strain distribution in the pavement structure layer of the geosynthetic-reinforced embankment under a moving load was also ***,a parametric study was conducted to examine the influences of the different types of reinforcement,overload,and the moving load velocity on the geosynthetic-reinforced embankment.

关键词： Geosynthetic-reinforced layer Numerical model Moving load Embankment Deformation Stress

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Ca_(2)MnO_(4)-layered perovskite modified by nanO_(3)for chemical-looping oxidative dehydrogenation of ethane to ethylene

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Chinese Journal of Chemical Engineering 2024年第4期68卷 53-64页

作者： Weixiao ding Kun zhao Shican Jiang Zhen Huang Fang He College of Chemistry and Bioengineering Guilin University of TechnologyGuilin 541006China Guangzhou Institute of Energy Conversion Chinese Academy of SciencesGuangzhou 510640China School of Chemistry and Chemical Engineering Nanjing University of Science and TechnologyNanjing 210094China

Chemical-looping oxidative dehydrogenation(CL-ODH)is a process designed for the conversion of alkanes into olefins through cyclic redox reactions,eliminating the need for gaseous O_(2).In this work,we investigated the use of Ca_(2)MnO_(4)-layered perovskites modified with nanO_(3) dopants,serving as redox catalysts(also known as oxygen carriers),for the CL-ODH of ethane within a temperature range of 700-780℃.Our findings revealed that the incorporation of nanO_(3) as a modifier significantly-nhanced the selectivity for-thylene generation from Ca_(2)MnO_(4).At 750℃and a gas hourly space velocity of 1300 h^(-1),we achieved an-thane conversion up to 68.17%,accompanied by a corresponding-thylene yield of 57.39%.X-ray photoelectron spectroscopy analysis unveiled that the doping nanO_(3) onto Ca_(2)MnO_(4) not only played a role in reducing the oxidation state of Mn ions but also increased the lattice oxygen content of the redox ***,formation of nanO_(3) shell on the surface of Ca_(2)MnO_(4) led to a reduction in the concentration of manganese sites and modulated the oxygen-releasing behavior in a step-wise *** modulation contributed significantly to the enhanced selectivity for ethylene of the nanO_(3)-doped Ca_(2)MnO_(4) *** findings provide compelling evidence for the potential of Ca_(2)MnO_(4)-layered perovskites as promising redox catalysts in the context of CL-ODH reactions.

关键词： Chemical-looping oxidative dehydrogenation Ethane Ethylene nanO_(3)-doped Ca_(2)MnO_(4)redox catalyst Layered perovskites

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An effective catalyst carrier SiO_(2):Enhancing catalytic and combustion properties of CuFe_(2)O_(4)on energetic components

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Defence Technology（防务技术） 2024年第2期32卷 383-392页

作者： Li ding Chong Wan Suhang Chen zhao Qin Kangzhen Xu School of Chemical Engineering/Xi'an Key Laboratory of Special Energy Materials Northwest UniversityXi'an710069China Xi’an Modern Chemistry Research Institute Xi'an710065China

To enhance the catalytic activity of copper ferrite(CuFe_(2)O_(4))nanoparticle and promote its application as combustion catalyst,a low-cost silicon dioxide(SiO_(2))carrier was employed to construct a novel CuFe_(2)O_(4)/SiO_(2)binary composites via solvothermal *** phase structure,morphology and catalytic activity of CuFe_(2)O_(4)/SiO_(2)composites were studied firstly,and thermal decomposition,combustion and safety performance of ammonium perchlorate(AP)and 1,3,5-trinitroperhydro-1,3,5-triazine(RDX)with it affecting were then systematically *** results show that CuFe_(2)O_(4)/SiO_(2)composite can remarkably either advance the decomposition peak temperature of AP and RDX,or reduce the apparent activation energy at their main decomposition ***,the flame propagation rate of RDX was promoted by about 2.73 times with SiO_(2)content of 3 wt%,and safety property of energetic component was also improved greatly,in which depressing the electrostatic discharge sensitivity of pure RDX by about 1.89 *** addition,the effective range of SiO_(2)carrier content in the binary catalyst is found to be 3 to 5 wt%.Therefore,SiO_(2)opens a new insight on the design of combustion catalyst carrier and will promote the application of CuFe_(2)O_(4)catalyst in solid propellant.

关键词： Copper ferrite Silicon dioxide Combustion catalyst Thermal decomposition Laser ignition

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Surface integrity evolution during creep feed profile grinding ofγ-TiAl blade tenon

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Chinese Journal of Aeronautics 2024年第8期37卷 496-512页

作者： Tao CHEN Xiaowei WANG Biao zhao Wenfeng ding Jiuhua XU National Key Laboratory of Science and Technology on Helicopter Transmission Nanjing University of Aeronautics and AstronauticsNanjing 210016China

Gamma titanium-aluminum(γ-TiAl)intermetallic compounds are increasingly used in manufacturing key hot-end components(e.g.,blade tenon)in aero engines due to their high specific strength and lightweight *** feed profile grinding(CFPG)as a crucial precision process that is applied to produce the final profile of the blade ***,sudden surface burns and microcracks of machined c-TiAl blade tenon often occur because of its low plasticity and high strength during grinding processes,leading to poor surface *** this work,CFPG experiments based on the profile characteristics ofγ-TiAl blade tenon were performed and an associated undeformed chip thickness model considering grain–workpiece contact condition was established to explore the evolution of the surface ***,the surface integrity was analyzed at different positions of the blade tenon in terms of surface roughness and morphology,metallographic structure,microhardness,and residual *** show that the profile characteristics of blade tenon have a significant influence on machined surface integrity because of the thermomechanical effect at various detecting *** residual stress was established based on the undeformed chip thickness model considering the profile structure,with a prediction error of 10%–15%.The thermomechanical effect is more obvious at the bottom area,where the surface roughness,work hardening degree,and subsurface plastic deformation range are the largest,while the values at the bevel area are the *** on the undeformed chip thickness model,a residual stress finite element simulation was conducted by employing thermomechanical coupled *** addition,the error between the simulation and the experiment was between 10%–15%.Strain and strain rate equations were established through the relationship between material displacement and *** average strain and strain rate of the ground surface when ap is 1.0 mm are 18.8%and 33.2%l

关键词： Gamma titanium-aluminum intermetallic compounds Blade tenon Creep feed profile grinding Surface integrity Thermomechanical effect

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Chord length sampling correction analysis for dispersion fuel in Monte Carlo simulation

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Nuclear Science and Techniques 2024年第4期35卷 57-64页

作者： zhao-Yu Liang ding She Yu-Tong Wen Lei Shi Institute of Nuclear and New Energy Technology(INET) Key Laboratory of Advanced Reactor Engineering and Safety of Ministry of EducationTsinghua UniversityBeijing 100084China

Dispersion fuels,knowned for their excellent safety performance,are widely used in advanced reactors,such as hightemperature gas-cooled *** with deterministic methods,the Monte Carlo method has more advantages in the geometric modeling of stochastic *** explicit modeling method has high computational accuracy and high computational *** chord length sampling(CLS)method can improve computational efficiency by sampling the chord length during neutron transport using the matrix chord length?s probability density *** study shows that the excluded-volume effect in realistic stochastic media can introduce certain deviations into the CLS.A chord length correction approach is proposed to obtain the chord length correction factor by developing the Particle code based on equivalent transmission *** numerical analysis against reference solutions from explicit modeling in the RMC code,it was demonstrated that CLS with the proposed correction method provides good accuracy for addressing the excludedvolume effect in realistic infinite stochastic media.

关键词： Stochastic media Monte Carlo Chord length sampling Excluded-volume effect Chord length correction

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