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Novel heterozygous USH1C mutation impacts hair cell mechanotransduction and causes progressive hearing loss

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Science Bulletin 2024年第2期69卷 167-172页

作者： Yanyan jia Gaogan jia Luo Guo Nan song Yu-meng Wang Luoying jiang Yilai Shu Yan Chen Shujia Zhu Huawei Li Wenyan Li ENT Institute and Otorhinolaryngology Department of Eye&ENT Hospital State Key Laboratory of Medical Neurobiology and MOE Frontiers Center for Brain ScienceFudan UniversityShanghai 200031China Institutes of Biomedical Sciences Fudan UniversityShanghai 200032China NHC Key Laboratory of Hearing Medicine(Fudan University) Shanghai 200031China The Institutes of Brain Science and the Collaborative Innovation Center for Brain Science Fudan UniversityShanghai 200032China Shanghai Center for Brain Science and Brain-Inspired Intelligence Technology Shanghai 201210China Institute of Neuroscience State Key Laboratory of NeuroscienceCAS Center for Excellence in Brain Science and Intelligence TechnologyChinese Academy of SciencesShanghai 200031China Department of Cardiology and Institute for Developmental and Regenerative Cardiovascular MedicineXinhua Hospital Affiliated to Shanghai Jiaotong University School of MedicineShanghai 200092China

Hearing loss is one of the most common sensory disorders worldwide,affecting approximately 466 million people,including 34 million children[1].Genetic mutations accounts for approximately 60%of inherited hearing loss cases[2,3].These genetic changes result in a wide variety of clinical manifestations,ranging from nonsyndromic hearing loss(NSHL)to over 400 syndromes involving hearing loss and from mild adult-onset hearing loss to profound congenital deafness,covering the entire spectrum of Mendelian inheritance.

关键词： mec hearing approximate

Design of acceleration control schedule for adaptive cycle engine based on direct simulation model

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Chinese Journal of Aeronautics 2024年第6期37卷 307-318页

作者： Keran song Linyuan jia Yuchun CHEN Tian TAN School of Power and Energy Northwestern Polytechnical UniversityXi’an 710072China

To design the optimum acceleration control schedule for the Adaptive Cycle Engine(ACE)in the full flight envelope,this paper establishes a direct simulation model of the ACE transient *** this model,geometric parameters are used to replace the component state *** corresponding relationship between geometric parameters and component state parameters is determined by sensitivity *** geometric variables are controlled when the geometric adjustment speed exceeds the limit,and at the same time the corresponding component state parameters are *** gradient optimization algorism is used to optimize the ground acceleration process of ACE,and the control schedule in terms of operating point of compression components and corrected acceleration rate is used as the full-envelope acceleration control schedule based on the similarity *** acceleration control schedules of the triple-bypass mode and the double-bypass mode are designed in this *** acceleration processes under various flight conditions are simulated using the acceleration control *** with the acceleration process with the linear geometric adjustment schedule,the acceleration performance of ACE is improved by the acceleration control schedule,with the impulse of the acceleration process of the triple-bypass mode being increased by 8.7%-12.3% and the impulse of the double-bypass mode acceleration process being increased by 11.8%-14.1%.

关键词： Adaptive Cycle Engine(ACE) Direct simulation method Acceleration Control schedule Full flight envelope

Advances of graphdiyne-supported metal catalysts in thermocatalytic reactions

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Nano Research 2024年第4期17卷 2223-2233页

作者： jia Yu Yida Yang Yuliang Li Changyan Cao Weiguo song Beijing National Laboratory for Molecular Sciences CAS Research/Education Center for Excellence in Molecular SciencesLaboratory of Molecular Nanostructures and NanotechnologyInstitute of ChemistryChinese Academy of SciencesBeijing 100190China School of Chemical Sciences University of Chinese Academy of SciencesBeijing 100049China Beijing National Laboratory for Molecular Sciences CAS Research/Education Center for Excellence in Molecular SciencesLaboratory of organic solidsInstitute of ChemistryChinese Academy of SciencesBeijing 100190China

Supported metal catalysts are widely used in the modern chemical *** electronic interaction between supports and active components is of great significance for heterogeneous ***(GDY),a new type of carbon allotrope with sp-hybridized carbon atoms,πconjugate structure,and electron transmission capability,is a promising candidate as catalyst *** years have witnessed the rapid progress of GDY-supported metal catalysts for different catalysis *** that most processes in the current chemical industry are thermocatalytic reactions,we herein give an overview about the advances and particular characteristics of GDY-supported catalysts in these *** geometric structure and electronic properties of GDY are first ***,the synthesis methods for GDY-supported metal catalysts and their applications in thermocatalytic reactions are discussed,in which the effect of electronic interaction on catalytic performance is ***,the current challenges and future directions of GDY-supported metal catalysts for thermocatalysis are *** is expected that this review will enrich our understanding of the advances of GDY as a superior support for metal catalysts in thermocatalytic reactions.

关键词： graphdiyne metal catalysts thermocatalytic reactions heterogeneous catalysis

Design Strategies for Aqueous Zinc Metal Batteries with High Zinc Utilization: From Metal Anodes to Anode-Free Structures

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Nano-Micro Letters 2024年第4期16卷 305-349页

作者： Xianfu Zhang Long Zhang Xinyuan jia Wen song Yongchang Liu School of Materials Science and Engineering University of Science and Technology Beijing30 College RoadBeijing 100083People’s Republic of China Beijing Advanced Innovation Center for Materials Genome Engineering Institute for Advanced Materials and TechnologyState Key Laboratory for Advanced Metals and MaterialsUniversity of Science and Technology BeijingBeijing 100083People’s Republic of China

Aqueous zinc metal batteries(AZMBs)are promising candidates for next-generation energy storage due to the excellent safety, environmental friendliness, natural abundance, high theoretical specific capacity, and low redox potential of zinc(Zn) metal. However,several issues such as dendrite formation, hydrogen evolution, corrosion, and passivation of Zn metal anodes cause irreversible loss of the active materials. To solve these issues, researchers often use large amounts of excess Zn to ensure a continuous supply of active materials for Zn anodes. This leads to the ultralow utilization of Zn anodes and squanders the high energy density of AZMBs. Herein, the design strategies for AZMBs with high Zn utilization are discussed in depth, from utilizing thinner Zn foils to constructing anode-free structures with theoretical Zn utilization of 100%, which provides comprehensive guidelines for further research. Representative methods for calculating the depth of discharge of Zn anodes with different structures are first summarized. The reasonable modification strategies of Zn foil anodes, current collectors with pre-deposited Zn, and anode-free aqueous Zn metal batteries(AF-AZMBs) to improve Zn utilization are then detailed. In particular, the working mechanism of AF-AZMBs is systematically introduced. Finally, the challenges and perspectives for constructing high-utilization Zn anodes are presented.

关键词： Aqueous zinc metal batteries Zinc anodes High zinc utilization Depth of discharge Anode-free structures

Propagation Properties of Shock Waves in Polyurethane Foam based on Atomistic Simulations

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Defence Technology（防务技术） 2024年第1期31卷 117-129页

作者： Zhiqiang Hu jianli Shao Shiyu jia Weidong song Cheng Wang State Key Laboratory of Explosion Science and Technology Beijing Institute of TechnologyBeijing 100081China

Porous materials are widely used in the field of protection because of their excellent energy absorption *** this work,a series of polyurethane microscopic models are established and the effect of porosity on the shock waves is studied with classical molecular dynamics ***,shock Hugoniot relations for different porosities are obtained,which compare well with the experimental *** pores collapse and form local stress wave,which results in the complex multi-wave structure of the shock *** microstructure analysis shows that the local stress increases and the local velocity decreases gradually during the process of pore collapse to complete ***,it leads to stress relaxation and velocity *** shock stress peaks can be fitted with two exponential functions,and the amplitude of attenuation coefficient decreases with the increase of ***,the pore collapse under shock or non-shock are discussed by the entropy increase rate of the *** energy is dissipated mainly through the multiple interactions of the waves under *** energy is dissipated mainly by the friction between atoms under non-shock.

关键词： Polyurethane foam Shock wave Attenuation Atomistic simulation

Neutron irradiation influence on high-power thyristor device under fusion environment

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Nuclear Science and Techniques 2024年第4期35卷 65-81页

作者： Wei Tong Hua Li meng Xu Zhi-Quan song Bo Chen School of Electrical Engineering and Automation Hefei University of TechnologyHefei 230009China Institute of Plasma Physics Hefei Institutes of Physical ScienceChinese Academy of SciencesHefei 230031China University of Science and Technology of China Hefei 230026China

Because of their economy and applicability,high-power thyristor devices are widely used in the power supply systems for large fusion *** high-dose neutrons produced by deuterium–tritium(D–T)fusion reactions are irradiated on a thyristor device for a long time,the electrical characteristics of the device change,which may eventually cause irreversible *** this study,with the thyristor switch of the commutation circuit in the quench protection system(QPS)of a fusion device as the study object,the relationship between the internal physical structure and external electrical parameters of the irradiated thyristor is ***,a series of targeted thyristor physical simulations and neutron irradiation experiments are conducted to verify the accuracy of the theoretical *** addition,the effect of irradiated thyristor electrical characteristic changes on the entire QPS is studied by accurate simulation,providing valuable guidelines for the maintenance and renovation of the QPS.

关键词： Fusion device Neutron irradiation effects Thyristor Quench protection

Research on Data Tampering Prevention Method for ATC Network Based on Zero Trust

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Computers, Materials & Continua 2024年第3期78卷 4363-4377页

作者： Xiaoyan Zhu Ruchun jia Tingrui Zhang song Yao School of Computer Science Hefei University of TechnologyHefei230000China College of Computer Science Sichuan UniversityChengdu610065China Sichuan University-Pittsburgh Institute Sichuan UniversityChengdu610065China School of Information and Business Management Chengdu Neusoft UniversityChengdu610065China

The traditional air traffic control information sharing data has weak security characteristics of personal privacy data and poor effect,which is easy to leads to the problem that the data is *** from the application of the ATC(automatic train control)network,this paper focuses on the zero trust and zero trust access strategy and the tamper-proof method of information-sharing network *** the improvement of ATC’s zero trust physical layer authentication and network data distributed feature differentiation calculation,this paper reconstructs the personal privacy scope authentication structure and designs a tamper-proof method of ATC’s information sharing on the *** the single management authority to the unified management of data units,the systematic algorithm improvement of shared network data tamper prevention method is realized,and RDTP(Reliable Data Transfer Protocol)is selected in the network data of information sharing resources to realize the effectiveness of tamper prevention of air traffic control data during *** results show that this method can reasonably avoid the tampering of information sharing on the Internet,maintain the security factors of air traffic control information sharing on the Internet,and the Central Processing Unit(CPU)utilization rate is only 4.64%,which effectively increases the performance of air traffic control data comprehensive security protection system.

关键词： Zero trust access policy air traffic information sharing network privacy data tam-per-proof certification features

The influence of inter-band rock on rib spalling in longwall panel with large mining height

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International Journal of Mining Science and Technology 2024年第4期34卷 427-442页

作者： jiachen Wang meng Li Zhaohui Wang Zheng Li Han Zhang Shixiong song School of Energy and Mining Engineering China University of Mining and Technology-BeijingBeijing 100083China Coal Industry Engineering Research Center of Top-coal Caving Mining Beijing 100083China Faculty of Engineering Hokkaido UniversitySapporo 060-8628Japan

In order to improve rib stability,failure criteria and instability mode of a thick coal seam with inter-band rock layer are analysed in this study.A three-dimensional mechanical model is established for the rib by considering the rock layer.A safety factor is defined foy the rib,and it is observed that the safety factor exhibits a positive correlation with the thickness and strength of the inter-band rock.A calculation method for determining critical parameters of the rock layer is presented to ensure the rib *** is revealed that incomplete propagation of the fracture at the hard rock constitutes a fundamental prerequisite for ensuring the rib *** influence of the position of the inter-band rock in the coal seam on failure mechanism of the rib was thoroughly investigated by developing a series of physical models for the rib at the face *** best position for the inter-band rock in the coal seam is at a height of 1.5 m away from the roof line,which tends to provide a good stability state for the *** different inter-band rock positions,two ways of controlling rib by increasing supports stiffness and flexible grouting reinforcement are proposed.

关键词： Rib spalling Inter-band rock Large mining height Limit analysis Physical model experiment Numerical simulation

A star-like photodetector for angle-based light sensing in 3D space

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Nano Research 2024年第8期17卷 7567-7573页

作者： Qi Pan Sisi Chen Hongfei Xie Qiling Xu meng Su Yanlin song College of Chemistry Zhengzhou UniversityZhengzhou 450051China Key Laboratory of Green Printing CAS Research/Education Center for Excellence in Molecular SciencesInstitute of ChemistryChinese Academy of Sciences(ICCAS)Beijing Engineering Research Center of Nanomaterials for Green Printing TechnologyBeijing National Laboratory for Molecular Sciences(BNLMS)Beijing 100190China University of Chinese Academy of Sciences Beijing 100049China

The development of three-dimensional(3D)space light angle detection is vital in optical technology for applications such as 3D imaging,computer vision,and augmented *** methods involve advanced sensors and algorithms,including time-offlight cameras,which need multiple cameras and light sources to improve ***,it is a great challenge to integrate these complex components into compact *** semiconductor structures offer optical resonance characteristics,enabling precise light–matter interaction regulation.A 3D star-like photodetector,fabricated using a template assistant printing strategy,demonstrates optical resonances of the subwavelength facade and the shielding effect of spatial *** achieves light angle detection with the resolution of 10°in vertical space and the resolution of 36°in horizontal space,making it a promising prototype for various applications.

关键词： photodetector light angle detection printing subwavelength structure shielding effect