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Both gain-and loss-of-function variants of KCNA1 are associated with paroxysmal kinesigenic dyskinesia

Journal of Genetics and Genomics 2024年第8期51卷 801-810页

作者： Wan-Bing Sun jing-xin fu Yu-Lan Chen Hong-fu Li Zhi-Ying Wu Dian-fu Chen Department of Medical Genetics and Center for Rare Diseases and Department of Neurologyand Zhejiang Key Laboratory of Rare Diseases for Precision Medicine and Clinical Translation in Second Affiliated HospitalZhejiang University School of MedicineHangzhouZhejiang 310009China Nanhu Brain-computer Interface Institute HangzhouZhejiang 314050China MOE Frontier Science Center for Brain Science and Brain-machine Integration School of Brain Science and Brain MedicineZhejiang UniversityHangzhouZhejiang 310012China

KCNA1 is the coding gene for Kv1.1 voltage-gated potassium-channelα*** variants of KCNA1 have been reported to manifest as paroxysmal kinesigenic dyskinesia(PKD),but the correlation between them remains unclear due to the phenotypic complexity of KCNA1 variants as well as the rarity of PKD *** the whole exome sequencing followed by Sanger sequencing,we screen for potential pathogenic KCNA1 variants in patients clinically diagnosed with paroxysmal movement disorders and identify three previously unreported missense variants of KCNA1 in three unrelated Chinese *** proband of one family(c.496G>A,p.A166T)manifests as episodic ataxia type 1,and the other two(c.877G>A,p.V293I and c.1112C>A,p.T371A)manifest as *** pathogenicity of these variants is confirmed by functional studies,suggesting that p.A166T and p.T371A cause a loss-of-function of the channel,while p.V293I leads to a gain-of-function with the property of voltage-dependent gating and activation kinetic *** reviewing the locations of PKD-manifested KCNA1 variants in Kv1.1 protein,we find that these variants tend to cluster around the pore domain,which is similar to ***,our study strengthens the correlation between KCNA1 variants and PKD and provides more information on genotype–phenotype correlations of KCNA1 channelopathy.

关键词： Paroxysmal kinesigenic dyskinesia KCNA1 Loss-of-function Gain-of-function Channelopathy Episodicataxiatype1

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Tumor necrosis factor α deficiency promotes myogenesis and muscle regeneration

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Zoological Research 2024年第4期45卷 951-960页

作者： Yu fu jing-Ru Nie Peng Shang Bo Zhang Da-Wei Yan xin Hao Hao Zhang State Key Laboratory of Animal Biotech Breeding China Agricultural UniversityBeijing 100193China College of Animal Science Xizang Agricultural and Animal Husbandry UniversityLinzhiXizang 860000China College of Animal Science and Technology Yunnan Agricultural UniversityKunmingYunnan 650201China

Tumor necrosis factorα(TNFα)exhibits diverse biological functions;however,its regulatory roles in myogenesis are not fully *** the present study,we explored the function of TNFαin myoblast proliferation,differentiation,migration,and myotube fusion in primary myoblasts and C2C12 *** this end,we constructed TNFαmuscle-conditional knockout(TNFα-CKO)mice and compared them with flox mice to assess the effects of TNFαknockout on skeletal *** indicated that TNFα-CKO mice displayed phenotypes such as accelerated muscle development,enhanced regenerative capacity,and improved exercise endurance compared to flox mice,with no significant differences observed in major visceral organs or skeletal *** label-free proteomic analysis,we found that TNFα-CKO altered the distribution of several muscle development-related proteins,such as Hira,Casz1,Casp7,Arhgap10,Gas1,Diaph1,Map3k20,Cfl2,and Igf2,in the nucleus and *** set enrichment analysis(GSEA)further revealed that TNFαdeficiency resulted in positive enrichment in oxidative phosphorylation and MyoD targets and negative enrichment in JAK-STAT *** findings suggest that TNFα-CKO positively regulates muscle growth and development,possibly via these newly identified targets and pathways.

关键词： TNFα Muscle-conditional knockout Myogenesis Regeneration Muscle development

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Evaluation of 3D crustal seismic velocity models in southwest China:Model performance,limitation,and prospects

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Science China Earth Sciences 2024年第2期67卷 604-619页

作者： xin WANG Ling CHEN Qi-fu CHEN Key Laboratory of Earth and Planetary Physics Institute of Geology and GeophysicsChinese Academy of SciencesBeijing100029China College of Earth and Planetary Sciences University of Chinese Academy of SciencesBeijing100049China State Key Laboratory of Lithospheric Evolution Institute of Geology and GeophysicsChinese Academy of SciencesBeijing100029China

Southwest China is a tectonically and seismically active region,witnessing strong deformation due to the collision between the Indian and Eurasian *** the subsurface velocity structure of this region is thus important in understanding the tectonics and geodynamic processes of continental collision and in mitigating seismic *** studies have provided various 3D seismic velocity models in southwest ***,discrepancies exist among these models,and less effort has been made to quantify the reliability and accuracy of these existing velocity *** this study,we use regional 3D waveform simulation to evaluate the performance of various regional crustal 3D velocity models in reproducing observed *** particularly focus on two recent earthquake sequence in the region,the 2021 Yunnan Yangbi MS_(6.4) earthquake sequence and the 2022 Sichuan Luding MS_(6.8) earthquake *** tested 3D velocity models include the Southwest China Community Velocity model V1.0,the Unified Seismic Tomography Models for Continental China Lithosphere V2.0,the adjoint full waveform tomography model of the crustal and upper mantle beneath Eastern Tibetan Plateau,and the shallow seismic structure model beneath continental *** results show that the tested 3D velocity models generally capture well long-period(full-waveform tomography model shows a slighter better performance than the other models,especially for the body-waves,highlighting the advantages

关键词： Southwest China Seismic tomography models Shallow Structures Waveform simulation Model evaluation

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Polyoxometalate-organic cage with{Ni_(4)SiW_(9)}node for photocatalytic hydrogen evolution

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Chinese Chemical Letters 2024年第3期35卷 177-181页

作者： jing Li Yeqin Feng Fangyu fu xing xin Guoyu Yang Hongjin Lv MOE Key Laboratory of Cluster Science Beijing Key Laboratory of Photoelectroic/Electrophotonic Conversion MaterialsSchool of Chemistry and Chemical EngineeringBeijing Institute of TechnologyBeijing 102488China

The design and syntheses of metal-organic cages(MOCs)based on polyoxometalates(POMs)building blocks have attracted increasing attention due to their intriguing molecular architectures and physicochemical *** this work,we have successfully synthesized and systematically characterized a tetrahedral polyoxometalate-based organic cage(POC),K_(3)Na_(17)H_(12)[(C_(4)H_(6)O_(6))_(6)[Ni_(4)(OH)_(3)(A-α-SiW_(9)O_(34))]_(4)]·96H_(2)O(Ni_(16)L_(6)(SiW_(9))_(4)),using tritopic Ni_(4)-substituted Keggin cluster(Ni_(4)SiW_(9))as nodes and flexible L-(+)-tartaric acid ligands as *** resulting POC tetrahedron has been firstly investigated as efficient catalyst for visible-light-driven hydrogen production,achieving a turnover number of 15,500 after 96-h *** high catalytic performance of Ni_(16)L_(6)(SiW_(9))_(4)POC catalyst could be attributed to its unique cage structure,thereby offering more efficient catalytic component *** addition,spectroscopic analyses illustrated the photocatalytic mechanism and the structural stability of the TBA-Ni_(16)L_(6)(SiW_(9))_(4)catalyst during the photocatalytic process.

关键词： Polyoxometalate Polyoxometalate-organic cages Photocatalytic hydrogen production Visible light illumination

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Interfacial Electronic Modulation of Dual-Monodispersed Pt–Ni_(3)S_(2) as Efficacious Bi-functional Electrocatalysts for Concurrent H_(2) Evolution and Methanol Selective Oxidation

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Nano-Micro Letters 2024年第4期16卷 415-431页

作者： Qianqian Zhao Bin Zhao xin Long Renfei Feng Mohsen Shakouri Alisa Paterson Qunfeng Xiao Yu Zhang Xian‑Zhu fu jing‑Li Luo Shenzhen Key Laboratory of Energy Electrocatalytic Materials Shenzhen Key Laboratory of Polymer Science and TechnologyGuangdong Research Center for Interfacial Engineering of Functional MaterialsCollege of Materials Science and EngineeringShenzhen UniversityShenzhen 518060People’s Republic of China Canadian Light Source Inc. SaskatoonSK S7N 0X4Canada Instrumental Analysis Center of Shenzhen University(Lihu Campus) Shenzhen UniversityShenzhen 518055People’s Republic of China

Constructing the efficacious and applicable bifunctional electrocatalysts and establishing out the mechanisms of organic electro-oxidation by replacing anodic oxygen evolution reaction(OER) are critical to the development of electrochemicallydriven technologies for efficient hydrogen production and avoid CO_(2) emission. Herein, the hetero-nanocrystals between monodispersed Pt(~ 2 nm) and Ni_(3)S_(2)(~ 9.6 nm) are constructed as active electrocatalysts through interfacial electronic modulation, which exhibit superior bi-functional activities for methanol selective oxidation and H_(2) generation. The experimental and theoretical studies reveal that the asymmetrical charge distribution at Pt–Ni_(3)S_(2) could be modulated by the electronic interaction at the interface of dual-monodispersed heterojunctions, which thus promote the adsorption/desorption of the chemical intermediates at the interface. As a result, the selective conversion from CH_(3)OH to formate is accomplished at very low potentials(1.45 V) to attain 100 m A cm^(-2) with high electronic utilization rate(~ 98%) and without CO_(2) emission. Meanwhile, the Pt–Ni_(3)S_(2) can simultaneously exhibit a broad potential window with outstanding stability and large current densities for hydrogen evolution reaction(HER) at the cathode. further, the excellent bi-functional performance is also indicated in the coupled methanol oxidation reaction(MOR)//HER reactor by only requiring a cell voltage of 1.60 V to achieve a current density of 50 m A cm^(-2) with good reusability.

关键词： Dual-monodispersed heterostructure Electronic interactive modulation Reaction mechanism Methanol oxidation reaction Hydrogen generation

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Photoinduced Dehalocyclization to Access Oxindoles Using Formate as a Reductant

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Chinese Journal of Chemistry 2024年第11期42卷 1203-1208页

作者： Wei Ge Jia-xin Wang Ming-Chen fu Yao fu Hefei National Research Center for Physical Sciences at the Microscale iChEMCAS Key Laboratory of Urban Pollutant ConversionAnhui Province Key Laboratory of Biomass Clean EnergyUniversity of Science and Technology of ChinaHefeiAnhui230026 China Anhui Province Key Laboratory of Value-Added Catalytic Conversion and Reaction Engineering Hefei University of TechnologyHefeiAnhui230009 China

Herein,we report an efficient and practical protocol for the photoinduced dehalocyclization of ortho-halophenylacrylamides with formate by the engagement of a CO_(2) radical anion to access substituted *** method proceeds smoothly under mild conditions and exhibits a wide range of substrate as well as remarkable functional group compatibility.

关键词： Formate CO_(2)Radical ions Oxindole Photocatalysts Cyclization Organohalides

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Mechanical property of cylindrical sandwich shell with gradient core of entangled wire mesh

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Defence Technology（防务技术） 2024年第1期31卷 510-522页

作者： xin Xue Chao Zheng fu-qiang Lai Xue-qian Wu Institute of Metal-rubber&Vibration Noise School of Mechanical Engineering and AutomationFuzhou UniversityFuzhou350116China fujian Metallurgical Industry Design Institute Fuzhou350116China

To explore the wide-frequency damping and vibration-attenuation performances in the application of aerospace components,the cylindrical sandwich shell structure with a gradient core of entangled wire mesh was proposed in this ***,the gradient cores of entangled wire mesh in the axial and radial directions were prepared by using an in-house Numerical Control weaving machine,and the metallurgical connection between skin sheets and the gradient core was performed using vacuum ***,to investigate the mechanical properties of cylindrical sandwich shells with axial or radial gradient cores,quasi-static and dynamic mechanical experiments were carried *** primary evaluations of mechanical properties include secant stiffness,natural frequency,Specific Energy Absorption(SEA),vibration acceleration level,and so *** results suggest that the vibration-attenuation performance of the sandwich shell is remarkable when the high-density core layer is at the end of the shell or abuts the inner *** axial gradient material has almost no influence on the vibration frequencies of the shell,whereas the vibration frequencies increase dramatically when the high-density core layer approaches the ***,compared to the conventional sandwich shells,the proposed functional grading cylindrical sandwich shell exhibits more potential in mass reduction,stiffness designing,and energy dissipation.

关键词： Entangled wire mesh Gradient cylindrical sandwich shell Vacuum brazing Secant stiffness Damping

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Reputation-based joint optimization of user satisfaction and resource utilization in a computing force network

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Frontiers of Information Technology & Electronic Engineering 2024年第5期25卷 685-700页

作者： Yuexia fu jing WANG Lu LU Qinqin TANG Sheng ZHANG China Mobile Research Institute Beijing 100053China Purple Mountain Laboratories Nanjing 211111China China Mobile Communications Corporation Beijing 100033China

Under the development of computing and network convergence,considering the computing and network resources of multiple providers as a whole in a computing force network(CFN)has gradually become a new ***,since each computing and network resource provider(CNRP)considers only its own interest and competes with other CNRPs,introducing multiple CNRPs will result in a lack of trust and difficulty in unified *** addition,concurrent users have different requirements,so there is an urgent need to study how to optimally match users and CNRPs on a many-to-many basis,to improve user satisfaction and ensure the utilization of limited *** this paper,we adopt a reputation model based on the beta distribution function to measure the credibility of CNRPs and propose a performance-based reputation update ***,we formalize the problem into a constrained multi-objective optimization problem and find feasible solutions using a modified fast and elitist non-dominated sorting genetic algorithm(NSGA-II).We conduct extensive simulations to evaluate the proposed *** results demonstrate that the proposed model and the problem formulation are valid,and the NSGA-II is effective and can find the Pareto set of CFN,which increases user satisfaction and resource ***,a set of solutions provided by the Pareto set give us more choices of the many-to-many matching of users and CNRPs according to the actual situation.

关键词： Computing force network Resource scheduling Performance-based reputation User satisfaction

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Inactivation of TGF-βsignaling in CAR-T cells

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Cellular & Molecular Immunology 2024年第3期21卷 309-310页

作者： Zaopeng Yang Yang-xin fu Changping Laboratory Beijing102206China Department of Basic Medical Sciences School of MedicineTsinghua UniversityBeijing100084China

Accumulating evidence suggests that the transforming growth factor(TGF)-βpathway might be an ideal target for optimizing chimeric antigen receptor(CAR)-T-cell therapy for solid *** Cellular&Molecular Immunology,Liang et al.(2024)reported that CAR-T cells expressing SMAD7 displayed favorable antitumor efficacy and a reduced risk of systemic toxicity.

关键词： CAR Immunology

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Editorial:special topic on heterojunction in photocatalysts

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Science China Materials 2024年第2期67卷 379-381页

作者： Jiaguo Yu xin Li Junwei fu Kai Dai Laboratory of Solar fuel Faculty of Materials Science and ChemistryChina University of GeosciencesWuhan 430078China Institute of Biomass Engineering Key Laboratory of Energy Plants Resource and UtilizationMinistry of Agriculture and Rural AffairsSouth China Agricultural UniversityGuangzhou 510642China Hunan Joint International Research Center for Carbon Dioxide Resource Utilization State Key Laboratory of Powder MetallurgySchool of PhysicsCentral South UniversityChangsha 410083China Anhui Province Key Laboratory of Pollutant Sensitive Materials and Environmental Remediation College of Physics and Electronic InformationHuaibei Normal UniversityHuaibei 235000China

To suppress the recombination of photogenerated electron and hole in single photocatalysts,an important method is to design heterojunction by combining two *** method has widely used to enhance the photocatalytic performance of the *** the begining,most people use type-Ⅱcharge carrier transfer mechanism to explain the emhanced activity of the composite semiconductors.

关键词： composite heterojunction method

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