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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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Chiral Thermally Activated Delayed Fluorescence-Active Macrocycles Displaying Efficient Circularly Polarized Electroluminescence

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CCS Chemistry 2022年第11期4卷 3540-3548页

作者： Wen-Long Zhao Yin-Feng Wang Shi-Peng Wan hai-yan lu Meng Li Chuan-Feng Chen School of Chemical Sciences University of Chinese Academy of SciencesBeijing 100049 Beijing National Laboratory for Molecular Sciences CAS Key Laboratory of Molecular Recognition and FunctionInstitute of ChemistryChinese Academy of SciencesBeijing 100190

An efficient strategy for constructing chiral macrocycles with both thermally activated delayed fluorescence(TADF)and highly efficient circularly polarized electroluminescence(CPEL)properties was ***,a pair of macrocyclic enantiomers(+)-(R,R)-MC and(−)-(S,S)-MC was synthesized by a combination of chiral octahydro-binaphthol moiety with triazine-based TADF *** chiral macrocycles exhibited obvious TADF properties with a lowΔE_(ST) of 0.067 eV,aggregation-induced emission behaviors,and high photoluminescence quantum yields of up to 79.7%.Moreover,the macrocyclic enantiomers showed mirror images in circular dichroism spectra and circularly polarized luminescence ***,the chiralmacrocycleswere suitable for the preparation of solution-processed circularly polarized organic light-emitting diodes,which displayed excellent device performances with a highmaximum external quantum efficiency of up to 17.1%,low-efficiency roll-off of 3.5%at 1000 cdm^(−2),andintenseCPELalong with electroluminescence dissymmetry factor of 1.7×10^(−3).

关键词： chiral macrocycle thermally activated delayed fluorescence circularly polarized luminescence organic light-emitting diode

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Spatial construction of ultrasmall Pt-decorated 3D spinel oxidemodified N-doped graphene nanoarchitectures as high-efficiency methanol oxidation electrocatalysts

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Rare Metals 2024年第1期43卷 186-197页

作者： Qi Zhang Min-Min yan Wen-Fa Du Chen-Yu Yin Jian Zhang lu yang Yun-Qing Kang hai-yan He Hua-Jie Huang College of Mechanics and Materials Hohai UniversityNanjing 210098China New Energy Technology Engineering Lab of Jiangsu Province College of ScienceNanjing University of Posts&Telecommunications(NUPT)Nanjing 210023China International Center for Materials Nanoarchitectonics(WPIMANA) National Institute for Materials ScienceTsukuba 305-0044IbarakiJapan

Direct methanol fuel cell technology recently becomes the focus of both academic and engineering circles,which stimulates the exploitation and utilization of advanced electrode catalysts with high activity and long ***,we demonstrate a robust bottom-up approach to the spatial construction of three-dimensional(3D) spinel manganese-cobalt oxide-modified N-doped graphene nanoarchitectures decorated with ultrasmall Pt nanoparticles(Pt/MnCo_(2)O_(4)-NG) via a controllable selfassembly *** incorporation of MnCo_(2)O_(4)nanocrystals provides abundant hydroxyl sources to promote the oxidative removal of CO-like byproducts on Pt sites,while the existence of 3D porous N-doped graphene networks facilitates the transportation of both ions and electrons in the hybrid system,thus giving rise to remarkable synergetic coupling effects during the methanol oxidation ***,the optimized Pt/MnCo_(2)O_(4)-NG nano architecture expres ses exceptional electrocatalytic properties with a large electrochemically active surface area of 99.5 m^(2)·g^(-1),a high mass activity of1508.3 mA·mg^(-1),strong toxicity resistance and reliable long-term durability,which have obvious competitive advantages over those of conventional Pt/carbon black,Pt/carbon nano tube,Pt/graphene,and Pt/N-doped graphene catalysts with the same Pt usage.

关键词： Platinum MnCo_(2)O_(4) 3D graphene Electrocatalysts Methanol oxidation

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Study on Optimal Water ControlMethods for Horizontal Wells in Bottom Water Clastic Rock Reservoirs

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Fluid Dynamics & Materials Processing 2024年第10期20卷 2377-2392页

作者： Xianghua Liu hai Song lu Zhao yan Zheng Neng yang Dongling Qiu Sinopec Northwest Oilfield Branch Northwest Oilfield Branch of China Petroleum and Chemical CorporationUrumqi830011China School of Petroleum and Natural Gas Engineering Southwest Petroleum UniversityChengdu610500China

The segmented water control technology for bottom water reservoirs can effectively delay the entry of bottom water and adjust the production *** clarify the impact of different methods on horizontal well production with different reservoir conditions and to provide theoretical support for the scientific selection of methods for bottom water reservoirs,a numerical simulation method is presented in this study,which is able to deal with wellbore reservoir coupling under screen tube,perforation,and ICD(Inflow Control Device)*** the geological characteristics of the bottom-water conglomerate reservoir in the Triassic Formation of the Tahe Block 9 as a test case,the three aforementioned completion methods are tested to predict the transient production *** impact of completion parameters,reservoir permeability,bottom-water energy,and individual well control on the time to encounter water in horizontal wells(during a water-free production period)is discussed.A boundary chart for the selection of completion methods is introduced *** results show that the optimized ICD completion development effect for heterogeneous reservoirs is the best,followed by optimized perforation *** is the main factor affecting the performances of completion methods,while bottom water energy and single well controlled reserves have a scarce *** average permeability of the reservoir is less than 500 mD,and ICD has the best water control *** the permeability is greater than 500 mD,the water control effect of perforation completion becomes a better option.

关键词： Completion method bottom water low-permeability oil reservoir horizontal well development effect

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Fast measurement and prediction method for electromagnetic susceptibility of receiver

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Journal of Systems Engineering and Electronics 2024年第2期35卷 275-285页

作者： CHEN yan lu Zhonghao LIU Yunxia State Key Laboratory of Complex Electromagnetic Environment Effects on Electronics and Information System College of Electronic Science and TechnologyNational University of Defense TechnologyChangsha 410073China State Key Laboratory of Astronautic Dynamics Xi’an 710043China

Aiming at evaluating and predicting rapidly and accurately a high sensitivity receiver’s adaptability in complex electromagnetic environments,a novel testing and prediction method based on dual-channel multi-frequency is proposed to improve the traditional two-tone ***,two signal generators are used to generate signals at the radio frequency(RF)by frequency scanning,and then a rapid measurement at the intermediate frequency(IF)output port is carried out to obtain a huge amount of sample data for the subsequent ***,the IF output response data are modeled and analyzed to construct the linear and nonlinear response constraint equations in the frequency domain and prediction models in the power domain,which provide the theoretical criteria for interpreting and predicting electromagnetic susceptibility(EMS)of the *** experiment performed on a radar receiver confirms the reliability of the method proposed in this *** shows that the interference of each harmonic frequency and each order to the receiver can be identified and predicted with the sensitivity *** on this,fast and comprehensive evaluation and prediction of the receiver’s EMS in complex environment can be efficiently realized.

关键词： electromagnetic susceptibility(EMS) receiver dualchannel multi-frequency nonlinear response frequency domain power domain

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Ultra-flexible all-in-one anti-freeze photothermally enhanced supercapacitors

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Nano Research 2024年第8期17卷 7221-7229页

作者： Yongxin lu Keke Li yan-yan Song Zhida Gao Condense matter of physics College of ScienceNortheazistern UniversityShenyang 110004China

Wearable electronics powered by flexible energy storage devices have captured global *** low-temperature conditions,unfortunately,solidification of flexible hydrogel electrolyte and decreased pseudo-capacity of these devices largely hamper their practical *** this study,photothermally-active Prussian blue(PB)was introduced onto poly(3,4-ethylenedioxythiophene)/polyacrylamide(PEDOT/PAM)networks to address the challenges of electrolyte solidification and degraded pseudo-capacitance for flexible all-in-one device at low *** as-constructed PB/PEDOT/PAM hydrogel device delivers stable electrochemical performance and remarkable mechanical property with 1652%***,this hydrogel device well retains its flexibility in cold environment with a freezing point below−30℃.The incorporation of PB extends the voltage range to 1.5 V as a single device,thus significantly enhancing the electrochemical performance as an all-inone integrated *** from the outstanding photo-to-thermal conversion ability of embedded PB nanocubes,the temperature of the assembled all-in-one PB/PEDOT/PAM device increased from^(−2)0 to 17.7℃ after solar-light irradiation for only 5 ***,the degraded pseudo-capacitance was subsequently boosted to 287.1%of its original capacitance at−20℃.This study establishes a connection between flexible all-solid-state hydrogel devices and photothermally enhanced pseudocapacitors in freezing environments,thereby expanding the potential applications of multi-functional pseudo-capacitors.

关键词： all-in-one energy storage devices all-solid-state photothermal flexible low temperatures

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Significant effect of ordered micro-domain on cell boundary phase distribution and demagnetization curve squareness of Sm_(2)Co_(17)-type magnet

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Rare Metals 2024年第4期43卷 1724-1735页

作者： Zhuang Liu hai-Chen Wu Chao-Yue Zhang Chao-Qun Zhu Guo-Xin Chen Huan-Ming lu Ren-Jie Chen Aru yan CISRI&NIMTE Joint Innovation Center for Rare Earth Permanent Magnets Ningbo Institute of Materials Technology and EngineeringChinese Academy of SciencesNingbo315201China University of Chinese Academy of Sciences Beijing100049China Analytical Center Ningbo Institute of Materials Technology and EngineeringChinese Academy of SciencesNingbo315201China

Cu-rich cell boundary phase is difficult to precipitate evenly,resulting in a generally poor demagnetization curve squareness for Fe-rich Sm_(2)Co_(17)-type magnet,which is a key factor limiting the further improvement of magnetic energy *** this study,we report that nanoscale strip-like ordered micro-domains distributed in1:7H disordered matrix phase of the solid solution precursor is a new factor significantly affecting the precipitation and distribution of the cell boundary *** strip-like and continuous micro-twin structure with twin boundaries neatly perpendicular to the C-axis is observed after sintering *** solution treatment,sequential and long strip-like micro-twins gradually transform into disordered state along the basal plane,forming narrow disordered 1:7H(TbCu_(7)-type structure)phase between the separated strip-like ordered *** disordering transformation takes place via broken down of the long strip-like ordered micro-domains,which is accomplished by narrowing along the width direction followed by reduction of the ***,a new model revealing the effect of the ordered micro-domains on the formation of the cell boundary phase is *** boundaries enriched in Cu have already existed in the precursor with long strip-like ordered ***,the Cu-rich cell boundary phase acting as strong pinning centers cannot be precipitated homogeneously and distributed continuously after aging,resulting in a poor demagnetization curve squareness of Sm_(2)Co_(17)-type *** results indicate that significant broken down of the nanoscale ordered micro-domains in solution precursor is the key factor improving the distribution of cell boundary phase in Sm_(2)Co_(17)-type magnets.

关键词： Sm_(2)Co_(17) magnets Solution precursor Ordered micro-domains Disordering transformation Cellular structure

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Snowpack variations and their hazardous effects under climate warming in the central Tianshan Mountains

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Advances in Climate Change Research 2024年第3期15卷 442-451页

作者： Jian-Sheng HAO yan WANG Lan-hai LI Key Laboratory of Land Surface Pattern and Simulation Institute of Geographic Sciences and Natural Resources ResearchChinese Academy of Sciences(CAS)Beijing 100101China Xinjiang Key Laboratory of Water Cycle and Utilization in Arid Zone Urumqi 830011China State Key Laboratory of Desert and Oasis Ecology Xinjiang Institute of Ecology and GeographyChinese Academy of SciencesUrumqi 830011China Tianshan Snowcover and Avalanche Observation and Research Station of Xinjiang Xinyuan 835800China CAS Research Center for Ecology and Environment of Central Asia Urumqi 830011China

Climate change alters snowpack evolution,which in turn influences the likelihood of snow avalanches and flood *** lack of systemic observational data on key snow characteristics in high mountains remains a scientific challenge in terms of systematically elucidating the dynamic chain of variations in climate-snowpack-snow *** restricts our understanding and poses challenges in the prediction of snow-related disaster *** such,this study analysed the variations of temperature and snowfall and the physical characteristics of snowpacks based on ground-based observations from the Kunse River Valley situated in the Tianshan Mountains from 1967 to *** results reveal that the temperature increased significantly by 0.32°C per decade(p<0.01)during the snow season,along with more extreme snowfall *** snow-cover duration was observed to have been shortened by 4.77 d per decade(p<0.01)from 1967 to 2021,which is characterised by later snow-cover onset and earlier ***,average and maximum snow depths increased along with an increase in peak snow water equivalent,thus indicating a higher frequency of extremely scarce or abundant snow *** low snowpack temperature gradient and earlier snowmelt dates in spring lead to earlier occurrences of snowmelt floods and wet *** the risks of these events increase,they pose greater threats to farmlands,road transportation,water-electricity infrastructure and several other human ***,these insights are critical for providing vital information that can deepen our understanding of the impact of climate change on snowpack characteristics and improve management strategies for snow-related disaster prevention and mitigation.

关键词： Climate change Snowpack variations Snow-related disaster Tianshan Mountains

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Upconversion-based chiral nanoprobe for highly selective dual-mode sensing and bioimaging of hydrogen sulfide in vitro and in vivo

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Light(Science & Applications) 2024年第9期13卷 1878-1887页

作者： yang lu Xu Zhao Dongmei yan Yingqian Mi Peng Sun Xu yan Xiaomin Liu Geyu lu State Key Laboratory of Integrated Optoelectronics College of Electronic Science and EngineeringJilin University130012 ChangchunChina Department of Immunology College of Basic Medical SciencesJilin University130021 ChangchunChina

Chiral assemblies have become one of the most active research areas due to their versatility,playing an increasingly important role in bio-detection,imaging and *** this work,chiral UCNPs/CuxOS@ZIF nanoprobes are prepared by encapsulating upconversion nanoparticles(UCNPs)and CuxOS nanoparticles(NPs)into zeolitic imidazolate framework-8(ZIF-8).The novel excited-state energy distribution-modulated upconversion nanostructure(NaYbF4@-NaYF4:Yb,Er)is selected as the fluorescence source and energy donor for highly efficient fluorescence resonance energy transfer(FRET).CuxOS NP is employed as chiral source and energy acceptor to quench upconversion luminescence(UCL)and provide circular dichroism(CD)*** the natural adsorption and sorting advantages of ZIF-8,the designed nanoprobe can isolate the influence of other common disruptors,thus achieve ultra-sensitive and highly selective UCL/CD dual-mode quantification of H2S in aqueous solution and in living ***,the nanoprobe is also capable of in vivo intra-tumoral H2S *** work highlights the multifunctional properties of chiral nanocomposites in sensing and opens a new vision and idea for the preparation and application of chiral nanomaterials in biomedical and biological analysis.

关键词： chiral sulfide donor

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A Permanent Magnet Linear Synchronous Motor Drive for HTS Maglev Transportation Systems

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Journal of Electronic Science and Technology of China 2008年第2期6卷 125-129页

作者： You-Guang Guo Jian-Xun Jin lu-hai Zheng Jian-Guo Zhu hai-yan lu Province-Ministry Joint Key Laboratory of Electromagnetic Field and Electrical Apparatus Reliability Hebei University of Technology Tianjin 300130 China and the Faculty of Engineering and Information Technology University of Technology Sydney NSW 2007 Australia Center of Applied Superconductivity and Electrical Engineering University of Electronic Science and Technology of China Chengdu 610054 China Faculty of Engineering and Information Technology University of Technology Sydney NSW 2007Australia

A permanent magnet linear synchronous motor （PMLSM） for a high temperature superconducting （HTS） maglev system has been studied, including the motor structure, control strategy, and analysis techniques. Finite element analysis （FEA） of magnetic field is conducted to accurately calculate major motor parameters. Equivalent electrical circuit is used to predict the drive＇s steady-state characteristics, and a phase variable model is applied to predict the dynamic performance. Preliminary experiment with a prototype has been made to verify the theoretical analysis and the HTS-PM synchronous driving technology.

关键词： High temperature superconducting(HTS) HTS magnetic levitation maglev transportationsystem permanent magnet linear synchronous motor.

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