检索结果-南通市图书馆

Both gain-and loss-of-function variants of KCNA1 are associated with paroxysmal kinesigenic dyskinesia

Journal of Genetics and Genomics 2024年第8期51卷 801-810页

作者： Wan-Bing Sun Jing-Xin fu Yu-Lan chen Hong-fu Li Zhi-Ying Wu dian-fu chen Department of Medical Genetics and Center for Rare Diseases and Department of Neurologyand Zhejiang Key Laboratory of Rare Diseases for Precision Medicine and Clinical Translation in Second Affiliated HospitalZhejiang University School of MedicineHangzhouZhejiang 310009China Nanhu Brain-computer Interface Institute HangzhouZhejiang 314050China MOE Frontier Science Center for Brain Science and Brain-machine Integration School of Brain Science and Brain MedicineZhejiang UniversityHangzhouZhejiang 310012China

KCNA1 is the coding gene for Kv1.1 voltage-gated potassium-channelα*** variants of KCNA1 have been reported to manifest as paroxysmal kinesigenic dyskinesia(PKD),but the correlation between them remains unclear due to the phenotypic complexity of KCNA1 variants as well as the rarity of PKD *** the whole exome sequencing followed by Sanger sequencing,we screen for potential pathogenic KCNA1 variants in patients clinically diagnosed with paroxysmal movement disorders and identify three previously unreported missense variants of KCNA1 in three unrelated Chinese *** proband of one family(c.496G>A,p.A166T)manifests as episodic ataxia type 1,and the other two(c.877G>A,p.V293I and c.1112C>A,p.T371A)manifest as *** pathogenicity of these variants is confirmed by functional studies,suggesting that p.A166T and p.T371A cause a loss-of-function of the channel,while p.V293I leads to a gain-of-function with the property of voltage-dependent gating and activation kinetic *** reviewing the locations of PKD-manifested KCNA1 variants in Kv1.1 protein,we find that these variants tend to cluster around the pore domain,which is similar to ***,our study strengthens the correlation between KCNA1 variants and PKD and provides more information on genotype–phenotype correlations of KCNA1 channelopathy.

关键词： Paroxysmal kinesigenic dyskinesia KCNA1 Loss-of-function Gain-of-function Channelopathy Episodicataxiatype1

来源：

维普期刊数据库评论

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

Photoactive Poly（aryl ether）s Containing Azobenzene Moieties on Pendants for Fabrication of Thermal Stable Surface Relief Gratings

引用

Chemical Research in Chinese Universities 2013年第2期29卷 384-388页

作者： XU Da ZHANG Yu-xuan REN dian-fu chen Xing-bo ZHANG Hai-bo JIANG Zhen-hua College of Chemistry Jilin University Changchun 130012 P. R. China

A series of azobenzene functionalized poly（aryl ether） copolymers（azo-PAEs） containing azo moieties on pendants was prepared by direct copolymerization. The amount of azobenzene chromophores could be controlled by the feed ratio of azo-monomer. Their chemical structures and properties were characterized by means of infrared（IR） spectrometry, UV-Vis, hydrogen nuclear magnetic resonance（Ill NMR）, difference scanning calorimetry（DSC）, and thermogravimetric analysis（TGA）. The results suggest that azo-PAEs had high glass transition temperatures（Tgs） and good thermal stability. By exposing their spin-coated films to an interference pattern laser beam, azo-PAEs could be used for the fabrication of thermal stable surface relief gratings（SRGs）.

关键词： Azo polymer Poly(aryl ether) Surface relief grating Photoactive

来源：

维普期刊数据库评论

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

Global Stability of a Viral Infection Model with Defectively Infected Cells and Latent Age

引用

Chinese Annals of Mathematics,Series B 2024年第4期45卷 555-572页

作者： Jianquan LI Yuming chen Peijun ZHANG dian ZHANG School of Computer Science Xijing University Department of Mathematics Wilfrid Laurier University Xi'an Key Laboratory of Human-Machine Integration and Control Technology for Intelligent Rehabilitation Xijing University Equal contributor.Department of Immunology Xi'an Medical University

The authors propose and analyze a viral infection model with defectively infected cells and age of the latently infected cells. The existence of steady states is determined by the basic reproduction number of virus. With the Lyapunov's direct method, they establish a threshold dynamics of the model with the basic reproduction number of virus as the threshold parameter. To achieve it, a novel procedure is proposed. Its novelties are two-folded. On one hand, the coefficients involved in the specific forms of the used Lyapunov functionals for the two feasible steady states are determined by the same set of inequalities. On the other hand, for the infection steady state, a new approach is proposed to check whether the derivative of the Lyapunov functional candidate along solutions is negative(semi-)definite or not. This procedure not only simplifies the analysis but also exhibits the relationship between the two Lyapunov functionals for the two feasible steady states. Moreover, the procedure is expected to be applicable for other similar models.

关键词： Age structure Basic reproduction number Equilibrium Global stability Lyapunov direct method Viral infection model

来源：

同方期刊数据库评论

在线全文

同方期刊数据库

学校读者我要写书评

暂无评论

Evaluation of 3D crustal seismic velocity models in southwest China:Model performance,limitation,and prospects

引用

Science China Earth Sciences 2024年第2期67卷 604-619页

作者： Xin WANG Ling chen Qi-fu chen Key Laboratory of Earth and Planetary Physics Institute of Geology and GeophysicsChinese Academy of SciencesBeijing100029China College of Earth and Planetary Sciences University of Chinese Academy of SciencesBeijing100049China State Key Laboratory of Lithospheric Evolution Institute of Geology and GeophysicsChinese Academy of SciencesBeijing100029China

Southwest China is a tectonically and seismically active region,witnessing strong deformation due to the collision between the Indian and Eurasian *** the subsurface velocity structure of this region is thus important in understanding the tectonics and geodynamic processes of continental collision and in mitigating seismic *** studies have provided various 3D seismic velocity models in southwest ***,discrepancies exist among these models,and less effort has been made to quantify the reliability and accuracy of these existing velocity *** this study,we use regional 3D waveform simulation to evaluate the performance of various regional crustal 3D velocity models in reproducing observed *** particularly focus on two recent earthquake sequence in the region,the 2021 Yunnan Yangbi MS_(6.4) earthquake sequence and the 2022 Sichuan Luding MS_(6.8) earthquake *** tested 3D velocity models include the Southwest China Community Velocity model V1.0,the Unified Seismic Tomography Models for Continental China Lithosphere V2.0,the adjoint full waveform tomography model of the crustal and upper mantle beneath Eastern Tibetan Plateau,and the shallow seismic structure model beneath continental *** results show that the tested 3D velocity models generally capture well long-period(full-waveform tomography model shows a slighter better performance than the other models,especially for the body-waves,highlighting the advantages

关键词： Southwest China Seismic tomography models Shallow Structures Waveform simulation Model evaluation

来源：

维普期刊数据库

同方期刊数据库评论

在线全文

学校读者我要写书评

暂无评论

Low Selenium and Low Protein Exacerbate Myocardial Damage in Keshan Disease by Affecting the PINK1/Parkin-mediated Mitochondrial Autophagy Pathway

引用

Current Medical Science 2024年第1期44卷 93-101页

作者： Li-wei ZHANG Hong-qi FENG Song-bo fu dian-jun SUN Center for Endemic Disease Control Chinese Center for Disease Control and PreventionHarbin Medical UniversityNHC Key Laboratory of Etiology and Epidemiology(Harbin Medical University)Key Lab of Etiology and EpidemiologyEducation Bureau of Heilongjiang ProvinceHarbin 150086China

Objective Keshan disease(KD)is a myocardial mitochondrial disease closely related to insufficient selenium(Se)and protein *** induced putative kinase 1(PINK1)/Parkin mediated mitochondrial autophagy regulates various physiological and pathological processes in the *** study aimed to elucidate the relationship between PINK1/Parkin-regulated mitochondrial autophagy and KD-related myocardial *** A low Se and low protein animal model was *** hundred Wistar rats were randomly divided into 5 groups(control group,low Se group,low protein group,low Se+low protein group,and corn from KD area group).The JC-1 method was used to detect the mitochondrial membrane potential(MMP).ELISA was used to detect serum creatine kinase MB(CK-MB),cardiac troponin I(cTnI),and mitochondrial-glutamicoxalacetic transaminase(M-GOT)***-PCR and Western blot analysis were used to detect the expression of PINK1,Parkin,sequestome 1(P62),and microtubule-associated proteins1A/1B light chain 3B(MAP1LC3B).Results The MMP was significantly decreased and the activity of CK-MB,cTnI,and M-GOT significantly increased in each experimental group(low Se group,low protein group,low Se+low protein group and corn from KD area group)compared with the control group(P<0.05 for all).The mRNA and protein expression levels of PINK1,Parkin and MAP1LC3B were profoundly increased,and those of P62 markedly decreased in the experimental groups compared with the control group(P<0.05 for all).Conclusion Low Se and low protein levels exacerbate myocardial damage in KD by affecting the PINK1/Parkin-mediated mitochondrial autophagy pathway.

关键词： Keshan disease low selenium and low protein myocardial mitochondrial injury PTEN induced putative kinase 1(PINK1)/Parkin mitochondrial autophagy

来源：

维普期刊数据库评论

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

The impact of the Rural Minimum Living Standard Guarantee(Rural Dibao)Program on child nutrition outcomes

引用

Journal of Integrative Agriculture 2024年第2期23卷 444-456页

作者： dian chen Xiangming Fang Yu chen Xiaodong Zheng Zhuo chen Rodney B.W.Smith Academy of Global Food Economics and Policy China Agricultural UniversityBeijing 100083China School of Public Health Georgia State UniversityAtlanta 30303USA School of Economics Zhejiang Gongshang UniversityHangzhou 310018China College of Public Health University of GeorgiaAthens 30602USA Department of Applied Economics University of MinnesotaSaint Paul 55108USA

The Rural Minimum Living Standard Guarantee(Rural Dibao)is an important unconditional cash transfer program to alleviate poverty in rural *** the importance of children’s nutrition in breaking poverty cycles,little is known about the impact of Rural Dibao on child nutrition *** China Family Panel Studies(CFPS),this paper examines the effects of Rural Dibao on child nutrition outcomes and investigates potential pathways and heterogeneous *** exploit propensity score matching and difference-in-differences techniques to evaluate the effects of the Rural Dibao program on child nutrition *** results suggest that Rural Dibao significantly impacts the nutrition outcomes of children up to 15 years of ***,our results suggest that Rural Dibao improves child height-to-age z-scores by 1.05 standard deviations and lowers the probability of stunting by 11.9 percentage *** analyses suggest that increased protein intake is the main pathway through which Rural Dibao participation contributes to better nutrition *** also find that the effect of the program is more pronounced among girls,children who are non-left-behind or live with highly educated mothers,and those from low-income families and poor *** findings suggest that Rural Dibao participation helps improve child nutrition outcomes through improving diet quality.

关键词： Rural Minimum Living Standard Guarantee nutrition height-to-age z-scores stunting rural China

来源：

维普期刊数据库

同方期刊数据库评论

在线全文

学校读者我要写书评

暂无评论

Mathematics of multi-agent learning systems at the interface of game theory and artificial intelligence

引用

Science China(Information Sciences) 2024年第6期67卷 445-447页

作者： Long WANG Feng fu Xingru chen Center for Systems and Control College of EngineeringPeking University Center for Multi-Agent Research Institute for Artificial IntelligencePeking University Department of Mathematics Dartmouth College Department of Biomedical Data Science Geisel School of Medicine at Dartmouth School of Science Beijing University of Posts and Telecommunications

Evolutionary game theory (EGT) and artificial intelligence(AI) are two fields that,at first glance,might seem distinct,but they have notable connections and *** former focuses on the evolution of behaviors (or strategies)in a population,where individuals interact with others and update their strategies based on imitation (or social learning).

关键词：

来源：

同方期刊数据库评论

在线全文

同方期刊数据库

学校读者我要写书评

暂无评论

Superatom-assembled boranes,carboranes,and low-dimensional boron nanomaterials based on aromatic icosahedral B_(12)and C_(2)B_(10)

引用

Nano Research 2024年第7期17卷 6734-6740页

作者： Qiao-Qiao Yan Yan-Fang Wei Qiang chen Yue-Wen Mu Si-dian Li Institute of Molecular Science Shanxi UniversityTaiyuan 030006China

Using the experimentally known aromatic icosahedral superatoms I_(h)B_(12)H_(12)2−and D_(5d)1,12-C_(2)B_(10)H_(12)as building blocks and based on extensive density functional theory calculations,we predict herein a series of core–shell superpolyhedral boranes and carboranes in a bottom-up approach,including the high-symmetry Th B_(12)@B_(152)H_(72)2−(2),C2h C_(2)B_(10)@B_(152)H_(72)(3),D_(3d)B_(12)@B_(144)H_(66)(4),I_(h)B_(12)@C_(24)B_(12)0H_(72)2−(6),and D_(5d)C_(2)B_(10)@C_(24)B_(12)0H_(72)(7).More interestingly,the superatom-assembled linear D2h B_(36)H_(32)^(2−)(8),close-packed planar D_(3d)B_(84)H_(60)^(2−)(10),and nearly close-packed core−shell D_(3d)B_(12)@B144H_(6)6(4)can be extended periodically to form the one-dimensional(1D)α-rhombohedral borane nanowire B_(12)H_(10)(Pmmm)(9),two-dimensional(2D)α-rhombohedral monolayer borophane B_(12)H_(6)(P m1)(11),and the experimentally known three-dimensional(3D)α-rhombohedral boron(R m)(12)which can be viewed as an assembly of the monolayer B_(12)H_(6)(11)staggered in vertical direction,setting up a bottom-up strategy to form low-dimensional boron-based nanomaterials from their borane“seeds”via partial or complete *** bonding analyses indicate that the high stability of these nanostructures originates from the spherical aromaticity of their icosahedral B_(12)or C_(2)B_(10)structural units which possess the universal skeleton electronic configuration of 1S21P61D101F8 following the Wade’s n+1 *** infrared(IR)and Raman spectra of the most-concerned neutral B_(12)@B144H_(6)6(4)and C_(2)B_(10)@C_(24)B_(12)0H_(72)(7)are computationally simulated to facilitate their experimental characterizations.

关键词： superatom-assembling bottom-up approach density functional theory superpolyhedral boranes superpolyhedral carboranes borophanes

来源：

维普期刊数据库评论

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

High-Level Security Dimension Evaluation of Blockchain BaaS System and Key Technology

引用

China Communications 2024年第6期21卷 176-191页

作者： chen Wanfa Zheng Qing’an chen Shuzhen fu Hongyi chen Liang fujian Police College Fuzhou 350007China Computer Science and Information Security Management Department Fujian Police CollegeFuzhou 350007China fujian Province Key Laboratory for Digital Network Crime Supervision and Prevention Fuzhou 350007China Beijing University of Posts and Telecommunications Beijing 100876China fujian Provincial Prison Administration Fuzhou 350002China

In recent years,blockchain technology integration and application has gradually become an important driving force for new technological innovation and industrial *** blockchain technology and applications are developing rapidly,the emerging security risks and obstacles have gradually become *** can still find security issues in blockchain systems and conduct attacks,causing increasing losses from network attacks every *** response to the current demand for blockchain application security detection and assessment in all industries,and the insufficient coverage of existing detection technologies such as smart contract detectiontechnology,this paper proposes a blockchain core technology security assessment system model,and studies the relevant detection and assessment key technologies and systems.A security assessment scheme based on a smart contract and consensus mechanism detection scheme is *** the underlying blockchain architecture supports the traceability of detection results using super ***,the functionality and performance of the system were tested,and the test results show that the model and solutions proposed in this paper have good feasibility.

关键词： blockchain security consensus mechanis smart contract

来源：

维普期刊数据库

同方期刊数据库评论

在线全文

学校读者我要写书评

暂无评论

Honokiol Prevents Intestinal Barrier Dysfunction in Mice with Severe Acute Pancreatitis and Inhibits JAK/STAT1 Pathway and Acetylation of HMGB1

引用

Chinese Journal of Integrative Medicine 2024年第6期30卷 534-542页

作者： LI Jie chen Ya-feng GAO Lei LI Yi-jie FENG dian-xu Department of General Surgery Putuo HospitalShanghai University of Traditional Chinese MedicineShanghai200062China

Objective To investigate the effect of honokiol(HON)and the role of high-mobility group protein B1(HMGB1)on the pathogenesis of severe acute pancreatitis(SAP).Methods Thirty mice were numbered according to weight,and randomly divided into 5 groups using a random number table,including control,SAP,SAP and normal saline(SAP+NS),SAP and ethyl pyruvate(SAP+EP),or SAP+HON groups,6 mice in each *** of pancreas,intestine,and blood were collected 12 h after SAP model induction for examination of pathologic changes,immune function alterations by enzyme linked immunosorbent assay(ELISA),and Western *** vitro experiments,macrophages were divided into 5 groups,the control,lipopolysaccharide(LPS),LPS+DMSO(DMSO),LPS+anti-HMGB1 monoclonal antibody(mAb),and LPS+HON *** tight connection level was determined by transmission electron microscopy and fluorescein *** location and acetylation of HMGB1 were measured by Western ***,pyridone 6 and silencing signal transducer and activator of the transcription 1(siSTAT1)combined with honokiol were added to determine whether the Janus kinase(JAK)/STAT1 participated in the regulation of honokiol on *** protein expression levels of HMGB1,JAK,and STAT1 were detected using Western *** Mice with SAP had inflammatory injury in the pancreas,bleeding of intestinal tissues,and cells with disrupted *** in the SAP+HON group had significantly fewer pathological *** with SAP also had significant increases in the serum levels of amylase,lipase,HMGB1,tumor necrosis factor-α,interleukin-6,diamine oxidase,endotoxin-1,and *** in the SAP+HON group did not show these abnormalities(P<0.01).Studies of Caco-2 cells indicated that LPS increased the levels of occludin and claudin-1 as well as tight junction permeability,decreased the levels of junctional adhesion molecule C,and elevated intercellular permeability(P<0.01).HON treatment blocked these *** of

关键词： severe acute pancreatitis intestinal barrier dysfunction honokiol high-mobility group protein B1 Januskinase signal transducerand activatorof transcription1

来源：

维普期刊数据库

同方期刊数据库评论

在线全文

学校读者我要写书评

暂无评论

欢迎您,

建议与咨询留下您的常用邮箱和电话号码，以便我们向您反馈解决方案和替代方法

时间限定

文献类型

馆藏选择

核心期刊

语言

文献类型

帮助

文字说明：

检索规则说明：

检索范例：

分类表

所选分类

限定检索结果

文献类型

馆藏范围

日期分布

学科分类号

主题

机构

作者

语言

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

请选择保存的检索档案：

请选择收藏分类：

通借通还

欢迎您,

建议与咨询 留下您的常用邮箱和电话号码，以便我们向您反馈解决方案和替代方法

时间限定

文献类型

馆藏选择

核心期刊

语言

文献类型

帮助

文字说明：

检索规则说明：

检索范例：

分类表

所选分类

限定检索结果

文献类型

馆藏范围

日期分布

学科分类号

主题

机构

作者

语言

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

请选择保存的检索档案： 新增检索档案 确定 取消

请选择收藏分类： 新增自定义分类 确定 取消

通借通还

建议与咨询留下您的常用邮箱和电话号码，以便我们向您反馈解决方案和替代方法

请选择保存的检索档案：

请选择收藏分类：