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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

How plants sense and respond to osmotic stress

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Journal of Integrative Plant Biology 2024年第3期66卷 394-423页

作者： Bo Yu Dai-Yin Chao Yang Zhao shanghai Center for Plant Stress Biology CAS Center for Excellence in Molecular Plant SciencesThe Chinese Academy of SciencesShanghai 200032China Key Laboratory of Plant Carbon Capture The Chinese Academy of SciencesShanghai 200032China National Key Laboratory of Plant Molecular Genetics CAS Center for Excellence in Molecular Plant SciencesInstitute of Plant Physiology and EcologyThe Chinese Academy of SciencesShanghai 200032China University of Chinese Academy of sciences Beijing 100049China

Drought is one of the most serious abiotic stresses to land *** sense and respond to drought stress to survive under water *** have studied how plants sense drought stress,or osmotic stress caused by drought,ever since Charles Darwin,and gradually obtained clues about osmotic stress sensing and signaling in *** stress is a physical stimulus that triggers many physiological changes at the cellular level,including changes in turgor,cell wall stiffness and integrity,membrane tension,and cell fluid volume,and plants may sense some of these stimuli and trigger downstream *** this review,we emphasized water potential and movements in organisms,compared putative signal inputs in cell wall-containing and cell wall-free organisms,prospected how plants sense changes in turgor,membrane tension,and cell fluid volume under osmotic stress according to advances in plants,animals,yeasts,and bacteria,summarized multilevel biochemical and physiological signal outputs,such as plasma membrane nanodomain formation,membrane water permeability,root hydrotropism,root halotropism,Casparian strip and suberin lamellae,and finally proposed a hypothesis that osmotic stress responses are likely to be a cocktail of signaling mediated by multiple *** also discussed the core scientific questions,provided perspective about the future directions in this field,and highlighted the importance of robust and smart root systems and efficient source-sink allocations for generating future high-yield stress-resistant crops and plants.

关键词： cell volume drought membrane tension osmotic stress turgor

Improvement in Tol2 transposon for efficient large-cargo capacity transgene applications in cultured cells and zebrafish(Danio rerio)

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Zoological Research 2024年第3期45卷 567-574页

作者： Peng-Cheng Wang Hao Deng Rang Xu Jiu-Lin Du Rongkun Tao Department of Pediatric Cardiology Xinhua HospitalShanghai Jiao Tong University School of MedicineShanghai 200092China Institute of Neuroscience State Key Laboratory of NeuroscienceCenter for Excellence in Brain Science and Intelligence TechnologyChinese Academy of SciencesShanghai 200031China Medical School Kunming University of Science and TechnologyKunmingYunnan 650500China Scientific Research Center Xinhua HospitalShanghai Jiao Tong University School of MedicineShanghai 200092China University of Chinese Academy of sciences Beijing 100049China School of Life science and technology ShanghaiTech UniversityShanghai 200031China

Most viruses and transposons serve as effective carriers for the introduction of foreign DNA up to 11 kb into vertebrate ***,their activity markedly diminishes with payloads exceeding 11 *** the payload capacity of transposons could facilitate more sophisticated cargo designs,improving the regulation of expression and minimizing mutagenic risks associated with molecular therapeutics,metabolic engineering,and transgenic animal *** this study,we improved the Tol2 transposon by increasing protein expression levels using a translational enhancer(QBI SP163,ST)and enhanced the nuclear targeting ability using the nuclear localization protein H2B(SHT).The modified Tol2 and ST transposon efficiently integrated large DNA cargos into human cell cultures(H1299),comparable to the well-established super PiggyBac ***,mRNA from ST and SHT showed a significant increase in transgene delivery efficiency of large DNA payloads(8 kb,14 kb,and 24 kb)into zebrafish(Danio rerio).This study presents a modified Tol2 transposon as an enhanced nonviral vector for the delivery of large DNA payloads in transgenic applications.

关键词： Zebrafish Tol2 transposase Transgene Large payload Synthetic biology

Memory Trace for Fear Extinction:Fragile yet Reinforceable

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Neuroscience Bulletin 2024年第6期40卷 777-794页

作者： Ying Liu Shuai Ye Xin-Ni Li Wei-Guang Li Department of Rehabilitation Medicine State Key Laboratory of Medical Neurobiology and Ministry of Education Frontiers Center for Brain ScienceHuashan HospitalInstitute for Translational Brain ResearchFudan UniversityShanghai200032China

Fear extinction is a biological process in which learned fear behavior diminishes without anticipated reinforcement,allowing the organism to re-adapt to ever-changing *** on the behavioral hypothesis that extinction is new learning and forms an extinction memory,this new memory is more readily forgettable than the original fear *** brain’s cellular and synaptic traces underpinning this inherently fragile yet reinforceable extinction memory remain *** questions are about the whereabouts of the engram neurons that emerged during extinction learning and how they constitute a dynamically evolving functional construct that works in concert to store and express the extinction *** this review,we discuss recent advances in the engram circuits and their neural connectivity plasticity for fear extinction,aiming to establish a conceptual framework for understanding the dynamic competition between fear and extinction memories in adaptive control of conditioned fear responses.

关键词： Fear extinction memory Memory trace Fear relapse Medial prefrontal cortex Basolateral amygdala Ventral hippocampus Insular cortex Synaptic adaptations

Interactions Between Extracellular Vesicles and Autophagy in Neuroimmune Disorders

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Neuroscience Bulletin 2024年第7期40卷 992-1006页

作者： Xiwen Ai Haojun Yu Yu Cai Yangtai Guan Department of Neurology Shanghai Jiao Tong University School of Medicine Affiliated Renji HospitalShanghai 200127China Department of Neurology Renaissance School of MedicineStony Brook UniversityStony BrookNY 11794USA

Neuroimmune disorders,such as multiple sclerosis,neuromyelitis optica spectrum disorder,myasthenia gravis,and Guillain–Barrésyndrome,are characterized by the dysfunction of both the immune system and the nervous *** evidence suggests that extracellular vesicles and autophagy are closely associated with the pathogenesis of these *** this review,we summarize the current understanding of the interactions between extracellular vesicles and autophagy in neuroimmune disorders and discuss their potential diagnostic and therapeutic *** we highlight the need for further research to fully understand the mechanisms underlying these disorders,and to develop new diagnostic and therapeutic strategies.

关键词： Autophagy Extracellular vesicles Neuroimmune disorders Multiple sclerosis Neuromyelitis optica spectrum disorder Myasthenia gravis Guillain-Barré syndrome

Sensing of membrane tensions:the pleiotropic functions of OSCA/TMEM63 mechanosensitive ion channels

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Journal of Genetics and Genomics 2024年第6期51卷 579-582页

作者： Bo Yu Alex Costa Yang Zhao shanghai Center for Plant Stress Biology CAS Center for Excellence in Molecular Plant SciencesChinese Academy of SciencesShanghai 200032China Key Laboratory of Plant Carbon Capture Chinese Academy of SciencesShanghai 200032China University of Chinese Academy of sciences Beijing 100049China Department of Biosciences University of MilanVia Celoria 26Milan 20133Italy

Organisms sense and respond to environmental and developmental signals to drive adaptive responses(Kefauver et al.,2020;Yu et al.,2024).These stimuli can be mainly divided into physical and chemical stimuli,and the physical stimuli include light,temperature,sound,vibration,pressure,stretch,shear stress,osmotic stress,and substrate *** of these physical stimuli trigger physical forces and alter membrane tension,which in turn activates mechanosensitive channels(MsCs)and converts the mechanical cues into biochemical signals.

关键词： deformation. convert mec

Minimizing interfacial energy losses in inverted perovskite solar cells by a dipolar stereochemical 2D perovskite interface

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Journal of Energy Chemistry 2024年第3期90卷 496-503,I0011页

作者： Junjie Qian Jingjing He Qihang Zhang Chenyue Zhu Shilin Chen Zhanpeng Wei Xuesong Leng Ziren Zhou Benben Shen Yu Peng Qiang Niu Shuang Yang Yu Hou Key Laboratory for Ultrafine Materials of Ministry of Education Shanghai Engineering Research Center of Hierarchical NanomaterialsSchool of Materials Science and EngineeringEast China University of Science and TechnologyShanghai 200237China National Enterprise technology Center Inner Mongolia Erdos Electric Power and Metallurgy Group Company LimitedOrdos 016064Inner MongoliaChina

Inverted perovskite solar cells(PSCs) have attracted broad research and industrial interest owing to their suppressed hysteresis,cost-effectiveness,and ***,the issue of non-radiative recombination losses at the n-type interface between the perovskite and fullerene has impeded further improvement of photovoltaic ***,we modify the n-type interface of FAPbI_(3) perovskite films by constructing a stereochemical two-dimensional(2D) perovskite interlayer,in which the organic cations comprise both pyridine and ammonium *** pyridine N donor can create stable bonding with the surface-uncoordinated Pb on the perovskite,thereby passivating the shallow-level defects and enhancing the air stability of the ***,the pyridine N donor also offers a positive polar interface to decrease the surface work function of the perovskite film,enabling n-type ***,we employ a p-i-n photovoltaic(PV) device with the positive dipole interlayer at perovskite/fullerene contact and achieve remarkable photoelectric conversion efficiency(PCE) of 22.0%.

关键词： Perovskite solar cells Dipole interlayer Stereochemistry Non-radiative recombination

Optical secret sharing by multi-step liquid crystal planar doublet panning and coupling

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CHINESE OPTICS LETTERS 2024年第8期22卷 080502-080502页

作者： Mo, Zhichang Chen, Yi Zhu, Xiangyu Zhang, Zhouhao Li, Dawei Shao, Jianda Zhao, Yuanan Wang, Jianguo Chinese Acad Sci Shanghai Inst Opt & Fine Mech Lab Thin Film Opt Shanghai 201800 Peoples R China Univ Chinese Acad Sci Ctr Mat Sci & Optoelect Engn Beijing 100049 Peoples R China Chinese Acad Sci Key Lab Mat High Power Laser Shanghai 201800 Peoples R China Henan Normal Univ Sch Phys Henan Key Lab Infrared Mat & Spectrum Measures & A Xinxiang 453007 Peoples R China Univ Chinese Acad Sci Hangzhou Inst Adv Study Hangzhou 310024 Peoples R China

The hologram, which is formed by phases coupled through cascade devices for secret information sharing, still carries a cracking risk. We propose a liquid crystal planar doublet as the information carrier, and new holograms generated by the new coupled phases when the relative displacements of the different liquid crystal layers change. The designed geometrical phases are generated by an optimized iterative restoration algorithm, and each holographic image formed by these phases is readable. This scheme achieves an increase in the capacity of the stored secret information and provides more misdirection, which is expected to have potential value in optical steganography and storage.

关键词： liquid crystal holography optical steganography displacement coupled geometrical phase PHASE FABRICATION

Thermally modulated photoelectronic synaptic behavior in HfS_(2)/VO_(2) heterostructure

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Rare Metals 2024年第8期43卷 3798-3809页

作者： Le Wang Lin Wang Xiao-Yun Ye Xiong-Hu Xu Li-Yan Shang Ya-Wei Li Jin-Zhong Zhang Liang-Qing Zhu Zhi-Gao Hu Technical Center for Multifunctional Magneto-Optical Spectroscopy(shanghai) Engineering Research Center of Nanophotonics&Advanced Instrument(Ministry of Education)Department of PhysicsSchool of Physics and Electronic ScienceEast China Normal UniversityShanghai 200241China Collaborative Innovation Center of Extreme Optics Shanxi UniversityTaiyuan 030006China

Neuromorphic computing is known for its efficient computational speed,low latency,and reduced power consumption,which is considered a pivotal technology to overcome the von Neumann *** synapses are an indispensable component of neuromorphic computational artificial neural *** guarantee effective and precise processing of optical signals,it must have a high responsivity,detectivity,and the ability to adapt to various ***,a synaptic transistor based on the HfS_(2)/VO_(2) heterojunction with a responsivity of8.6×10^(3) A·W^(-1)and a detectivity of 1.26×10^(14)Jones at405 nm laser was ***,the typical synaptic behavior was successfully simulated,including postsynaptic currents(PSCs),the transition from shortterm plasticity(STP)to long-term plasticity(LTP).When VO_(2) converts from the semiconductor state to the metal state,the HfS_(2)/VO_(2) heterojunction transforms into a Schottky heterojunction from a TypeⅡheterojunction with ***'s important,the heterojunction still exhibits excellent responsivity and detectivity,as well as stability of synaptic *** addition,the classical Pavlovian conditioning experiment is simulated under different laser intensity to study the brain's associative learning *** results demonstrate that the HfS_(2)/VO_(2) heterojunction synapse exhibits significant responsivity and detectivity and is adaptable to high-temperature environments,showing great potential for neuromorphic computational applic ations.

关键词： HfS_(2) VO_(2) Phase transition Photoelectric synapse Heterojunction