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Viral-mediated gene therapy in pediatric neurological disorders

World Journal of Pediatrics 2024年第6期20卷 533-555页

作者： Jing Peng Wei-Wei Zou Xiao-Lei Wang zhi-guo Zhang Ran Huo Li Yang Department of Pediatrics Clinical Research Center for Chidren Neurodevelopmental disablities of Hunan ProvinceXiangya HospitalCentral South UniversityChangsha 410008China Reproductive Medicine Center Department of Obstetrics and Gynecologythe First Affiliated Hospital of Anhui Medical UniversityHefeiChina State Key Laboratory of Reproductive Medicine Nanjing Medical UniversityNanjingChina

Background Due to the broad application of next-generation sequencing,the molecular diagnosis of genetic disorders in pediatric neurology is no longer an unachievable ***,treatments for neurological genetic disorders in children remain primarily *** the other hand,with the continuous evolution of therapeutic viral vectors,gene therapy is becoming a clinical *** this perspective,we wrote this review to illustrate the current state regarding viral-mediated gene therapy in childhood neurological *** sources We searched databases,including PubMed and Google Scholar,using the keywords"adenovirus vector,""lentivirus vector,"and"AAv"for gene therapy,and"immunoreaction induced by gene therapy vectors,""administration routes of gene therapy vectors,"and"gene therapy"with"NCL,""SMA,""DMD,""congenital myopathy,""MPS""leukodystrophy,"or"pediatric metabolic disorders".We also screened the database of *** using the keywords"gene therapy for children"and then filtered the results with the ones aimed at neurological *** time range of the search procedure was from the inception of the databases to the *** We presented the characteristics of commonly used viral vectors for gene therapy for pediatric neurological disorders and summarized their merits and drawbacks,the administration routes of each vector,the research progress,and the clinical application status of viral-mediated gene therapy on pediatric neurological *** Viral-mediated gene therapy is on the brink of broad clinical ***-mediated gene therapy will dramatically change the treatment pattern of childhood neurological disorders,and many children with incurable diseases will meet the dawn of a ***,the vectors must be optimized for better safety and efficacy.

关键词： Gene therapy Neurological disorders Pediatrics Viral vectors

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Research on the flow stability and noise reduction characteristics of quasi-periodic elastic support skin

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Defence Technology（防务技术） 2024年第3期33卷 222-236页

作者： Lu Chen Shao-gang Liu Dan Zhao Li-qiang Dong Kai Li Shuai Tang Jin Cui Hong guo Department of Mechanical and Electrical Engineering Harbin Engineering UniversityHarbin150001China

To enhance flow stability and reduce hydrodynamic noise caused by fluctuating pressure,a quasiperiodic elastic support skin composed of flexible walls and elastic support elements is proposed for fluid noise *** arrangement of the elastic support element is determined by the equivalent periodic distance and quasi-periodic *** this paper,a dynamic model of skin in a fluid environment is *** influence of equivalent periodic distance and quasi-periodic coefficient on flow stability is *** results suggest that arranging the elastic support elements in accordance with the quasi-periodic law can effectively enhance flow ***,the hydrodynamic noise calculation results demonstrate that the skin exhibits excellent noise reduction performance,with reductions of 10 dB in the streamwise direction,11 dB in the spanwise direction,and 10 dB in the normal *** results also demonstrate that the stability analysis method can serve as a diagnostic tool for flow fields and guide the design of noise reduction structures.

关键词： Flow stability Quasi-period Flexible wall Elastic support element Hydrodynamic noise

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Mismatch analysis of all-fiber coherent beam combiners based on the self-imaging effect

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High Power Laser Science and Engineering 2024年第2期12卷 1-15页

作者： Yuefang Yan Yu Liu Haoyu Zhang Yuwei Li Chao guo qiang Shu Wenhui Huang Feng Jing Rumao Tao Laser Fusion Research Center China Academy of Engineering PhysicsMianyangChina Key Laboratory of Particle&Radiation Imaging(Tsinghua University) Ministry of EducationBeijingChina

All-fiber coherent beam combiners based on the self-imaging effect can achieve a near-perfect single laser beam,which can provide a promising way to overcome the power limitation of a single-fiber *** of the key points is combining efficiency,which is determined by various mismatches during fabrication.A theoretical model has been built,and the mismatch error is analyzed numerically for the first *** mismatch errors have been numerically studied with the beam quality and combining efficiency being chosen as the evaluation *** tolerance of each mismatch error for causing 1%loss is calculated to guide the design of the beam *** simulation results are consistent with the experimental results,which show that the mismatch error of the square-core fiber is the main cause of the efficiency *** results can provide useful guidance for the fabrication of all-fiber coherent beam combiners.

关键词： all-fiber coherent beam combination mismatch analysis self-imaging

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Measurement of the high energyγ-rays from heavy ion reactions usingČerenkov detector

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Nuclear Science and Techniques 2024年第2期35卷 123-130页

作者： Da-Wei Si Yan Zhou Sheng Xiao zhi Qin Dong guo Yu-Hao Qin Yi-Jie Wang Bo-Yuan Zhang Bai-Ting Tian zhi-Gang Xiao Department of Physics Tsinghua UniversityBeijing 100084China

The energetic bremsstrahlung photons up to 100 MeV produced in heavy ion collisions can be used as a sensitive probe for short-range correlation in atomic nuclei. The energy of the γ-rays can be measured by collecting the Čerenkov light in the medium induced by the fast electrons generated in the Compton scattering or electromagnetic shower of the incident γray. Two types of detectors based on pure water and lead glass as sensitive materials were designed for this purpose. The γresponse and optical photon propagation in the detectors were simulated based on electromagnetic and optical processes in Geant4. The inherent energy resolutions of 0.022(4) + 0.51(2)∕E^(1/2)_(γ) for water and 0.0026(3) + 0.446(3)∕E^(1/2)_(γ) for lead glass were obtained. The geometry sizes of the lead glass and water were optimized to 30 cm × 30 cm × 30 cm and 60 cm × 60 cm ×120 cm, respectively, to detect high-energy γ-rays at 160 MeV. The Hough transform method was applied to reconstruct the direction of the incident γ-rays, providing the ability to experimentally distinguish the high-energy γ-rays produced in the reactions on the target from random background cosmic-ray muons.

关键词： Bremsstrahlungγ-rays Čerenkov Geant4 Energy resolution Direction reconstruction Hough transform

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Morphological Classification of Infrared Galaxies Based on WISE

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Research in Astronomy and Astrophysics 2024年第4期24卷 222-236页

作者： zhi-Ren Pan Bo Qiu Cui-Xiang Liu A-Li Luo Xia Jiang Xiao-Yu guo School of Electronic and Information Engineering Hebei University of TechnologyTianjin 300401China Key Laboratory of Optical Astronomy National Astronomical ObservatoriesChinese Academy of SciencesBeijing 100101China

This study introduces a novel convolutional neural network,the WISE Galaxy Classification Network(WGC),for classifying spiral and elliptical galaxies using Wide-field Infrared Survey Explorer(WISE)*** attains an accuracy of 89.03%,surpassing the combined use of K-means or SVM with the Color-Color method in more accurately identifying galaxy *** enhanced variant,WGC_mag,integrates magnitude parameters with image features,further boosting the accuracy to 89.89%.The research also delves into the criteria for galaxy classification,discovering that WGC primarily categorizes dust-rich images as elliptical galaxies,corresponding to their lower star formation rates,and classifies less dusty images as spiral *** paper explores the consistency and complementarity of WISE infrared images with SDSS optical images in galaxy morphology *** SDSS Galaxy Classification Network(SGC),trained on SDSS images,achieved an accuracy of 94.64%.The accuracy reached 99.30% when predictions from SGC and WGC were *** the complementarity of features in WISE and SDSS images,a novel variant of a classifier,namely the Multi-band Galaxy Morphology Integrated Classifier,has been *** classifier elevates the overall prediction accuracy to 95.39%.Lastly,the versatility of WGC was validated in other data *** the HyperLEDA data set,the distinction between elliptical galaxies and Sc,Scd and Sd spiral galaxies was most pronounced,achieving an accuracy of 90%,surpassing the classification results of the Galaxy Zoo 2 labeled WISE data *** research not only demonstrates the effectiveness of WISE images in galaxy morphology classification but also represents an attempt to integrate multi-band astronomical data to enhance understanding of galaxy structures and evolution.

关键词： methods:data analysis infrared:galaxies galaxies:spiral galaxies:elliptical and lenticular cD

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A review of linear friction welding of Ni -based superalloys

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International Journal of Minerals,Metallurgy and Materials 2024年第6期31卷 1382-1391页

作者： Xiawei Yang Tingxi Meng qiang Chu Yu Su Zhenguo guo Rui Xu Wenlong Fan Tiejun Ma Wenya Li State Key Laboratory of Solidification Processing Shaanxi Key Laboratory of Friction Welding TechnologiesNorthwestern Polytechnical UniversityXi’an 710072China Xi’an Aerospace Engine Co.Ltd. Xi’an 710100China

Ni-based superalloys are one of the most important materials employed in high-temperature applications within the aerospace and nuclear energy industries and in gas turbines due to their excellent corrosion,radiation,fatigue resistance,and high-temperature *** friction welding(LFW)is a new joining technology with near-net-forming characteristics that can be used for the manu-facture and repair of a wide range of aerospace *** paper reviews published works on LFW of Ni-based superalloys with the aim of understanding the characteristics of frictional heat generation and extrusion deformation,microstructures,mechanical proper-ties,flash morphology,residual stresses,creep,and fatigue of Ni-based superalloy weldments produced with LFW to enable future optim-um utilization of the LFW process.

关键词： Ni-based superalloys linear friction welding microstructures mechanical properties flash morphology

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Spatiotemporal variations in glacier area and surface velocity of the northern Antarctic Peninsula during 2018-2022

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Advances in Climate Change Research 2024年第2期15卷 297-311页

作者： Yu-Long KANG Shi-Chang KANG Wan-Qin guo Tao CHE Zong-Li JIANG Zhen-Feng WANG qiang-qiang XU Cheng-De YANG State Key Laboratory of Cryospheric Science Northwest Institute of Eco-Environment and ResourcesChinese Academy of SciencesLanzhou 730000China University of Chinese Academy of Sciences Beijing 100083China Key Laboratory of Remote Sensing of Gansu Province Northwest Institute of Eco-Environment and ResourcesChinese Academy of SciencesLanzhou 730000China School of Earth Sciences and Spatial Information Engineering Hunan University of Science and TechnologyXiangtan 411201China

Ice sheet serves as a crucial indicator for assessing climate *** loss in recent remote sensing-based studies indicated that the Antarctic Peninsula has rapid rates of glacier retreat and speed up of surface ***,observations of seasonal variability of ice speed are limited,and glacier-area changes require multi-temporal *** study investigated the changes in area and surface velocities of∼375 glaciers on the northern Antarctic Peninsula(NAP)utilizing satellite images acquired by the Sentinel 1&2 satellites during *** results indicate that the glacier area reduced by approximately 166.1±44.2 km^(2)(-0.2%±0.1%per year)during the study period,with an acceleration after 2020(-0.4%±0.3%per year),and the most dramatic reduction happened on the eastern *** maximum annual ice speeds on the NAP generally exceeded 3500 m per year,while the ice speeds in 2021 were the highest(exceeded 4210 m per year).The ice speed variability in austral autumn was higher than in other seasons,meanwhile the summer ice speeds showed an increasing *** glacier G012158E47018N,McNeile Glacier,glacier G299637E64094S and Drygalski Glacier showed the most remarkable ice speed variations represented by high daily velocities and strong fluctuations on their *** results demonstrated that the variations in glacier area and seasonal ice speed on the NAP were responsive to the ice-ocean-atmosphere ***,seasonal velocity and area variations should be considered when conducting accurate mass balance calculations,model validations and change mechanism analyses under climate warming scenarios.

关键词： Glacier area Seasonal ice speed Sentinel 1&2 Northern Antarctic Peninsula Ice-ocean-atmosphere processes

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Potential transformation of organic matter by microbes in cryoconite,Tibetan Plateau

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Science China Earth Sciences 2024年第6期67卷 1978-1989页

作者： Bixi guo Yongqin LIU Kevin Xu ZHONG Quan SHI Chen HE qiang ZHENG Ruanhong CAI State Key Laboratory of Tibetan Plateau Earth System Environment and Resources(TPESER)Institute of Tibetan Plateau ResearchChinese Academy of SciencesBeijing 100101China Center for the Pan-Third Pole Environment Lanzhou UniversityLanzhou 730000China Department of Earth Oceanand Atmospheric SciencesUniversity of British ColumbiaVancouverBC V6T 1Z4Canada State Key Laboratory of Heavy Oil Processing Petroleum Molecules Engineering Center(PMEC)China University of PetroleumBeijing 102249China State Key Laboratory for Marine Environmental Science Collage of Ocean and Earth SciencesXiamen UniversityXiamen 361102China Fujian Key Laboratory of Marine Carbon Sequestration Xiamen UniversityXiamen 361005China

Microorganisms play an essential role in the glacier carbon cycle;how they transform organic matter in mountain glacial cryoconite remains to be ***,we applied ultra-high resolution Fourier transform ion cyclotron resonance mass spectrometry(FT-ICR MS)and deep sequencing of 16S rRNA gene,to investigate the temporal microbial transformation of dissolved organic matter(DOM)of the Tibetan Plateau *** the 60-day incubation,we found that DOM in cryoconite underwent a three-stage transformation,with decreasing bioavailability over *** microbial community did not change much in the first week while degrading DOM molecules that were associated with higher H/C_(wa)and lower O/C_(wa).During days 15-30,DOM composition remained stable while microbial diversity *** day 60,the DOM was microbially converted into a higher state of recalcitrance,with higher values of aromatic index,O/C_(wa),and lower H/C_(wa),which contained molecules containing more *** among various microbial taxa,like Cyanobacteria,Bacteroidota,Gammaproteobacteria,Firmicutes,and Actinobacteriota,drove the DOM transformation in *** study sheds light on the in-situ transformation of DOM composition meditated by microbial populations in cryoconite at a temporal scale,providing new insights into understanding the microbial roles in the glacier organic carbon transformation.

关键词： Cryoconite Dissolved organic matter Biodegradation Microbial community Temporal variation

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A new two-step variational model for multiplicative noise removal with applications to texture images

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Applied Mathematics:A Journal of Chinese Universities 2024年第3期39卷 486-501页

作者： ZHANG Long-hui YAO Wen-juan SHI Sheng-zhu guo zhi-chang ZHANG Da-zhi School of Mathematics Harbin Institute of Technology

Multiplicative noise removal problems have attracted much attention in recent *** additive noise, multiplicative noise destroys almost all information of the original image,especially for texture images. Motivated by the TV-Stokes model, we propose a new two-step variational model to denoise the texture images corrupted by multiplicative noise with a good geometry explanation in this paper. In the first step, we convert the multiplicative denoising problem into an additive one by the logarithm transform and propagate the isophote directions in the tangential field smoothing. Once the isophote directions are constructed, an image is restored to fit the constructed directions in the second step. The existence and uniqueness of the solution to the variational problems are proved. In these two steps, we use the gradient descent method and construct finite difference schemes to solve the problems. Especially, the augmented Lagrangian method and the fast Fourier transform are adopted to accelerate the calculation. Experimental results show that the proposed model can remove the multiplicative noise efficiently and protect the texture well.

关键词： multiplicative noise removal texture images total variation two-step variational method aug-mented Lagrangian method

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Primary frequency control considering communication delay for grid-connected offshore wind power systems

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Global Energy Interconnection 2024年第3期7卷 241-253页

作者： Xueping Pan Qijie Xu Tao Xu Jinpeng guo Xiaorong Sun Yuquan Chen qiang Li Wei Liang School of Electrical and Power Engineering Hohai UniversityNanjing 211100P.R.China Electric Power Research Institute of State Grid Jiangsu Electric Power Co.Ltd Nanjing 211103P.R.China

Offshore wind farms are becoming increasingly distant from onshore centralized control centers,and the communication delays between them inevitably introduce time delays in the measurement signal of the primary frequency *** causes a deterioration in the performance of the primary frequency control and,in some cases,may even result in frequency instability within the power ***,a frequency response model that incorporates communication delays was established for power systems that integrate offshore wind *** Padéapproximation was used to model the time delays,and a linearized frequency response model of the power system was derived to investigate the frequency stability under different time *** influences of the wind power proportion and frequency control parameters on the system frequency stability were *** addition,a Smith delay compensation control strategy was devised to mitigate the effects of communication delays on the system frequency ***,a power system incorporating offshore wind power was constructed using the MATLAB/Simulink *** simulation results demonstrate the effectiveness and robustness of the proposed delay compensation control strategy.

关键词： Offshore wind power Primary frequency control Time delay Padéapproximation Time-delay compensation control

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