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Clinical manifestations and spermatogenesis outcomes in Chinese patients with congenital hypogonadotropic hypogonadism caused by inherited or de novo FGFR1 mutations

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Asian Journal of Andrology 2024年第4期26卷 426-432页

作者： Yu-Fan Yang Hai-Lu Ma Xi Wang min Nie jiang-Feng Mao Xue-Yan Wu Department of Endocrinology Peking Union Medical College HospitalChinese Academy of Medical SciencesPeking Union Medical CollegeBeijing 100730China.

Fibroblast growth factor receptor 1(FGFR1)mutations are associated with congenital hypogonadotropic hypogonadism(CHH)through inheritance or spontaneous *** detected FGFR1 mutations in a Chinese cohort of 210 CHH patients at Peking Union Medical College Hospital(Beijing,China)using next-generation and Sanger *** assessed missense variant pathogenicity using six bioinformatics tools and compared clinical features and treatment outcomes between inherited and de novo mutation *** 19 patients with FGFR1 mutations,three were recurrent,and 16 were novel *** of the novel mutations were likely pathogenic according to the American College of Medical Genetics and Genomics(ACMG)guidelines,with the prevalent P366L *** majority of FGFR1 mutations was inherited(57.9%),with frameshift mutations exclusive to the de novo mutation *** inherited mutation group had a lower incidence of cryptorchidism,short stature,and skeletal *** the inherited mutation group,luteinizing hormone(LH)levels were 0.5 IU l−1,follicle-stimulating hormone(FSH)levels were 1.0 IU l−1,and testosterone levels were 1.3 nmol l−*** contrast,the de novo group had LH levels of 0.2 IU l−1,FSH levels of 0.5 IU l−1,and testosterone levels of 0.9 nmol l−1,indicating milder hypothalamus–pituitary–gonadal axis(HPGA)functional deficiency in the inherited *** inherited mutation group showed a tendency toward higher spermatogenesis *** conclusion,this study underscores the predominance of inherited FGFR1 mutations and their association with milder HPGA dysfunction compared to de novo mutations,contributing to our understanding of the genetic and clinical aspects of FGFR1 mutations.

关键词： congenital hypogonadotropic hypogonadism de novo FGFR1 inherited

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Protective effects of baffles with different positions,row spacings,heights on debris flow impact

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Journal of Mountain Science 2024年第7期21卷 2352-2367页

作者： SUN Xinpo CHEN min BI Yuzhang ZHENG Lu CHE Chi XU Ao TIAN Zijian jiang Zheyuan College of Civil Engineering Sichuan University of Science and EngineeringZigong 643000China College of Resources and Environment Fujian Agriculture and Forestry UniversityFuzhou 350002China School of Civil Engineering Fuzhou UniversityFuzhou 350108China jiangsu Key Laboratory of Urban Underground Engineering&Environmental Safety Institute of Geotechnical EngineeringSoutheast UniversityNanjing 210096China Beijing Urban Construction Science Technology Promoting Association Beijing 100055China SUSE-BZ Geoenvironmental Engineering Joint Laboratory Sichuan University of Science and EngineeringZigong 643000China

The baffle effectively slowed down debris flow velocity,reduced its kinetic energy,and significantly shortened the distance of debris flow ***,they are widely used for protection against natural hazards such as landslides and *** study,based on the threedimensional DEM(Discrete Element Method),investigated the impact of different baffle positions on debris flow *** flow velocity and kinetic energy variations were studied through single-factor *** baffle positions were preliminarily selected by analyzing the influence of the first-row baffle position on the impact force and accumulation mass of debris ***,based on the selected baffle positions and four factors influencing the effectiveness of baffle protection(baffle position(P),baffle height(h),row spacing(S_(r)),and angle of transit area(α)),an orthogonal design was employed to further explore the optimal arrangement of *** research results indicate that the use of a baffle structure could effectively slow down the motion velocity of debris flows and dissipate their *** the baffle is placed in the transit area,the impact force on the first-row baffle is greater than that when the baffle is placed in the deposition ***,when the baffle is placed in the transit area,the obstruction effect on debris flow mass is also greater than that when the baffle is placed in the deposition *** orthogonal experimental range analysis,when the impact on the first row of baffles is used as the evaluation criterion,the importance of each influencing factor is ranked asα>P>S_(r)>*** the mass of debris flow behind the baffle is regarded as the evaluation criterion,the rank is changed to P>α>S_(r)>*** experimental simulation results show that the optimal baffle arrangement is:P_(5),S_(r)=16,α=35°,h=9.

关键词： Debris flow Baffle Protective effect Discrete element

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GRK2 mediated degradation of SAV1 initiates hyperplasia of fibroblast-like synoviocytes in rheumatoid arthritis

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Acta Pharmaceutica Sinica B 2024年第3期14卷 1222-1240页

作者： Paipai Guo Ji jiang Rui Chu Feng He mingli Ge Ruhong Fang Qiuyun guan Huijuan Cheng Chunru jiang Tiantian Su Zhenduo Zhu Hao Liu Wei Wei Shihao Zhang Qingtong Wang Institute of Clinical Pharmacology Anhui Medical UniversityKey Laboratory of Anti-inflammatory and Immune MedicineMinistry of EducationCollaborative Innovation Center of Anti-inflammatory and Immune MedicinesHefei 230032China School of Pharmacy Bengbu Medical CollegeBengbu 233030China The Third Affiliated Hospital of Anhui Medical University(the First People’s Hospital of Hefei) Hefei 230061China

Hyperplasia and migration of fibroblast-like synoviocytes(FLSs)are the key drivers in the pathogenesis of rheumatoid arthritis(RA)and joint *** Yes-associated protein(YAP),which is a powerful transcription co-activator for proliferative genes,was observed in the nucleus of inflammatory FLSs with unknown upstream *** Gene Expression Omnibus database analysis,it was found that Salvador homolog-1(SAV1),the pivotal negative regulator of the Hippo-YAP pathway,was slightly downregulated in RA ***,SAV1 protein expression is extremely ***,it was revealed that SAV1 is phosphorylated,ubiquitinated,and degraded by interacting with an important serine-threonine kinase,G protein-coupled receptor(GPCR)kinase 2(GRK2),which was predominately upregulated by GPCR activation induced by ligands such as prostaglandin E2(PGE2)in *** process further contributes to the decreased phosphorylation,nuclear translocation,and transcriptional potency of YAP,and leads to aberrant FLSs *** depletion of GRK2 or inhibition of GRK2 by paroxetine rescued SAV1 expression and restored YAP phosphorylation and finally inhibited RA FLSs proliferation and ***,paroxetine treatment effectively reduced the abnormal proliferation of FLSs in a rat model of collagen-induced arthritis which was accompanied by a significant improvement in clinical ***,these results elucidate the significance of GRK2 regulation of Hippo-YAP signaling in FLSs proliferation and migration and the potential application of GRK2 inhibition in the treatment of FLSs-driven joint destruction in RA.

关键词： Rheumatoid arthritis Fibroblast-like synoviocytes G protein-coupled receptor kinase 2 Salvador homolog-1 Yes-associated protein

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Enhanced Photocatalytic CO_(2) Reduction via Linkage Substitution in Porphyrinic Covalent Organic Frameworks

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CCS Chemistry 2024年第7期6卷 1689-1697页

作者： Suleman Suleman Kang Sun Yiming Zhao Xinyu guan Zhongyuan Lin Zheng Meng Hai-Long jiang Department of Chemistry University of Science and Technology of ChinaHefeiAnhui 230026

A catalytic performance is greatly impacted by the microenvironment surrounding the catalytic ***,efforts to modify the microenvironment are primarily directed towards the first coordination spheres,while controlling the secondary coordination spheres is entirely *** this work,a series of porphyrin-based covalent organic frameworks(COFs)with distinct linkage were rationally designed to investigate the structure-property relationship between the secondary coordination spheres of catalytic centers and CO_(2) *** secondary coordination spheres of COFs could be changed readily by manipulating the organic *** photocatalytic CO_(2) reduction,the COF withβ-ketoenamine as the linkage(NiPor-BDOB)exhibited superior activity,selectivity,and stability over its imine-linked counterpart(NiPor-BDA).Experiments and theoretical calculations indicated that the distinct secondary coordination sphere resulted in an electronic density change of Ni centers,leading to enhanced photocatalytic *** study highlights the significance of controlling the secondary coordination sphere around the active site.

关键词： covalent organic frameworks linkage substitution photocatalysis CO_(2)reduction activity selectivity

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The centrosymmetric to non-centrosymmetric transformation induced by alkaline-earth cations producing infrared nonlinear optical AeMn_(6)Ga_(6)S_(16)(Ae=Ca,Sr)

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Science China Chemistry 2024年第9期67卷 2941-2948页

作者： Yang-Ping Zhang Shao-min Pei Wen-Fa Chen Bin-Wen Liu Xiao-ming jiang Guo-Cong Guo State Key Laboratory of Structural Chemistry Fujian Institute of Research on the Structure of MatterChinese Academy of SciencesFuzhou 350002China Fujian Science&Technology Innovation Laboratory for Optoelectronic Information of China Fuzhou 350108China School of Physical Science and Technology Shanghai Tech UniversityShanghai 201210China

Non-centrosymmetric(NCS)structure is an indispensable prerequisite for second-order nonlinear optical(NLO)***,the mainstream approach for designing new NCS structures still relies on chemical substitution,necessitating an urgent infusion of fresh *** this work,two isomorphic sulfides AeMn_(6)Ga_(6)S_(16)(Ae=Ca,1;Sr,2)are afforded by modifying the skeleton of centrosymmetric(CS)Mn_(2)Ga_(2)S_(5) via introducing strong electropositive alkaline-earth ***,the incorporation of Ca^(2+)/Sr^(2+)cations allows the elementary[MnGaS_(9)]_(∞)chains to reconfigure into bumpy[MnS_(6)]-[GaS_(4)]layers in a new engagement manner,thus enabling the conversion from CS to NCS *** modifications cause not only strong second-harmonic generation(SHG)responses(~1.5×benchmark AgGaS_(2)@1,700 nm),but also significantly feasible bandgaps(2.60-2.64 e V)compared to the sulfide Mn_(2)Ga_(2)S_(5)(1.36 e V),which ultimately facilitate ultrahigh laser-induced damage thresholds(4.4-6.6×AgGaS_(2)@1,064 nm for compounds 1 and 2).The present study offers an ingenious approach for the transformation of CS to NCS structure.

关键词： second-harmonic generation non-centrosymmetrical structure NLO functional motif solid-state method

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FAM53B promotes pancreatic ductal adenocarcinoma metastasis by regulating macrophage M2 polarization

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World Journal of Gastrointestinal Oncology 2024年第4期16卷 1479-1499页

作者： Xuan-Zeng Pei min Cai Da-Wei jiang Song-Hai Chen Qing-Qing Wang Hui-min Lu Yi-Fan Lu Department of Hepatological Surgery The First Hospital of JiaxingJiaxing 314000Zhejiang ProvinceChina Department of General Surgery West China Hospital of Sichuan UniversityChengdu 610044Sichuan ProvinceChina

BACKGROUND Our study investigated the role of FAM53B in regulating macrophage M2 polarization and its potential mechanisms in promoting pancreatic ductal adenocarcinoma(PDAC)*** To further investigate the role of FAM53B in regulating macrophage M2 polarization and its potential mechanism in promoting PDAC *** goal is to determine how FAM53B affects macrophage M2 polarization and to define its underlying mechanism in PDAC *** Cell culture and various experiments,including protein analysis,immunohisto-chemistry,and animal model experiments,were *** compared FAM53B expression between PDAC tissues and healthy tissues and assessed the correlation of FAM53B expression with clinical *** study analyzed the role of FAM53B in macrophage M2 polarization in vitro by examining the expression of relevant ***,we used a murine model to study the role of FAM53B in PDAC metastasis and analyzed the potential underlying *** Our research showed that there was a significant increase in FAM53B levels in PDAC tissues,which was linked to adverse tumor *** findings indicated that FAM53B can enhance macrophage M2 polarization,leading to increased anti-inflammatory factor *** results from the mouse model further supported the role of FAM53B in PDAC metastasis,as blocking FAM53B prevented tumor cell invasion and *** FAM53B promotes PDAC metastasis by regulating macrophage M2 *** discovery could lead to the development of new strategies for treating *** example,interfering with the FAM53B signaling pathway may prevent cancer *** research findings also provide important information for expanding our understanding of PDAC pathogenesis.

关键词： FAM53B Pancreatic ductal adenocarcinoma Tumor metastasis Macrophage polarization

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Application of artificial synapse based on all-inorganic perovskite memristor in neuromorphic computing

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Nano Materials Science 2024年第1期6卷 68-76页

作者： Fang Luo Wen-min Zhong Xin-Gui Tang Jia-Ying Chen Yan-Ping jiang Qiu-Xiang Liu School of Physics&Optoelectric Engineering Guangdong University of TechnologyGuangzhou Higher Education Mega CenterGuangzhou510006China

Artificial synapse inspired by the biological brain has great potential in the field of neuromorphic computing and artificial *** memristor is an ideal artificial synaptic device with fast operation and good ***,we have prepared a memristor device with Au/CsPbBr_(3)/ITO *** memristor device exhibits resistance switching behavior,the high and low resistance states no obvious decline after 400 switching *** memristor device is stimulated by voltage pulses to simulate biological synaptic plasticity,such as long-term potentiation,long-term depression,pair-pulse facilitation,short-term depression,and short-term *** transformation from short-term memory to long-term memory is achieved by changing the stimulation *** addition,a convolutional neural network was constructed to train/recognize MNIST handwritten data sets;a distinguished recognition accuracy of~96.7%on the digital image was obtained in 100 epochs,which is more accurate than other memristor-based neural *** results show that the memristor device based on CsPbBr3 has immense potential in the neuromorphic computing system.

关键词： Memristor CsPbBr_(3) Resistive switching Artificial synapse Neuromorphic computing

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Understanding and addressing the challenges of online gambling addiction in China's Mainland

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General Psychiatry 2024年第2期37卷 315-318页

作者： Gangliang Zhong jiang Du min Zhao Shanghai Mental Health Center Shanghai Jiao Tong University School of MedicineShanghaiChina Shanghai Key Laboratory of Psychotic Disorders Shanghai Mental Health CenterShanghaiChina CAS Center for Excellence in Brain Science and Intelligence Technology(CEBSIT) Chinese Academy of SciencesShanghaiChina

Introduction Gambling in China has a long and evolving history,from ancient court cockfighting to preliberation casinos with games like baccarat.1 Over time,gambling in China has evolved to include a wide range of activities,including gaming machines,online gambling and commercial *** being declared illegal and regulated after the establishment of the People’s Republic of China,gambling remains deeply ingrained in Chinese culture.

关键词： mainland fighting spite

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Sono-photocatalytic amination of quinoxalin-2(1H)-ones with aliphatic amines

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Chinese Chemical Letters 2024年第10期35卷 295-298页

作者： Wen-Tao Ouyang Jun jiang Yan-Fang jiang Ting Li Yuan-Yuan Liu Hong-Tao Ji Li-Juan Ou Wei-min He School of Chemistry and Chemical Engineering University of South China National Engineering Research Center of Low-Carbon Processing and Utilization of Forest Biomass Nanjing Forestry University School of Materials Science and Engineering Hunan Institute of Technology

The first example of sono-photocatalytic bond formation was reported. With both visible light and ultrasound wave as the energy, various 3-aminoquinoxalin-2(1H)-ones were efficiently obtained with good functional group tolerance in the absence of any additive or external photocatalyst. Compared with the conventional photocatalysis, sono-photocatalysis not only dramatically improved the reaction rates and yields, but also reduced energy consumption.

关键词： Aliphatic amines Amination External photocatalyst-free Quinoxalin-2(1H)-ones Sono-photocatalysis

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Flexible bidirectional self-powered photodetector with significantly reduced volume and accelerated response speed based on hydrogel and lift-off GaN-based nanowires

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Fundamental Research 2024年第2期4卷 369-378页

作者： min jiang Yukun Zhao Penghui Zheng Jianya Zhang Wenxian Yang min Zhou Yuanyuan Wu Renjun Pei Shulong Lu Key Lab of Nanodevices and Applications Suzhou Institute of Nano-Tech and Nano-Bionics(SINANO)Chinese Academy of Sciences(CAS)Suzhou 215123China School of Nano-Tech and Nano-Bionics University of Science and Technology of ChinaHefei 230026China CAS Key Laboratory of Nano-Bio Interface Suzhou Institute of Nano-Tech and Nano-BionicsChinese Academy of SciencesSuzhou 215123China Nanchang Research Institute Suzhou Institute of Nano-Tech and Nano-BionicsChinese Academy of SciencesNanchang 330200China

Due to the wide range of potential applications for next-generation multi-functional devices,the flexible selfpowered photodetector(PD)with polarity-switchable behavior is essential but very challenging to be ***,a wearable bidirectional self-powered PD based on detached(Al,Ga)N and(In,Ga)N nanowires has been proposed and demonstrated *** from the photovoltage-competing dynamics across(Al,Ga)N and(In,Ga)N nanowire photoelectrodes,such PD can generate the positive(33.3 mA W−1)and negative(-0.019 mA W−1)photo-responsivity under ultraviolet(UV)and visible illumination,respectively,leading to the bidirectional photocurrent *** to the introduction of quasi solid-state hydrogel,the PD can work without the liquid-electrolyte,thus remarkably reducing the volume from about 482 cm3 to only 0.18 ***,the use of hydrogel is found to enhance response speed in the UV range by reducing the response time for more than 95%,which is mainly attributed to the increased open circuit potential and reduced ion transport *** the GaN connecting segment is pretty thin,the piezoelectric charges generated by stress are proposed to have only a limited effect on the photocurrent ***,both the stable on-off switching characteristics and photocurrent densities can still be achieved after being bent 400 *** an excellent flexibility,this work creates opportunities for technological applications of bidirectional photocurrent PDs in flexible optoelectronic devices,e.g.,wearable intelligent sensors.

关键词： Flexible photodetector Bidirectional photocurrent Lift-off GaN-based nanowires Hydrogel Self-powered device

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