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Association of copy number variation in X chromosome-linked PNPLA4 with heterotaxy and congenital heart disease

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中华医学杂志(英文版) 2024年第15期137卷 2024 Jul 8页

作者： Gao Han Huang Xianghui Chen Weicheng Feng Zhiyu Zhao Zhengshan Li Ping Tan Chaozhong Wang Jinxin Zhuang Quannan Gao Yuan Min Shaojie Yao Qinyu Qian Maoxiang Ma Xiaojing Wu Feizhen Yan Weili Sheng Wei Huang Guoying Children’s Hospital of Fudan University Shanghai China Shanghai Key Laboratory of birth defects Shanghai China Fujian Key Laboratory of Neonatal Diseases Xiamen Children’s Hospital Xiamen Fujian China research Unit of Early Intervention of Genetically Related Childhood Cardiovascular Diseases Chinese Academy of Medical Sciences Shanghai China

Background: Heterotaxy (HTX) is a thoracoabdominal organ anomaly syndrome and commonly accompanied by congenital heart disease (CHD). The aim of this study was to analyze rare copy number variations (CNVs) in a HTX/CHD cohort and to examine the potential mechanisms contributing to HTX/***: Chromosome microarray analysis was used to identify rare CNVs in a cohort of 120 unrelated HTX/CHD patients, and available samples from parents were used to confirm the inheritance pattern. Potential candidate genes in CNVs region were prioritized via the DECIPHER database, andPNPLA4 was identified as the leading candidate gene. To validate, we generatedPNPLA4-overexpressing human induced pluripotent stem cell lines as well aspnpla4-overexpressing zebrafish model, followed by a series of transcriptomic, biochemical and cellular ***: Seventeen rare CNVs were identified in 15 of the 120 HTX/CHD patients (12.5%). Xp22.31 duplication was one of the inherited CNVs identified in this HTX/CHD cohort, andPNPLA4 in the Xp22.31 was a candidate gene associated with HTX/***4 is expressed in the lateral plate mesoderm, which is known to be critical for left/right embryonic patterning as well as cardiomyocyte differentiation, and in the neural crest cell lineage. Through a series ofin vivo andin vitro analyses at the molecular and cellular levels, we revealed that the biological function ofPNPLA4 is importantly involved in the primary cilia formation and function via its regulation of energy metabolism and mitochondria-mediated ATP ***: Our findings demonstrated a significant association between CNVs and HTX/CHD. Our data strongly suggested that an increased genetic dose ofPNPLA4 due to Xp22.31 duplication is a disease-causing risk factor for HTX/CHD.

关键词： PNPLA4 Copy number variations X-chromosome Heterotaxy Congenital heart diseases

Microstructure and properties of coarse-grained WC-10Co cemented carbides with different carbon contents during heat treatments

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Rare Metals 2024年第4期43卷 1786-1792页

作者： Yuan-Feng Xie Xing-Cheng Xie Zhong-Wu Li Rui-Jun Cao Zhong-Kun Lin Qing Li Chen-Guang Lin Powder Metallurgy and Special Materials research Department General Research Institute for Nonferrous MetalsBeijing100088China

The effects of cryogenic treatment(CT)and tempering-cryogenic treatment(TCT)on the microstructure and properties of coarse-grained WC-10Co cemented carbides with different carbon contents were *** binder phase,WC mean grain sizes,W solubility in the binder,relative magnetic s aturation,densities,hardness,wear resistance and second phase precipitation of cemented carbides with different heat treatments were *** results show that there are significant changes of microstructure and properties in the samples with CT and TCT,especially due to the precipitation of metastable nanoparticles W_(x)Co_(y)C_(z) in the binder during the heat treatments of CT and *** the simultaneous combination of microstructure and nanoparticle-reinforced binder,a dramatically improved combination of hardness and wear resistance of the samples after TCT has been achieved.

关键词： Cryogenic treatment Tempering Coarsegrained cemented carbide Carbon content Nanoparticlereinforced binder

Functional Optical Fiber Sensors Detecting Imperceptible Physical/Chemical Changes for Smart Batteries

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Nano-Micro Letters 2024年第8期16卷 268-308页

作者： Yiding Li Li Wang Youzhi Song Wenwei Wang Cheng Lin Xiangming He institute of Nuclear and New Energy Technology Tsinghua UniversityBeijing 100084People’s Republic of China National Engineering research Center of Electric Vehicles Beijing Institute of Technology(BIT)Beijing 100081People’s Republic of China Shenzhen Automotive research institute of BIT(Shenzhen research institute of National Engineering research Center of Electric Vehicles) Shenzhen 518118People’s Republic of China

The battery technology progress has been a contradictory process in which performance improvement and hidden risks *** the battery is still a“black box”,thus requiring a deep understanding of its internal *** battery should“sense its internal physical/chemical conditions”,which puts strict requirements on embedded sensing *** paper summarizes the application of advanced optical fiber sensors in lithium-ion batteries and energy storage technologies that may be mass deployed,focuses on the insights of advanced optical fiber sensors into the processes of one-dimensional nano-micro-level battery material structural phase transition,electrolyte degradation,electrode-electrolyte interface dynamics to three-dimensional macro-safety *** paper contributes to understanding how to use optical fiber sensors to achieve“real”and“embedded”*** the inherent advantages of the advanced optical fiber sensor,it helps clarify the battery internal state and reaction mechanism,aiding in the establishment of more detailed *** advancements can promote the development of smart batteries,with significant importance lying in essentially promoting the improvement of system ***,with the help of smart batteries in the future,the importance of consistency can be weakened or even *** application of advanced optical fiber sensors helps comprehensively improve the battery quality,reliability,and life.

关键词： Smart battery Advanced embedded optical fiber sensor Battery internal physical/chemical state Quality-reliability-life characteristic

Dynamic characteristics of nonlinear vibration isolator for gas turbine

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International Journal of Mechanical System Dynamics 2024年第3期4卷 374-383页

作者： Teng Wang Linhan Feng Lei Zhang Chunhui Zhang Yue Wu Naval research institute BeijingChina

In this study,a theoretical model of the vibration isolation system of the gas turbine is developed and numerically solved.A simplified finite element(FE)model was also established to determine the response under the shock *** results of the FE model are used to verify the effectiveness of the theoretical model and the numerical *** influence of isolator stiffness,vibration isolator damping,and vibration isolator nonlinear stiffness coefficient on the shock response of the vibration isolation system is studied using the controlled-variable *** parameters(stiffness,damping,and nonlinear coefficient)enter into the shock resistance design of gas turbine vibration isolators.

关键词： gas turbine vibration isolator theoretical model damping dynamic characteristics

Weatherability and heat resistance enhanced by interaction between AG25 and Mg/AI-LDH

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Rare Metals 2024年第6期43卷 2758-2768页

作者： Pei-Yao Yang Xiang-Cui Liu Yu-Chao Wang Huan-Ran Zheng Jin-Xian Wang Jia-Yi Zheng Meng-Jie Liu Dan-Ni Deng Yu Bai Ying-Bi Chen Tong Zhang Zhi-Ming Liu Yong-Peng Lei State Key Laboratory of Powder Metallurgy Powder Metallurgy Research InstituteCentral South UniversityChangsha 410083China research institute of Chemical Defense Beijing 102205China

Although inorganic pigments in common spectral tuning materials show good weatherability and heat resistance,the limited color choices,weak coloring power,poor dispersibility,and a possibility of toxicity limit their *** the basis of organic pigments which possess a wide range of colors,high coloring power,good transparency,and high safety,herein,the modified pigment and biomimetic coating with improved weatherability,especially ultraviolet(UV)resistance(from 2 to 6 days),was achieved by intercalating acid green 25(AG25)pigment into Mg/Al-layered double hydroxides(Mg/Al-LDH).Furthermore,the heat resistance of AG25 was also significantly ***,the spectral stability of pigments after heat treatment is superior with almost unchanged spectral profile and green reflection *** formation of strong N-H bonds and the S-M(Mg,Al)bonds between Mg/Al-LDH laminates and AG25 molecules contributes to the *** work shows potential for biomimetic leaf materials in respect of reflective spectra stability.

关键词： Layered double hydroxide Biomimetic Acid green 25 Interaction Weatherability Heat resistance

Roles of neuronal lysosomes in the etiology of Parkinson’s disease

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Neural Regeneration research 2024年第9期19卷 1981-1983页

作者： Mattia Volta institute for Biomedicine Eurac ResearchBolzanoItaly

Therapeutic progress in neurodegenerative conditions such as Parkinson’s disease has been hampered by a lack of detailed knowledge of its molecular *** advancements in genetics and genomics have provided fundamental insights into specific protein players and the cellular processes involved in the onset of *** this respect,the autophagy-lysosome system has emerged in recent years as a strong point of convergence for genetics,genomics,and pathologic indications,spanning both familial and idiopathic Parkinson’s ***,if not all,genes linked to familial disease are involved,in a regulatory capacity,in lysosome function(e.g.,LRRK2,alpha-synuclein,VPS35,Parkin,and PINK1).Moreover,the majority of genomic loci associated with increased risk of idiopathic Parkinson’s cluster in lysosome biology and regulation(GBA as the prime example).Lastly,neuropathologic evidence showed alterations in lysosome markers in autoptic material that,coupled to the alpha-synuclein proteinopathy that defines the disease,strongly indicate an alteration in *** this Brief Review article,I present a personal perspective on the molecular and cellular involvement of lysosome biology in Parkinson’s pathogenesis,aiming at a larger vision on the events underlying the onset of the *** attempts at targeting autophagy for therapeutic purposes in Parkinson’s have been mostly aimed at“indiscriminately”enhancing its activity to promote the degradation and elimination of aggregate protein accumulations,such as alpha-synuclein Lewy ***,this approach is based on the assumption that protein pathology is the root cause of disease,while pre-pathology and pre-degeneration dysfunctions have been largely observed in clinical and pre-clinical *** addition,it has been reported that unspecific boosting of autophagy can be ***,it is important to understand the mechanisms of specific autophagy forms and,even more,the adjustment of specific lysosome f

关键词： alpha-synuclein autophagy LRRK2 lysosome neuroprotection neurotransmission Parkinson’s disease Rit2 synapse

Comparative study between three carbonaceous nanoblades and nanodarts for antimicrobial applications

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Journal of Environmental Sciences 2024年第2期136卷 594-605页

作者： Mohamed S.Selim Ahmed M.Azzam Mohamed A.Shenashen Shimaa A.Higazy Bayaumy B.Mostafa Sherif A.El-Safty National institute for Materials Science(NIMS) 1-2-1 SengenTsukuba-ShiIbaraki-Ken 305-0047Japan Petroleum Application Department Egyptian Petroleum Research InstituteNasr City 11727Egypt Department of Environmental research Theodor Bilharz Research InstituteGizaEgypt

The design of nanostructured materials occupies a privileged position in the development and management of affordable and effective technology in the antibacterial ***,we discuss the antimicrobial properties of three carbonaceous nanoblades and nanodarts materials of graphene oxide(GO),reduced graphene oxide(RGO),and single-wall carbon nanotubes(SWCNTs)that have a mechano-bactericidal effect,and the ability to piercing or slicing bacterial *** demonstrate the significance of size,morphology and composition on the antibacterial activity mechanism,the designed nanomaterials have been *** minimum inhibitory concentration(MIC),standard agar well diffusion,and transmission electron microscopy were utilized to evaluate the antibacterial activity of GO,RGO,and *** on the evidence obtained,the three carbonaceous materials exhibit activity against all microbial strains tested by completely encapsulating bacterial cells and causing morphological disruption by degrading the microbial cell membrane in the order of RGO>GO>*** of the external cell wall structure and outer membrane proteins,the synthesized carbonaceous nanomaterials exhibited higher antibacterial activity against Gram-positive bacterial strains than Gram-negative and fungal *** had the lowest MIC values(0.062,0.125,and 0.25 mg/mL against ***,***,and ***,respectively),as well as minimum fungal concentrations(0.5 mg/mL for both *** and ***).At 12 hr,the cell viability values against tested microbial strains were completely *** lysis and death occurred as a result of severe membrane damage caused by microorganisms perched on RGO *** work gives an insight into the design of effective graphene-based antimicrobial materials for water treatment and remediation.

关键词： Antimicrobial properties Nanoblades Mechano-bactericidal effect RGO nanosheets Gram-positive bacteria Cell viability

Ru-doped functional porous materials for electrocatalytic water splitting

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Nano research 2024年第3期17卷 982-1002页

作者： Chongao Tian Rui Liu Yu Zhang Wenxiu Yang Bo Wang Beijing Key Laboratory of Photoelectronic/Electrophotonic Conversion Materials Advanced Technology Research Institute(Jinan)Advanced Research Institute of Multidisciplinary ScienceSchool of Chemistry and Chemical EngineeringBeijing Institute of TechnologyBeijing 100081China Beijing institute of Technology Library Beijing Institute of TechnologyBeijing 100081China

Electrolytic water splitting(EWS)is an attractive and promising technique for the production of hydrogen ***,the sluggish kinetic rate of hydrogen/oxygen evolution reactions leads to a high overpotential and low energy *** to date,Pt/Ir-based nanocatalysts have become the state-of-the-art EWS catalysts,but disadvantages such as high cost and low earth abundance greatly limit their applications in EWS *** an attractive candidate for the Pt/Ir catalysts,series of Ru-based nanomaterials have aroused much attention for their low price,Pt-like hydrogen bond strength,and high EWS *** particular,Ru-doped functional porous materials have been becoming one of the most representative EWS catalysts,which can not only achieve the dispersion and adjustment for active Ru sites,but also simultaneously solve the problems of mass transfer and catalytic conversion in *** this review,the design and preparation strategies of Ru-doped functional porous materials toward EWS in recent years are summarized,including Ru-doped metal organic frameworks(MOFs),Ru-doped porous organic polymers(POPs),and their ***,detailed structure–activity relationships induced by the tuned geometric/electronic structures of Ru-doped functional porous materials are further depicted in this *** but not least,the challenges and perspectives of Ru-doped functional porous materials catalysts are reasonably proposed to provide fresh ideas for the design of Ru-based EWS catalysts.

关键词： Ru-based catalyst functional porous materials electrolytic water splitting oxygen evolution reaction hydrogen evolution reaction

Distributed Application Addressing in 6G Network

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China Communications 2024年第4期21卷 193-207页

作者： Liu Jie Chen Sibo Liu Yuqin Mo Zhiwei Lin Yilin Zhu Hongmei He Yufeng China Telecom research institute Guangzhou 510632China

To ensure the extreme performances of the new 6G services,applications will be deployed at deep edge,resulting in a serious challenge of distributed application *** paper traces back the latest development of mobile network application addressing,analyzes two novel addressing methods in carrier network,and puts forward a 6G endogenous application addressing scheme by integrating some of their essence into the 6G network architecture,combining the new 6G capabilities of computing&network convergence,endogenous intelligence,and communication-sensing *** paper further illustrates how that the proposed method works in 6G networks and gives preliminary experimental verification.

关键词： application addressing CNC(Computing&Network Convergence) DNS(Domain Name System) ICN(Information-Centric Network) 6G