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WEB BASED TRANSLATION OF CHINESE ORGANIZATION NAME
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作者 Yang Muyun Liu Daxin +2 位作者 Zhao Tiejun Qi Haoliang Lin Kaiming 《Journal of Electronics(China)》 2009年第2期279-284,共6页
A web-based translation method for Chinese organization name is proposed.After ana-lyzing the structure of Chinese organization name,the methods of bilingual query formulation and maximum entropy based translation re-... A web-based translation method for Chinese organization name is proposed.After ana-lyzing the structure of Chinese organization name,the methods of bilingual query formulation and maximum entropy based translation re-ranking are suggested to retrieve the English translation from the web via public search engine.The experiments on Chinese university names demonstrate the validness of this approach. 展开更多
关键词 named entity Chinese organization name translation Bilingual query Web search
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Attention Markets of Blockchain-Based Decentralized Autonomous Organizations 被引量:1
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作者 Juanjuan Li Rui Qin +3 位作者 Sangtian Guan Wenwen Ding Fei Lin Fei-Yue Wang 《IEEE/CAA Journal of Automatica Sinica》 SCIE EI CSCD 2024年第6期1370-1380,共11页
The attention is a scarce resource in decentralized autonomous organizations(DAOs),as their self-governance relies heavily on the attention-intensive decision-making process of“proposal and voting”.To prevent the ne... The attention is a scarce resource in decentralized autonomous organizations(DAOs),as their self-governance relies heavily on the attention-intensive decision-making process of“proposal and voting”.To prevent the negative effects of pro-posers’attention-capturing strategies that contribute to the“tragedy of the commons”and ensure an efficient distribution of attention among multiple proposals,it is necessary to establish a market-driven allocation scheme for DAOs’attention.First,the Harberger tax-based attention markets are designed to facilitate its allocation via continuous and automated trading,where the individualized Harberger tax rate(HTR)determined by the pro-posers’reputation is adopted.Then,the Stackelberg game model is formulated in these markets,casting attention to owners in the role of leaders and other competitive proposers as followers.Its equilibrium trading strategies are also discussed to unravel the intricate dynamics of attention pricing.Moreover,utilizing the single-round Stackelberg game as an illustrative example,the existence of Nash equilibrium trading strategies is demonstrated.Finally,the impact of individualized HTR on trading strategies is investigated,and results suggest that it has a negative correlation with leaders’self-accessed prices and ownership duration,but its effect on their revenues varies under different conditions.This study is expected to provide valuable insights into leveraging attention resources to improve DAOs’governance and decision-making process. 展开更多
关键词 ATTENTION decentralized autonomous organizations Harberger tax Stackelberg game.
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Integrating Levels of Hierarchical Organization in Porous Organic Molecular Materials
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作者 Jesus Ferrando‑Soria Antonio Fernandez 《Nano-Micro Letters》 SCIE EI CAS CSCD 2024年第5期134-153,共20页
Porous organic molecular materials(POMMs)are an emergent class of molecular-based materials characterized by the formation of extended porous frameworks,mainly held by non-covalent interactions.POMMs represent a varie... Porous organic molecular materials(POMMs)are an emergent class of molecular-based materials characterized by the formation of extended porous frameworks,mainly held by non-covalent interactions.POMMs represent a variety of chemical families,such as hydrogen-bonded organic frameworks,porous organic salts,porous organic cages,C-H···πmicroporous crystals,supramolecular organic frameworks,π-organic frameworks,halogen-bonded organic framework,and intrinsically porous molecular materials.In some porous materials such as zeolites and metal organic frameworks,the integration of multiscale has been adopted to build materials with multifunctionality and optimized properties.Therefore,considering the significant role of hierarchy in porous materials and the growing importance of POMMs in the realm of synthetic porous materials,we consider it appropriate to dedicate for the first time a critical review covering both topics.Herein,we will provide a summary of literature examples showcasing hierarchical POMMs,with a focus on their main synthetic approaches,applications,and the advantages brought forth by introducing hierarchy. 展开更多
关键词 Porous organic molecular materials HIERARCHY Hydrogen-bonded organic frameworks Porous cages FULLERENE
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3D genome organization and its study in livestock breeding
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作者 Jie Cheng Xiukai Cao +7 位作者 Shengxuan Wang Jiaqiang Zhang Binglin Yue Xiaoyan Zhang Yongzhen Huang Xianyong Lan Gang Ren Hong Chen 《Journal of Integrative Agriculture》 SCIE CSCD 2024年第1期39-58,共20页
Eukaryotic genomes are hierarchically packaged into cell nucleus,affecting gene regulation.The genome is organized into multiscale structural units,including chromosome territories,compartments,topologically associati... Eukaryotic genomes are hierarchically packaged into cell nucleus,affecting gene regulation.The genome is organized into multiscale structural units,including chromosome territories,compartments,topologically associating domains(TADs),and DNA loops.The identification of these hierarchical structures has benefited from the development of experimental approaches,such as 3C-based methods(Hi-C,ChIA-PET,etc.),imaging tools(2D-FISH,3D-FISH,Cryo-FISH,etc.)and ligation-free methods(GAM,SPRITE,etc.).In recent two decades,numerous studies have shown that the 3D organization of genome plays essential roles in multiple cellular processes via various mechanisms,such as regulating enhancer activity and promoter-enhancer interactions.However,there are relatively few studies about the 3D genome in livestock species.Therefore,studies for exploring the function of 3D genomes in livestock are urgently needed to provide a more comprehensive understanding of potential relationships between the genome and production traits.In this review,we summarize the recent advances of 3D genomics and its biological functions in human and mouse studies,drawing inspiration to explore the 3D genomics of livestock species.We then mainly focus on the biological functions of 3D genome organization in muscle development and its implications in animal breeding. 展开更多
关键词 3D genome organization 3D genomic methodology regulatory mechanisms muscle development livestock breeding
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Industrial Organization in the Digital Economy Era:Evolution and Effects
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作者 Guo Chaoxian Hui Wei 《China Economist》 2024年第4期15-36,共22页
Advances in digital technology and vast data applications have resulted in the emergence of the digital economy paradigm,which is a new techno-economic paradigm for the digital economy age.Under this paradigm,the indu... Advances in digital technology and vast data applications have resulted in the emergence of the digital economy paradigm,which is a new techno-economic paradigm for the digital economy age.Under this paradigm,the industrial organization has shifted toward networked,platform-based,boundaryless,and integrated development with new characteristics.Specifically,there has been a fierce“positional arms race”fueled by financial capital,accelerating the growth of platform companies.The“hierarchical monopolistic competition”market structure has taken shape,resulting in a“coexistence without disruption”monopolistic competition.As platform businesses expand into new business sectors,competition among industrial ecosystems intensifies.Data and algorithms have become secret weapons for corporate success,allowing platform companies to expand their monopolistic forces.Industrial organization in the digital era has revolutionized the operating rules and logic of industrial economy,giving rise to new industries,business models,and paradigms,as well as being conducive to international cooperation and intelligent regulation.Meanwhile,it has introduced new challenges to socioeconomic development,making platform companies’anti-competitive and monopolistic behaviors more implicit,privacy and security concerns more conspicuous,and antitrust identification and investigation more difficult.The government should embrace the evolving trends of industrial organization in the digital economy era,accelerate regulatory transition,and strengthen governance and regulation under the digital economy paradigm. 展开更多
关键词 Digital economy evolution of industrial organization platform companies governance and regulation
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Auxiliary Software for Defining the Parameters of the Structural Organization of a Complex System
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作者 Branislav M. Savic 《Journal of Software Engineering and Applications》 2024年第2期109-128,共20页
The developed auxiliary software serves to simplify, standardize and facilitate the software loading of the structural organization of a complex technological system, as well as its further manipulation within the pro... The developed auxiliary software serves to simplify, standardize and facilitate the software loading of the structural organization of a complex technological system, as well as its further manipulation within the process of solving the considered technological system. Its help can be especially useful in the case of a complex structural organization of a technological system with a large number of different functional elements grouped into several technological subsystems. This paper presents the results of its application for a special complex technological system related to the reference steam block for the combined production of heat and electricity. 展开更多
关键词 Complex System Structural organization Auxiliary Software PARAMETERS
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An Exploration of Organizational Development in the Process of Innovation and Transformation of Pharmaceutical Enterprises-Taking X Pharmaceutical Enterprise as an Example
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作者 Gao Lin Sun Lihua 《Asian Journal of Social Pharmacy》 2024年第1期72-80,共9页
Objective To study how to promote the smooth innovation and transformation of enterprises and enhance the market competitiveness of enterprises from the perspective of human resources organization development.In the w... Objective To study how to promote the smooth innovation and transformation of enterprises and enhance the market competitiveness of enterprises from the perspective of human resources organization development.In the wave of pharmaceutical industry reform,some entrepreneurs realize the importance of innovation and technology in the future.However,domestic pharmaceutical enterprises are still in the production-oriented stage.In the process of transformative innovation,pharmaceutical enterprises should not only rely on the support of scientific and technological and economic forces,but also need a suitable organizational change and many excellent talents as the source of development.Methods Through retrieving relevant literature and combined with the organizational development practice of X pharmaceutical enterprise,the organizational development model was explored in this paper.Results and Conclusion By forming a system of strategy,organization,talent,culture and mechanism,and building an organizational development model,we can improve the organizational atmosphere and organizational efficiency in the process of enterprise innovation and transformation,so as to enhance the competitiveness of enterprises in the market. 展开更多
关键词 innovation transformation organization development organizational culture personnel training
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A Semantic-Sensitive Approach to Indoor and Outdoor 3D Data Organization
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作者 Youchen Wei 《Journal of World Architecture》 2024年第1期1-6,共6页
Building model data organization is often programmed to solve a specific problem,resulting in the inability to organize indoor and outdoor 3D scenes in an integrated manner.In this paper,existing building spatial data... Building model data organization is often programmed to solve a specific problem,resulting in the inability to organize indoor and outdoor 3D scenes in an integrated manner.In this paper,existing building spatial data models are studied,and the characteristics of building information modeling standards(IFC),city geographic modeling language(CityGML),indoor modeling language(IndoorGML),and other models are compared and analyzed.CityGML and IndoorGML models face challenges in satisfying diverse application scenarios and requirements due to limitations in their expression capabilities.It is proposed to combine the semantic information of the model objects to effectively partition and organize the indoor and outdoor spatial 3D model data and to construct the indoor and outdoor data organization mechanism of“chunk-layer-subobject-entrances-area-detail object.”This method is verified by proposing a 3D data organization method for indoor and outdoor space and constructing a 3D visualization system based on it. 展开更多
关键词 Integrated data organization Indoor and outdoor 3D data models Semantic models Spatial segmentation
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Exploration of Construction Organization for Maintenance of Steam Turbine Equipment in Thermal Power Plants
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作者 Zhu Yang 《Journal of Electronic Research and Application》 2024年第4期148-153,共6页
The continuous development of the power industry has had a positive impact on thermal power plants,helping them maintain a good production form.In the use of steam turbine equipment in thermal power plants,to prolong ... The continuous development of the power industry has had a positive impact on thermal power plants,helping them maintain a good production form.In the use of steam turbine equipment in thermal power plants,to prolong its lifespan and avoid safety hazards,it is necessary to pay attention to strengthening maintenance and construction organization,better implementing effective organizational work,and effectively applying steam turbine equipment to ensure the sustainable development of thermal power plants.This article discusses the concept of equipment maintenance from the perspective of steam turbine equipment in thermal power plants,analyzes the current situation of equipment maintenance,and proposes a specific construction organization to provide a reference for steam turbine equipment maintenance. 展开更多
关键词 Thermal power plant Steam turbine equipment Construction organization
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Molecular Cloning, Escherichia coli Expression and Genomic Organization of Squalene Synthase Gene from Artemisia annua 被引量:5
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作者 刘彦 叶和春 +1 位作者 王红 李国凤 《Acta Botanica Sinica》 CSCD 2003年第5期608-613,共6页
A 1 539 by squalene synthase (AaSQS) cDNA was cloned from a high-yield Artemisia annua L. strain 001 by reverse transcription-polymerise chain reaction (RT-PCR). The amino acid sequence of AaSQS is 70%, 77%, 44% and 3... A 1 539 by squalene synthase (AaSQS) cDNA was cloned from a high-yield Artemisia annua L. strain 001 by reverse transcription-polymerise chain reaction (RT-PCR). The amino acid sequence of AaSQS is 70%, 77%, 44% and 39%a identical to that of squalene synthases from Arabidopsis thaliana, tobacco, human and yeast, respectively. The AaSQS genomic DNA has a complex organization containing 14 exons and 13 introns. Full-length or C-terminal truncated cDNA was subcloned into prokaryotic expression vector pET30a and the constructed plasmid was introduced to Escherichia coli strain BL21 (DE3) for induced overexpression. No squalene synthase protein with expected molecular mass was observed in E. cola containing the putative full-length squalene synthase cDNA, however, overexpression in E. coli was achieved by truncating 30 amino acids of hydrophobic region at the carboxy terminus. 展开更多
关键词 Artemisia annua squalene synthase Escherachia coli overexpression genomic organization CLONING
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Complete Sequence and Gene Organization of the Mitochondrial Genome of Tokay (Gekko gecko) 被引量:8
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作者 韩德民 周开亚 《Zoological Research》 CAS CSCD 北大核心 2005年第2期123-128,共6页
Long-PCR amplification, clone and primer-walking sequencing methods were employed in determine the complete sequence of mitochondrial genome of tokay (Gekko gecko). The genome is 16 435 bp in size, contains 13 protein... Long-PCR amplification, clone and primer-walking sequencing methods were employed in determine the complete sequence of mitochondrial genome of tokay (Gekko gecko). The genome is 16 435 bp in size, contains 13 protein-coding, 2 ribosomal and 22 transfer RNA genes. The mt genome of Gekko is similar to most of the vertebrates in gene components, order, orientation, tRNA structures, low percentage of guanine and high percentage of thymine, and skews of base GC and AT. Base A was preferred at third codon positions for protein genes is similar to amphibians and fishes rather than amnion vertebrates. The standard stop codes (TAA) present only in three protein genes, less than those of most vertebrates. Transfer RNA genes range in length from 63 to 76 nt, their planar structure present characteristic clover leaf, except for tRNA-Cys and tRNA-Ser (AGY) because of lacking the D arm. 展开更多
关键词 Gekko gecko SQUAMATE Complete sequence of mitochondrial genome Gene organization
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Characteristics and Functions of Cooperative Economic Organizations for Water-saving Irrigation in Agricultural Development in Arid Areas 被引量:4
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作者 吴开波 朱美玲 +1 位作者 董新光 李金 《Agricultural Science & Technology》 CAS 2011年第12期1979-1982,共4页
Agricultural cooperative economic organization for water-saving irrigation in arid areas is a new form of economic organization in production,operation and management during the application process of water-saving irr... Agricultural cooperative economic organization for water-saving irrigation in arid areas is a new form of economic organization in production,operation and management during the application process of water-saving irrigation technologies.Currently,there are few researches on this cooperative economic organization.In this study,connotations of cooperative economic organizations for water-saving irrigation are specifically defined,and the characteristics and functions of this cooperative economic organization are analyzed.Based on that,several suggestions are proposed on the continuous development of cooperative economic organizations for water-saving irrigation. 展开更多
关键词 Cooperative economic organizations Water-saving irrigation CHARACTERISTICS FUNCTIONS
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COUPLING MODEL OF EXTENDED MANUFACTURING ORGANIZATION AND ITS APPLICATION 被引量:1
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作者 郭宇 安波 廖文和 《Transactions of Nanjing University of Aeronautics and Astronautics》 EI 2011年第1期137-144,共8页
For the feature of complex weapon manufacturing on internet,a coupling model is proposed.By using the model,the correlation between manufacturing cells in an extended manufacturing organization can be evaluated quanti... For the feature of complex weapon manufacturing on internet,a coupling model is proposed.By using the model,the correlation between manufacturing cells in an extended manufacturing organization can be evaluated quantitatively,so an appropriate control plan is determined.A strategy to improve and reduce the coupling relationship of the organization is studied.A correlation matrix of extended tasks is built to analyze the relationship between sub-tasks and manufacturing resources.An optimization method for manufacturing resource configuration is presented based on the coupling model.Finally,a software system for analyzing coupling model about manufacturing organization on internet is developed,and the result shows that the coupling model is effective. 展开更多
关键词 networked manufacturing manufacturing organization correlation matrix coupling model
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Multilevel analysis of the central-peripheral-target organ pathway:contributing to recovery after peripheral nerve injury
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作者 Xizi Song Ruixin Li +6 位作者 Xiaolei Chu Qi Li Ruihua Li Qingwen Li Kai-Yu Tong Xiaosong Gu Dong Ming 《Neural Regeneration Research》 SCIE CAS 2025年第10期2807-2822,共16页
Peripheral nerve injury is a common neurological condition that often leads to severe functional limitations and disabilities.Research on the pathogenesis of peripheral nerve injury has focused on pathological changes... Peripheral nerve injury is a common neurological condition that often leads to severe functional limitations and disabilities.Research on the pathogenesis of peripheral nerve injury has focused on pathological changes at individual injury sites,neglecting multilevel pathological analysis of the overall nervous system and target organs.This has led to restrictions on current therapeutic approaches.In this paper,we first summarize the potential mechanisms of peripheral nerve injury from a holistic perspective,covering the central nervous system,peripheral nervous system,and target organs.After peripheral nerve injury,the cortical plasticity of the brain is altered due to damage to and regeneration of peripheral nerves;changes such as neuronal apoptosis and axonal demyelination occur in the spinal cord.The nerve will undergo axonal regeneration,activation of Schwann cells,inflammatory response,and vascular system regeneration at the injury site.Corresponding damage to target organs can occur,including skeletal muscle atrophy and sensory receptor disruption.We then provide a brief review of the research advances in therapeutic approaches to peripheral nerve injury.The main current treatments are conducted passively and include physical factor rehabilitation,pharmacological treatments,cell-based therapies,and physical exercise.However,most treatments only partially address the problem and cannot complete the systematic recovery of the entire central nervous system-peripheral nervous system-target organ pathway.Therefore,we should further explore multilevel treatment options that produce effective,long-lasting results,perhaps requiring a combination of passive(traditional)and active(novel)treatment methods to stimulate rehabilitation at the central-peripheral-target organ levels to achieve better functional recovery. 展开更多
关键词 central nervous system central peripheral target organ multilevel pathological analysis nerve regeneration peripheral nerve injury peripheral nervous system target organs therapeutic approach
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Ultra‑Transparent and Multifunctional IZVO Mesh Electrodes for Next‑Generation Flexible Optoelectronics
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作者 Kiran A.Nirmal Tukaram D.Dongale +3 位作者 Atul C.Khot Chenjie Yao Nahyun Kim Tae Geun Kim 《Nano-Micro Letters》 SCIE EI CAS 2025年第1期293-309,共17页
Mechanically durable transparent electrodes are essential for achieving long-term stability in flexible optoelectronic devices.Furthermore,they are crucial for applications in the fields of energy,display,healthcare,a... Mechanically durable transparent electrodes are essential for achieving long-term stability in flexible optoelectronic devices.Furthermore,they are crucial for applications in the fields of energy,display,healthcare,and soft robotics.Conducting meshes represent a promising alternative to traditional,brittle,metal oxide conductors due to their high electrical conductivity,optical transparency,and enhanced mechanical flexibility.In this paper,we present a simple method for fabricating an ultra-transparent conducting metal oxide mesh electrode using selfcracking-assisted templates.Using this method,we produced an electrode with ultra-transparency(97.39%),high conductance(Rs=21.24Ωsq^(−1)),elevated work function(5.16 eV),and good mechanical stability.We also evaluated the effectiveness of the fabricated electrodes by integrating them into organic photovoltaics,organic light-emitting diodes,and flexible transparent memristor devices for neuromorphic computing,resulting in exceptional device performance.In addition,the unique porous structure of the vanadium-doped indium zinc oxide mesh electrodes provided excellent flexibility,rendering them a promising option for application in flexible optoelectronics. 展开更多
关键词 Self-cracking template Vanadium-doped indium zinc oxide mesh Organic solar cells Organic light-emitting diodes Flexible transparent memory
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Multi-level access control model for tree-like hierarchical organizations
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作者 於光灿 李瑞轩 +3 位作者 卢正鼎 Mudar Sarem 宋伟 苏永红 《Journal of Southeast University(English Edition)》 EI CAS 2008年第3期393-396,共4页
An access control model is proposed based on the famous Bell-LaPadula (BLP) model.In the proposed model,hierarchical relationships among departments are built,a new concept named post is proposed,and assigning secur... An access control model is proposed based on the famous Bell-LaPadula (BLP) model.In the proposed model,hierarchical relationships among departments are built,a new concept named post is proposed,and assigning security tags to subjects and objects is greatly simplified.The interoperation among different departments is implemented through assigning multiple security tags to one post, and the more departments are closed on the organization tree,the more secret objects can be exchanged by the staff of the departments.The access control matrices of the department,post and staff are defined.By using the three access control matrices,a multi granularity and flexible discretionary access control policy is implemented.The outstanding merit of the BLP model is inherited,and the new model can guarantee that all the information flow is under control.Finally,our study shows that compared to the BLP model,the proposed model is more flexible. 展开更多
关键词 multi-level access control hierarchical organization multiple security tags
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Genistein:a possible solution for the treatment of Alzheimer’s disease
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作者 Karolina Pierzynowska Bartosz Karaszewski Grzegorz Węgrzyn 《Neural Regeneration Research》 SCIE CAS 2025年第10期2903-2905,共3页
Neurodegenerative diseases are defined as disorders resulting from the slow and progressive loss of function and eventual death of neural cells that result from the primary pathology of nervous tissue.They can lead to... Neurodegenerative diseases are defined as disorders resulting from the slow and progressive loss of function and eventual death of neural cells that result from the primary pathology of nervous tissue.They can lead to severe nervous system dysfunction,eventually affecting multiple organs and systems.Several hundred neurodegenerative diseases have been described,and owing to their prevalence,severity. 展开更多
关键词 ORGANS ALZHEIMER DEATH
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Organization and storage model of marine information and its application in the "China Digital Ocean"
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作者 刘金 李昊倩 +2 位作者 朱吉才 姜晓轶 张峰 《Marine Science Bulletin》 CAS 2011年第2期83-96,共14页
Based on the experience and achievement of the"China Digital Ocean", the classification plan for Marine data elements is made, which can be classified into five, including marine point elements, marine line elements... Based on the experience and achievement of the"China Digital Ocean", the classification plan for Marine data elements is made, which can be classified into five, including marine point elements, marine line elements, marine polygon elements, marine grid elements and marine dynamic elements. In this paper, the technology of features and object-oriented method, a spatial-temporal data model is proposed, which can be applied in the large information system engineering like the "Digital Ocean", and this paper discusses the application of spatial data model, marine three-dimensional raster data model and relation data model in the building of Data Warehouse in "China Digital Ocean", and concludes the merits of these models. 展开更多
关键词 digital ocean sphere model data warehouse ocean elements data organization and storage
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Translational machinery and translation regulation in axon regeneration
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作者 Homaira Nawabi Stephane Belin 《Neural Regeneration Research》 SCIE CAS 2025年第5期1392-1394,共3页
Over the centuries,the regeneration field has been puzzled by the dual response of the central nervous system(CNS-brain,spinal cord,cranial nervesⅠandⅡ)and the peripheral nervous system(PNS that refers to all the ne... Over the centuries,the regeneration field has been puzzled by the dual response of the central nervous system(CNS-brain,spinal cord,cranial nervesⅠandⅡ)and the peripheral nervous system(PNS that refers to all the nerves that innervate muscles,skin,organs,bones among others).Even Ramon y Cajal had noticed that an injury to the PNS often leads to axon regrowth,in contrast to the CNS. 展开更多
关键词 NERVES ORGANS muscles
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Exploiting fly models to investigate rare human neurological disorders
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作者 Tomomi Tanaka Hyung-Lok Chung 《Neural Regeneration Research》 SCIE CAS 2025年第1期21-28,共8页
Rare neurological diseases,while individually are rare,collectively impact millions globally,leading to diverse and often severe neurological symptoms.Often attributed to genetic mutations that disrupt protein functio... Rare neurological diseases,while individually are rare,collectively impact millions globally,leading to diverse and often severe neurological symptoms.Often attributed to genetic mutations that disrupt protein function or structure,understanding their genetic basis is crucial for accurate diagnosis and targeted therapies.To investigate the underlying pathogenesis of these conditions,researchers often use non-mammalian model organisms,such as Drosophila(fruit flies),which is valued for their genetic manipulability,cost-efficiency,and preservation of genes and biological functions across evolutionary time.Genetic tools available in Drosophila,including CRISPR-Cas9,offer a means to manipulate gene expression,allowing for a deep exploration of the genetic underpinnings of rare neurological diseases.Drosophila boasts a versatile genetic toolkit,rapid generation turnover,and ease of large-scale experimentation,making it an invaluable resource for identifying potential drug candidates.Researchers can expose flies carrying disease-associated mutations to various compounds,rapidly pinpointing promising therapeutic agents for further investigation in mammalian models and,ultimately,clinical trials.In this comprehensive review,we explore rare neurological diseases where fly research has significantly contributed to our understanding of their genetic basis,pathophysiology,and potential therapeutic implications.We discuss rare diseases associated with both neuron-expressed and glial-expressed genes.Specific cases include mutations in CDK19 resulting in epilepsy and developmental delay,mutations in TIAM1 leading to a neurodevelopmental disorder with seizures and language delay,and mutations in IRF2BPL causing seizures,a neurodevelopmental disorder with regression,loss of speech,and abnormal movements.And we explore mutations in EMC1 related to cerebellar atrophy,visual impairment,psychomotor retardation,and gain-of-function mutations in ACOX1 causing Mitchell syndrome.Loss-of-function mutations in ACOX1 result in ACOX1 deficiency,characterized by very-long-chain fatty acid accumulation and glial degeneration.Notably,this review highlights how modeling these diseases in Drosophila has provided valuable insights into their pathophysiology,offering a platform for the rapid identification of potential therapeutic interventions.Rare neurological diseases involve a wide range of expression systems,and sometimes common phenotypes can be found among different genes that cause abnormalities in neurons or glia.Furthermore,mutations within the same gene may result in varying functional outcomes,such as complete loss of function,partial loss of function,or gain-of-function mutations.The phenotypes observed in patients can differ significantly,underscoring the complexity of these conditions.In conclusion,Drosophila represents an indispensable and cost-effective tool for investigating rare neurological diseases.By facilitating the modeling of these conditions,Drosophila contributes to a deeper understanding of their genetic basis,pathophysiology,and potential therapies.This approach accelerates the discovery of promising drug candidates,ultimately benefiting patients affected by these complex and understudied diseases. 展开更多
关键词 ACOX1 Drosophila melanogaster GLIA lipid metabolism model organisms NEUROINFLAMMATION neurologic disorders NEURON rare disease VLCFA
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