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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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Dielectric Resonator Magnetoelectric Dipole Arrays with Low Cross Polarization,Backward Radiation,and Mutual Coupling for MIMO Base Station Applications

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China Communications 2024年第6期21卷 290-304页

作者： Song Simin Da Yiran Qian Bingyi Huang Xiaoyu Chen Xiaoming li Yingsong Ahmed A.Kishk School of Information and Communications Engineering Xi’an Jiaotong UniversityXi’an 710049China Key Laboratory of Intelligent Computing and Signal Processing Ministry of Education Anhui UniversityHefei 230039China Department of Electrical and Computer Engineering Concordia UniversityMontrealQC H3G 1M8Canada

Dielectric resonator magnetoelectric dipole(DRMED)arrays with enhanced isolation,reduced cross-polarization,and backward radiation are proposed for base station(BS)*** proposed antenna comprises an elevated dielectric resonator antenna(DRA)on a small metal plate above a sizeable common ground *** DRA is designed in its T Eδ11 mode,acting like a magnetic *** surface current excited by the differential probes flowing on the small ground plane is equivalent to an electric *** these two equivalent dipoles are orthogonal,they have the magnetoelectric dipole characteristics with reduced backward ***,the small ground planes can be treated as decoupling structures to provide a neutralization path to cancel the original coupling path.A linearly-polarized 4-element prototype array was verified experimentally in previous ***,a dual-polarized DRMED antenna is presented to construct a 2-element and 4×4 array for BS *** investigate its MIMO performance,sophisticated multi-cell scenario simulations are carried *** using the proposed dualpolarized DRMED array,the cellular system capacityis improved by 118.6%compared to a conventional DRA *** significant MIMO system improvement is mainly due to the reduced backward radiation and,therefore,reduced inter-cell *** align well with the simulations.

关键词： backward radiation dielectric resonator antennas(DRAs) isolation magnetoelectric-dipole(ME-dipole)antennas

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MusicFace: Music-driven expressive singing face synthesis

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Computational Visual Media 2024年第1期10卷 119-136页

作者： Pengfei liu Wenjin Deng Hengda li Jintai Wang Yinglin Zheng Yiwei Ding Xiaohu Guo Ming Zeng School of Informatics Xiamen UniversityXiamen361000China Department of Computer Science The University of Texas at DallasRichardsonTexas75080-3021USA

It remains an interesting and challenging problem to synthesize a vivid and realistic singing face driven by music. In this paper, we present a method for this task with natural motions for the lips, facial expression, head pose, and eyes. Due to the coupling of mixed information for the human voice and backing music in common music audio signals, we design a decouple-and-fuse strategy to tackle the challenge. We first decompose the input music audio into a human voice stream and a backing music stream. Due to the implicit and complicated correlation between the two-stream input signals and the dynamics of the facial expressions, head motions, and eye states, we model their relationship with an attention scheme, where the effects of the two streams are fused seamlessly. Furthermore, to improve the expressivenes of the generated results, we decompose head movement generation in terms of speed and direction, and decompose eye state generation into short-term blinking and long-term eye closing, modeling them separately. We have also built a novel dataset, SingingFace, to support training and evaluation of models for this task, including future work on this topic. Extensive experiments and a user study show that our proposed method is capable of synthesizing vivid singing faces, qualitatively and quantitatively better than the prior state-of-the-art.

关键词： face synthesis singing music generative adversarial network

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The Static Stability Region of an Integrated Electricity-Gas System Considering Voltage and Gas Pressure

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Engineering 2024年第5期36卷 132-145页

作者： Yunfei Mu Zhibin liu Xiangwei Guo Hongjie Jia Kai Hou Xiaodan Yu Bofeng Luo Hairun li Key Laboratory of Smart Grid of Ministry of Education Tianjin UniversityTianjin 300072China State Grid Tianjin Electric Power Company Tianjin 300201China China Southern Power Grid Digital Grid Research Institute Co. Ltd.Guangzhou 510700China

In an integrated electricity-gas system(IEGS),load fluctuations affect not only the voltage in the power system but also the gas pressure in the natural gas *** static voltage stability region(SVSR)method is a tool for analyzing the overall static voltage stability in a power ***,in an IEGS,the SVSR boundary may be overly optimistic because the gas pressure may collapse before the voltage ***,the SVSR method cannot be directly applied to an *** this paper,the concept of the SVSR is extended to the IEGS-static stability region(SSR)while considering voltage and gas ***,criteria for static gas pressure stability in a natural gas system are proposed,based on the static voltage stability criteria in a power ***,the IEGS-SSR is defined as a set of active power injections that satisfies multi-energy flow(MEF)equations and static voltage and gas pressure stability constraints in the active power injection space of natural gas-fired generator units(NGUs).To determine the IEGSSSR,a continuation MEF(CMEF)method is employed to trace the boundary point in one specific NGU scheduling direction.A multidimensional hyperplane sampling method is also proposed to sample the NGU scheduling directions *** obtained boundary points are further used to form the IEGSSSR in three-dimensional(3D)space via a Delaunay triangulation hypersurface fitting ***,the numerical results of typical case studies are presented to demonstrate that the proposed method can effectively form the IEGS-SSR,providing a tool for IEGS online monitoring and dispatching.

关键词： Integrated electricity-gas system Static stability region Continuation multi-energy flow Multidimensional hyperplane sampling

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The effect of secondary phase on corrosion behaviors of the titanium–zirconium–molybdenum alloy

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Tungsten 2024年第2期6卷 342-354页

作者： Song-Wei Ge Ping Hu Jie Deng Shi-Lei li Hai-Rui Xing Jia-Yu Han Xing-Jiang Hua li Wang Jun-Zhou Yang Bo Jin Wen Zhang Kuai-She Wang School of Metallurgy Engineering Xi’an University of Architecture and TechnologyXi’an 710055China National and Local Joint Engineering Research Center for Functional Materials Processing Xi’an University of Architecture and TechnologyXi’an 710055China Western Xinxing Rare and Precious Metals Co. Ltd.Shangluo 726000China Northwest Institute for Non-Ferrous Metal Research Xi’an 710016China

As the range of applications for molybdenum and its alloys has expanded,the corrosive environment for molybdenum alloys has become more *** the past,the content of doping elements has been studied to investigate their infl uence on the corrosion performance of titanium–zirconium–molybdenum(TZM)*** this paper,it is considered that the second phase in the alloy is the main factor aff ecting the corrosion performance of TZM *** comparing the corrosion behavior of molybdenum metal and TZM alloy,the eff ect of the secondary phase on corrosion behaviors of TZM alloy has been *** results show that the second phase reduces the corrosion resistance of the TZM *** potential of the second phase is 73.7 mV higher than that of the Mo matrix,which contributes to the formation of microscopic electric *** the action of microscopic electric couples,pitting corrosion is preferentially formed at the interface between the second phase and the matrix,which accelerates the corrosion of the *** paper provides a theoretical basis for the application of TZM alloys in corrosive environments.

关键词： Secondary phase Electrochemical corrosion Titanium–zirconium–molybdenum(TZM) Galvanic corrosion Corrosion resistance

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Metagenomic analysis revealing the metabolic role of microbial communities in the free amino acid biosynthesis of Monascus rice vinegar during fermentation

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Food Science and Human Wellness 2024年第4期13卷 2317-2326页

作者： Hang Gao Jian Zhang li liu lijun Fu Yan Zhao Germán Mazza Xin Zhang Beijing Academy of Food Sciences 100068 BeijingChina Institute for Research and Development in Process Engineering Biotechnology and Alternative Energies(PROBIENCONICET-UNCo)8300 NeuquénArgentina

Free amino acid(FAA)is the important component of vinegar that infl uences quality perception and consumer *** is one of the major metabolites produced by microorganisms;however,the microbial metabolic network on FAA biosynthesis remains *** metagenomic analysis,this work aimed to elucidate the roles of microbes in FAA biosynthesis during Monascus rice vinegar *** profiles from functional analyses showed 14 dominant genera with high contributions to the metabolism *** metabolic network for FAA biosynthesis was then constructed,and the microbial distribution in different metabolic pathways was *** results revealed that 5 functional genera were closely involved in FAA *** study illuminated the metabolic roles of microorganisms in FAA biosynthesis and provided crucial insights into the functional attributes of microbiota in vinegar fermentation.

关键词： Monascus rice vinegar Metagenomic analysis Free amino acid synthesis Metabolic pathway Microbial distribution

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Weakly Supervised Object Localization with Background Suppression Erasing for Art Authentication and Copyright Protection

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Machine Intelligence Research 2024年第1期21卷 89-103页

作者： Chaojie Wu Mingyang li Ying Gao Xinyan Xie Wing W.Y.Ng Ahmad Musyafa School of Computer Science and Engineering South China University of TechnologyGuangzhou 511442China Guangdong Provincial Key Laboratory of Artificial Intelligence in Medical Image Analysis and Application Guangdong Provincial People's HospitalGuangzhou 510180China

The problem of art forgery and infringement is becoming increasingly prominent,since diverse self-media contents with all kinds of art pieces are released on the Internet every *** art paintings,object detection and localization provide an efficient and ef-fective means of art authentication and copyright ***,the acquisition of a precise detector requires large amounts of ex-pensive pixel-level *** alleviate this,we propose a novel weakly supervised object localization(WSOL)with background su-perposition erasing(BSE),which recognizes objects with inexpensive image-level ***,integrated adversarial erasing(IAE)for vanilla convolutional neural network(CNN)dropouts the most discriminative region by leveraging high-level semantic ***,a background suppression module(BSM)limits the activation area of the IAE to the object region through a self-guidance ***,in the inference phase,we utilize the refined importance map(RIM)of middle features to obtain class-agnostic loc-alization *** experiments are conducted on paintings,CUB-200-2011 and ILSVRC to validate the effectiveness of our BSE.

关键词： Weakly supervised object localization erasing method deep learning computer vision art authentication and copyright protection

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lncRNA ZNF593-AS inhibits cardiac hypertrophy and myocardial remodeling by upregulating Mfn2 expression

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Frontiers of Medicine 2024年第3期18卷 484-498页

作者： Xiang Nie Jiahui Fan Yanwen Wang Rong Xie Chen Chen Huaping li Dao Wen Wang Division of Cardiology Department of Internal MedicineTongji HospitalTongji Medical CollegeHuazhong University of Science and TechnologyWuhan430030China Hubei Key Laboratory of Genetics and Molecular Mechanisms of Cardiological Disorders Wuhan430030China

lncRNA ZNF593 antisense(ZNF593-AS)transcripts have been implicated in heart failure through the regulation of myocardial *** decreased transcriptional activity of ZNF593-AS has also been detected in cardiac ***,the function of ZNF593-AS in cardiac hypertrophy remains ***,we report that the expression of ZNF593-AS reduced in a mouse model of left ventricular hypertrophy and cardiomyocytes in response to treatment with the hypertrophic agonist phenylephrine(PE).In vivo,ZNF593-AS aggravated pressure overload–induced cardiac hypertrophy in knockout *** contrast,cardiomyocyte-specific transgenic mice(ZNF593-AS MHC-Tg)exhibited attenuated TAC-induced cardiac *** vitro,vector-based overexpression using murine or human ZNF593-AS alleviated PE-induced myocyte hypertrophy,whereas GapmeR-induced inhibition aggravated hypertrophic *** using RNA-seq and gene set enrichment analyses,we identified a link between ZNF593-AS and oxidative phosphorylation and found that mitofusin 2(Mfn2)is a direct target of ***593-AS exerts an antihypertrophic effect by upregulating Mfn2 expression and improving mitochondrial ***,it represents a promising therapeutic target for combating pathological cardiac remodeling.

关键词： lncRNA ZNF593-AS cardiac hypertrophy Mfn2 oxidative phosphorylation

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Functionalized selenium nanoparticles ameliorated acetaminophen-induced hepatotoxicity through synergistically triggering PKCδ/Nrf2 signaling pathway and inhibiting CYP 2E1

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Food Science and Human Wellness 2024年第2期13卷 932-945页

作者： Si Zou Yetao Gong Xiujie li Yanbin Wu Jinzhong Wu Jianguo Wu Ka-Hing Wong College of Pharmacy Fujian University of Traditional Chinese MedicineFuzhou 350122China Research Institute for Future Food The Hong Kong Polytechnic UniversityHong Kong 999077China Department of Food Science and Nutrition The Hong Kong Polytechnic UniversityHong Kong 999077China

Selenium nanoparticles(SeNPs)have been demonstrated potential for use in diseases associated with oxidative *** SeNPs with lower toxicity and higher biocompatibility could bring better therapeutic activity and clinical application ***,this work was conducted to investigate the protective effect of Pleurotus tuber-regium polysaccharide-protein complex funtionnalized SeNPs(PTR-SeNPs)against acetaminophen(APAP)-induced oxidative injure in HepG2 cells and C57BL/6J mouse *** elucidation of the underlying molecular mechanism,in particular their modulation of Nrf2 signaling pathway was also *** results showed that PTR-SeNPs could significantly ameliorate APAP-induced oxidative injury as evidenced by a range of biochemical analysis,histopathological examination and immunoblotting ***-SeNPs could hosphorylate and activate PKCδ,depress Keap1,and increase nuclear accumulation of Nrf2,resulting in upregulation of GCLC,GCLM,HO-1 and NQO-1 ***,PTR-SeNPs suppressed the biotransformation of APAP to generate intracellular ROS through CYP 2E1 inhibition,restoring the mitochondrial ***,the protective effect of PTR-SeNPs against APAP induced hepatotoxicity was weakened as Nrf2 was depleted in vivo,indicating the pivotal role of Nrf2 signaling pathway in PTR-SeNPs mediated hepatoprotective *** a potential hepatic protectant,PTR-SeNPs could serve as a new source of selenium supplement for health-promoting and biomedical applications.

关键词： PTR-SeNPs(polysaccharide-proteincomplex functionalized selenium nanoparticles) Acetaminophen-induced hepatotoxicity Nuclear factor erythroid 2-related factor 2 Cytochrome P450 enzyme 2E1 Mitochondria

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Natural evidence of coronaviral 2′-O-methyltransferase activity affecting viral pathogenesis via improved substrate RNA binding

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Signal Transduction and Targeted Therapy 2024年第6期9卷 2819-2834页

作者： Jikai Deng Shimin Yang Yingjian li Xue Tan Jiejie liu Yanying Yu Qiang Ding Chengpeng Fan Hongyun Wang Xianyin Chen Qianyun liu Xiao Guo Feiyu Gong li Zhou Yu Chen State Key Laboratory of Virology RNA InstituteCollege of Life Sciences and Frontier Science Center for Immunology and MetabolismWuhan UniversityWuhanChina School of Medicine Tsinghua UniversityBeijingChina School of Basic Medical Sciences Wuhan UniversityWuhanChina Animal Bio-Safety Level Ⅲ Laboratory/Institute for Vaccine Research Wuhan University School of MedicineWuhanChina

Previous studies through targeted mutagenesis of K-D-K-E motif have demonstrated that 2′-O-MTase activity is essential for efficient viral replication and immune ***,the K-D-K-E catalytic motif of 2′-O-MTase is highly conserved across numerous viruses,including flaviviruses,vaccinia viruses,coronaviruses,and extends even to ***,we observed a stronger 2′-O-MTase activity in SARS-CoV-2 compared to SARS-CoV,despite the presence of a consistently active catalytic *** further identified critical residues(Leu-36,Asn-138 and Ile-153)which served as determinants of discrepancy in 2′-O-MTase activity between SARS-CoV-2 and *** residues significantly enhanced the RNA binding affinity of 2′-O-MTase and boosted its versatility toward RNA *** interest,a triple substitution(Leu^(36)→Ile^(36),Asn^(138)→His^(138),Ile^(153)→Leu^(153),from SARS-CoV-2 to SARS-CoV)within nsp16 resulted in a proportional reduction in viral 2′-O-methylation and impaired viral ***,it led to a significant upregulation of type I interferon(IFN-I)and proinflammatory cytokines both in vitro and vivo,relying on the cooperative sensing of melanoma differentiation-associated protein 5(MDA5)and laboratory of genetics and physiology 2(LGP2).In conclusion,our findings demonstrated that alterations in residues other than K-D-K-E of 2′-O-MTase may affect viral replication and subsequently influence *** changes in nsp16 residues is crucial as it may aid in identifying and assessing future alteration in viral pathogenicity resulting from natural mutations occurring in nsp16.

关键词： pathogenesis impaired alterations

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