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A targeted sequencing approach to find novel pathogenic genes associated with sporadic aortic dissection

Science China(Life Sciences) 2018年第12期61卷 1545-1553页

作者： Zongzhe Li Chengming Zhou Lun Tan Peng Chen Yanyan Cao Xianqing Li Jiangtao Yan Hesong zeng dao-Wu wang dao-Wen wang Division of Cardiology Departments of Internal Medicine and Genetic Diagnosis CenterTongji HospitalTongji Medical CollegeHuazhong University of Science and TechnologyWuhan 430030China Hubei Key Laboratory of Genetics and Molecular Mechanisms of Cardiological Disorders Wuhan 430030China State Key Laboratory of Reproductive Medicine Clinical Center of Reproductive Medicine and Department of Cardiologythe First Affiliated HospitalNanjing Medical UniversityNanjing 210029China

Aortic dissection(AD) is a heterogeneous genetic disease of the aorta with high mortality and poor prognosis. However, only few genetic causes of AD have been explored till date. After conducting a broad literature review focused on identifying potential pathogenic pathways, we designed a panel containing 152 AD-associated genes to conduct massively parallel targeted nextgeneration sequencing of 702 sporadic aortic dissection patients and 163 matched healthy controls. After validation by Sanger sequencing, we identified 21 definitely pathogenic and 635 likely pathogenic variants in 61.25%(430/702) of patients. In these patients, 34.88%(150/430) harbored more than one variant that was either definitely or likely to be pathogenic. Among the candidate genes, we identified 546 likely pathogenic variants in 47.72%(335/702) of patients. Importantly, we identified 94 lossof-function(LOF) variants in 45 genes in AD patients, but only five LOF variants in the controls(P=1.34×10^(-4)). With a burden test, we highlighted RNF213 as an important new gene for AD pathogenesis. We also performed transcriptome sequencing of human aorta tissues to evaluate the expression levels of these newly identified genes. Our study has compiled a comprehensive genetic map of sporadic AD in the Han Chinese population. We believe it will facilitate risk predicting and genetic diagnosis of this severe disease in the future.

关键词： aortic dissection next-generation sequencing genetic diagnosis

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Dual modulation of homogeneous nanomaterialization and electrochemical activation enhancing zinc ion storage

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Science China Chemistry 2025年第01期 385-393页

作者： Jianwei wang Huan wang Kangning wang Wenhui wang Wenlin Zhang Yanzhong Zhen School of Chemistry & Chemical Engineering Yan'an University

Vanadium-based electrode materials are widely investigated,but the low specific capacity and slow electrochemical kinetics in aqueous zinc-ion batteries still limit their commercial ***,the VS2/Ca V4O9material with the morphology of nanoflower was synthesized by a one-step hydrothermal *** to the blocky structure of pure VS2material,the VS2/Ca V4O9material is composed of thinner homogeneous *** open structures could provide abundant electrochemical active sites and ion transport channels,and then promote the electrochemical reaction *** addition,they can also buffer the bulk strain during the reaction *** improve the utilization of vanadium elements,an in-situ electrochemical activation strategy is used to explore the storage performance of the VS2/Ca V4O9material,the different activation voltage range of 0.4–1.6 and 0.4–1.4 V are selected,*** with the longer activation plateau of activated-VS2,the VS2/Ca V4O9cathode could quickly reach the activation state in the range of 1.4–1.6 V and cause the release of additional Zn storage sites *** VS2/Ca V4O9cathode delivers a higher power density of 37,000 W kg-1and a significant energy density of 423 Wh *** the high current density of 15 A g-1,the VS2/Ca V4O9cathode still has a discharge capacity of 183.9m Ah g-1after 5,000 cycles,and the capacity decay rate per cycle is only 0.0042%.Continuous cyclic voltammetry （CV） curves,electrochemical impedance spectroscopy （EIS） measurements,density functional theory （DFT） calculation and galvanostatic intermittent titration technique （GITT） measurements demonstrate that the VS2/Ca V4O9cathode has a faster ion diffusion/charge transfer ***,the assembled flexible device has an excellent mechanical stability.

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一种ICEEMDAN-CNN-SVR滑坡位移组合预测模型

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三峡大学学报（自然科学版） 2025年第1期47卷 37-43页

作者：石化波王刚曾怀恩中煤湖北地质勘察基础工程有限公司武汉430070 中铁建城建交通发展有限公司江苏苏州215000 湖北长江三峡滑坡国家野外科学观测研究站湖北宜昌443002 三峡大学土木与建筑学院湖北宜昌443002

滑坡位移预测是滑坡早期预警系统的重要组成部分,针对位移分解程度与特征选取深入程度不够导致滑坡位移预测精度不高的问题,提出一种ICEEMDAN-CNN-SVR滑坡位移组合预测模型:为了解决位移分解程度不够的问题,该模型首先运用ICEEMDAN分解... 详细信息

滑坡位移预测是滑坡早期预警系统的重要组成部分,针对位移分解程度与特征选取深入程度不够导致滑坡位移预测精度不高的问题,提出一种ICEEMDAN-CNN-SVR滑坡位移组合预测模型:为了解决位移分解程度不够的问题,该模型首先运用ICEEMDAN分解模型对滑坡位移曲线进行分解,将平滑性较好且具有递增趋势的IMF曲线作为趋势项位移,将其他具有波动趋势的IMF曲线总和重构为周期项位移;为了解决特征选取深入程度不够的问题,针对不同位移特性进行了特征变量选取,通过二维平铺与CNN特征提取得到特征变量更深层次的信息,将提取到的特征信息输入SVR预测模型中实现对趋势项位移与周期项位移的精准预测.以典型堆积层滑坡———八字门滑坡为例,选取ZG110与ZG111监测点2007年1月—2012年9月典型变形阶段水平位移数据进行研究,结果表明:ZG110与ZG111监测点预测评价指标R2,ERMSE,EMAE分别为0.9951、0.9989、5.7489、2.7532,4.5091、1.8529,预测效果良好;将模型预测结果与EEMDCNN-SVR预测模型及CNN-SVR预测模型结果作对比,相较其他预测模型,新模型的预测精度有所提升.

关键词：八字门滑坡 ICEEMDAN分解特征提取 CNN-SVR模型对比分析

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Variants of genes encoding collagens and matrix metalloproteinase system increased the risk of aortic dissection

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Science China(Life Sciences) 2017年第1期60卷 57-65页

作者： Zongzhe Li Chengming Zhou Lun Tan Peng Chen Yanyan Cao Chenze Li Xianqing Li Jiangtao Yan Hesong zeng dao-Wu wang dao-Wen wang Division of Cardiology Departments of Internal Medicine and Genetic Diagnosis CenterTongji HospitalTongji Medical CollegeHuazhong University of Science and Technology Department of Cardiology the First Affiliated HospitalNanjing Medical University

Aortic dissection （AD） is a devastating, heterogeneous condition of aorta. The homeostasis between collagens and matrix metalloproteases （MMPs）/tissue inhibitors of MMPs （TIMPs） system in the extracellular matrix plays an important role for structure and functions of aorta. However, our knowledge on association between variants of genes in this system and pathogenesis of AD is very limited. We analyzed all yet known coding human genes of collagens （45 genes）, MMPs/TIMPs （27 genes） in 702 sporadic AD patients and in 163 matched healthy controls, by using massively targeted next-generation and Sanger sequencing. To define the pathogenesis of potential disease-causing candidate genes, we performed transcriptome sequencing and pedigree co-segregation analysis in some genes and generated Col5a2 knockout rats. We identified 257 pathogenic or likely pathogenic variants which involved 88.89% （64/72） genes in collagens-MMPs/TIMPs system and accounted for 31.05% （218/702） sporadic AD patients. In them, 84.86% patients （185/218） carried one variant, 12.84% two variants and 2.30% more than two variants. Importantly, we identified 52 novel probablY pathogenic loss-of-function （LOF） variants （20 nonsense, 16 frameshift, 14 splice sites, one stop-loss, one initiation codon） in 11.06% （50/452） AD patients, which were absent in 163 controls （P=2.5-10-5）. Transcriptome sequencing revealed that identified variants induced dyshomeostasis in expression of collagens-TIMPs/MMPs systems. The Col5a2-/- rats manifested growth retardation and aortic dysplasia. Our study provides a first comprehensive map of genetic alterations in collagens-MMPs/TIMPs system in sporadic AD patients and suggests that variants of these genes contribute largely to AD pathogenesis.

关键词： aortic dissection collagen matrix metalloproteinase next-generation sequencing genetic diagnosis

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High-energy-density lithium manganese iron phosphate for lithium-ion batteries: Progresses, challenges, and prospects

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Journal of Energy Chemistry 2025年第1期100卷 1-17页

作者： Bokun Zhang Xiaoyun wang Shuai wang Yan Li Libo Chen Handong Jiao Zhijing Yu Jiguo Tu State Key Laboratory of Advanced Metallurgy University of Science and Technology Beijing Research Institute for Intelligent Wearable Systems The Hong Kong Polytechnic University Institute of Advanced Structure Technology Beijing Institute of Technology

The soaring demand for smart portable electronics and electric vehicles is propelling the advancements in high-energy–density lithium-ion *** manganese iron phosphate (LiMnxFe1-xPO4) has garnered significant attention as a promising positive electrode material for lithium-ion batteries due to its advantages of low cost,high safety,long cycle life,high voltage,good high-temperature performance,and high energy *** LiMnxFe1-xPO4has made significant breakthroughs in the past few decades,there are still facing great challenges in poor electronic conductivity and Li-ion diffusion,manganese dissolution affecting battery cycling performance,as well as low tap *** review systematically summarizes the reaction mechanisms,various synthesis methods,and electrochemical properties of LiMnxFe1-xPO4to analyze reaction processes accurately and guide material ***,the main challenges currently faced are concluded,and the corresponding various modification strategies are discussed to enhance the reaction kinetics and electrochemical performance of LiMnxFe1-xPO4,including multi-scale particle regulation,heteroatom doping,surface coating,as well as microscopic morphology ***,in view of the current research challenges faced by intrinsic reaction processes,kinetics,and energy storage applications,the promising research directions are *** importantly,it is expected to provide key insights into the development of high-performance and stable LiMnxFe1-xPO4materials,to achieve practical energy storage requirements.

关键词： Lithium manganese iron phosphate High energy density Lithium-ion batteries Reaction mechanism Tap density

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Flexible molecules dedicate to release strain of inverted inorganic perovskite solar cell

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Journal of Energy Chemistry 2025年第1期100卷 87-93页

作者： Hongrui Sun Sanlong wang Pengyang wang Yali Liu Shanshan Qi Biao Shi Ying Zhao Xiaodan Zhang Institute of Photoelectronic Thin Film Devices and Technology Renewable Energy Conversion and Storage Center State Key Laboratory of Photovoltaic Materials and CellsNankai University Key Laboratory of Photoelectronic Thin Film Devices and Technology of Tianjin Haihe Laboratory of Sustainable Chemical Transformations Engineering Research Center of Thin Film Photoelectronic Technology of Ministry of Education Collaborative Innovation Center of Chemical Science and Engineering (Tianjin)

The tensile strain in inorganic perovskite films induced by thermal annealing is one of the primary factors contributing to the inefficiency and instability of inorganic perovskite solar cells （IPSCs）,which reduces the defect formation ***,a flexible molecule 5-maleimidovaleric acid （5-MVA） was introduced as a strain buffer to release the residual strain of *** anhydride and carboxyl groups in 5-MVA interact strongly with the uncoordinated Pb2+through Lewis acid-base reaction,thus tightly“pull”the perovskite *** in-between soft carbon chain increased the structural flexibility of CsPbI2.85Br0.15perovskite materials,which effectively relieved the intrinsic internal strain of CsPbI2.85Br0.15,resisted the corrosion of external strain,and also reduced the formation of defects such as VIand *** addition,the introduction of 5-MVA improved crystal quality,passivated residual defects,and narrowed energy level ***,power conversion efficiency （PCE） of NiOxbased inverted IPSCs increased from 19.25% to 20.82% with the open-circuit voltage enhanced from 1.164 V to 1.230 *** release of strain also improved the stability of CsPbI2.85Br0.15perovskite films and devices.

关键词： Inverted inorganic perovskite solar cells Flexible molecules Strain release Crystallization Energy barrier High PCE

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Synergistically enhanced ORR and HER performance on Co-N-C coupled in-situ generated PtCo intermetallic

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Journal of Energy Chemistry 2025年第1期100卷 721-729页

作者： Guanyu Luo Min Song Lulu An Xiao Huang Qian Zhang Chenhao Zhang Tao Shen Shuang wang Deli wang Key Laboratory of Material Chemistry for Energy Conversion and Storage (Huazhong University of Science and Technology) Ministry of Education School of Chemistry and Chemical Engineering Huazhong University of Science and Technology

Integrating multi-scale sites in a composite catalyst is vital to realize efficient ***,a synergistic composite catalyst consisting of Co atomic sites and in-situ generated PtCo intermetallic compounds （IMCs）(o-PtCo@CoNC) is proposed through Co pre-anchoring and subsequent impregnation-reduction *** loading of Co atoms provides a chance for in-situ generating PtCo ordered intermetallic *** remaining Co single atoms and PtCo IMCs construct synergistic electrocatalytic *** from the ordered structure,synergistic effect of Pt Co IMCs and Co single atoms,o-PtCo@CoNC exhibits excellent electrocatalytic performance for oxygen reduction reaction （ORR） and hydrogen evolution reaction （HER） with mass activity of 1.21 A mgPt-1（at 0.9 V） and5.70 A mgPt-1（at an overpotential of 100 mV）,***,o-PtCo@CoNC delivers negligible loss of half-wave potential and overpotential during long-term stability test in acid solutions,with 13 mV decay after 50,000 potential cycles for ORR and a 2.7 mV decay after 20,000 potential cycles for *** integration strategy of single-atomic sites coupled IMCs paves the way for enhancing the activity and durability of Pt-based electrocatalysts.

关键词： Electrolytic reduction

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EZH2-dependent myelination following sciatic nerve injury

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Neural Regeneration Research 2025年第8期20卷 2382-2394页

作者： Hui Zhu Li Mu Xi Xu Tianyi Huang Ying wang Siyuan Xu Yiting wang Wencong wang Zhiping wang Hongkui wang Chengbin Xue Research Center of Clinical Medicine Affiliated Hospital of Nantong UniversityKey Laboratory of Neuroregeneration of Jiangsu and Ministry of EducationCo-innovation Center of NeuroregenerationNMPA Key Laboratory for Research and Evaluation of Tissue Engineering Technology ProductsNantong UniversityNantongJiangsu ProvinceChina Medical School of Nantong University NantongJiangsu ProvinceChina Department of Neurology Affiliated Hospital of Nantong UniversityNantongJiangsu ProvinceChina Department of Rehabilitation Medicine Affiliated Hospital of Nantong UniversityNantongJiangsu ProvinceChina Department of Critical Care Medicine Nantong Fourth People’s HospitalNantongJiangsu ProvinceChina

Demyelination and remyelination have been major focal points in the study of peripheral nerve regeneration following peripheral nerve ***,the gene regulatory network of regenerated myelin differs from that of native *** of enhancer of zeste homolog 2(EZH2)hinders the differentiation,maturation,and myelination of Schwann cells in *** further determine the role of EZH2 in myelination and recovery post-peripheral nerve injury,conditional knockout mice lacking Ezh2 in Schwann cells(Ezh2^(fl/fl);Dhh-Cre and Ezh2^(fl/fl);Mpz-Cre)were *** results show that a significant proportion of axons in the sciatic nerve of Ezh2-depleted mice remain *** highlights the crucial role of Ezh2 in initiating Schwann cell ***,we observed that 21 days after inducing a sciatic nerve crush injury in these mice,most axons had remyelinated at the injury site in the control nerve,while Ezh2^(fl/fl);Mpz-Cre mice had significantly fewer remyelinated axons compared with their wild-type *** suggests that the absence of Ezh2 in Schwann cells impairs myelin formation and *** conclusion,EZH2 has emerged as a pivotal regulatory factor in the process of demyelination and myelin regeneration following peripheral nerve *** EZH2 activity during these processes may offer a promising therapeutic target for the treatment of peripheral nerve injuries.

关键词： demyelination EZH2 myelination peripheral nerve injury PRC2 remyelination Schwann cells sciatic nerve crush sciatic nerve transection

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Dealloying corrosion of anodic and nanometric Mg41Nd5 in solid solution-treated Mg-3Nd-1Li-0.2Zn alloy

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Journal of Materials Science & Technology 2021年第24期83卷 161-178页

作者： Gui-Jia Gao Mei-Qi zeng En-Liang Zhang Rong-Chang zeng Lan-Yue Cui dao-kui Xu Feng-Qin wang M.Bobby Kannan Corrosion Laboratory for Light Metals College of Materials Science and EngineeringShandong University of Science and TechnologyQingdao266590China School of Materials Science and Engineering Zhengzhou UniversityZhengzhou450002China Key Laboratory of Nuclear Materials and Safety Assessment Institute of Metal ResearchChinese Academy of SciencesShenyang110016China School of Engineering University of NewcastleCallaghanNew South Wales 2308Australia

The microstructure and chemical compositions of the solid solution-treated Mg-3 Nd-1 Li-0.2 Zn alloy were characterized using optical microscope,scanning electron microscope(SEM),transmission electron microscope(TEM),electron probe micro-analyzer(EPMA)and X-ray photoelectron spectroscopy(XPS).The corrosion behaviour of the alloy was investigated via electrochemical polarization,electrochemical impedance spectroscopy(EIS),hydrogen evolution test and scanning Kelvin probe(SKP).The results showed that the microstructure of the as-extruded Mg-3 Nd-1 Li-0.2 Zn alloy containedα-Mg matrix and nanometric second phase Mg_(41)Nd_(5).The grain size of the alloy increased significantly with the increase in the heat-treatment duration,whereas the volume fraction of the second phase decreased after the solid solution *** surface film was composed of oxides(Nd_(2)O_(3),Mg O,Li_(2)O and Zn O)and carbonates(Mg CO3 and Li_(2)CO3),in addition to *** as-extruded alloy exhibited the best corrosion resistance after an initial soaking of 10 min,whereas the alloy with 4 h-solution-treatment possessed the lowest corrosion rate after a longer immersion(1 h).This can be attributed to the formation of Nd-containing oxide film on the alloys and a dense corrosion product *** dealloying corrosion of the second phase was related to the anodic Mg_(41)Nd_(5)with a more negative Volta potential relative toα-Mg *** preferential corrosion of Mg_(41)Nd_(5)is proven by in-situ observation and *** solid solution treatment of Mg-3 Nd-1 Li-0.2 Zn alloy led to a shift in corrosion type from pitting corrosion to uniform corrosion under long-term exposure.

关键词： Magnesium alloy Corrosion Second phase Rare metal Dealloying

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Satellite remote sensing reveals overwhelming recovery of forest from disturbances in Asia

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Atmospheric and Oceanic Science Letters 2025年第1期18卷 46-51页

作者： Yiying Zhu Hesong wang Anzhi Zhang School of Ecology and Nature Conservation Beijing Forestry UniversityBeijingChina Key Laboratory of Regional Climate and Environment for Temperate East Asia Institute of Atmospheric PhysicsChinese Academy of SciencesBeijingChina College of Forestry and Landscape Architecture Xinjiang Agricultural UniversityUrumqiChina

Forest ecosystems play key roles in mitigating human-induced climate change through enhanced carbon uptake;however,frequently occurring climate extremes and human activities have considerably threatened the stability of *** the same time,detailed accounts of disturbances and forest responses are not yet well quantified in *** study employed the Breaks For Additive Seasonal and Trend method-an abrupt-change detection method-to analyze the Enhanced Vegetation Index time series in East Asia,South Asia,and Southeast *** approach allowed us to detect forest disturbance and quantify the resilience after *** showed that 20%of forests experienced disturbance with an increasing trend from 2000 to 2022,and Southeast Asian countries were more severely affected by ***,95%of forests had robust resilience and could recover from disturbance within a few *** resilience of forests suffering from greater magnitude of disturbance tended to be stronger than forests with lower disturbance *** summary,this study investigated the resilience of forests across the low and middle latitudes of Asia over the past two *** authors found that most forests exhibited good resilience after disturbance and about two-thirds had recovered to a better state in *** findings of this study underscore the complex relationship between disturbance and resilience,contributing to comprehension of forest resilience through satellite remote sensing.

关键词： Forest ecosystem Enhanced vegetation index Breaks for additive seasonal and trend method Disturbance Resilience

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