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Novel homozygous ADAMTS17 missense variant in Weill-Marchesani syndrome

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International Journal of Ophthalmology(English edition) 2023年第5期16卷 694-699页

作者： Na Miao Yao Zhang Jin-Ying Liao Lin Zhou Ji-Cai He rong-qin yang Xu-yang Liu Li Tang Department of Ophthalmology West China Hospital of Sichuan UniversityChengdu 610041Sichuan ProvinceChina Department of Ophthalmology First People’s Hospital of Liangshan Yi Autonomous PrefectureXichang 615306Sichuan ProvinceChina Xiamen Eye Center Xiamen UniversityXiamen 361011Fujian ProvinceChina Department of Ophthalmology Shenzhen People’s Hospitalthe 2nd Clinical Medical CollegeJinan UniversityShenzhen 518040Guangdong ProvinceChina

AIM: To explore the phenotype and genotype of WeillMarchesani syndrome(WMS) in a Chinese family and review related ***: Three WMS patients and other unaffected individuals in this family with a history of consanguineous marriage were included in this study. Medical history, comprehensive ophthalmic examinations, and systemic evaluation, as well as whole exome and Sanger sequencing of specific genomic regions, were performed. RESULTS: The three affected siblings presented with short stature, brachydactyly and ocular disorders, including very shallow anterior chamber, high myopia, microspherophakia lens subluxation with stretched zonules and glaucoma. Genetic analysis verified a homozygous missense mutation(c.2983C>T: p. Arg995Trp) in ADAMTS17,which was correlated with the diseases in this family, indicating an autosomal recessive inherited manner of WMS. This review aims to summarize the mutation sites of WMS genes, so as to prevent the disease and better guide clinical diagnosis and ***: A novel homozygous missense variant of ADAMTS17 is identified in a WMS family with a history of consanguineous marriage. Our study expands the range of mutations associated with WMS and deepens our understanding of pathology in disease associated with ADAMTS17 variants.

关键词： Weill-Marchesani syndrome ADAMTS17 missense variation molecular genetics

Empirical likelihood for spatial cross-sectional data models with matrix exponential spatial specification

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Applied Mathematics(A Journal of Chinese Universities) 2024年第1期39卷 125-139页

作者： LIU Yan rong Jian-rong qin Yong-song Nanning University Nanning 530200China Center for Applied Mathematics of Guangxi and College of Mathematics and Statistics Guangxi Normal UniversityGuilin 541004China

In this paper,we study spatial cross-sectional data models in the form of matrix exponential spatial specification(MESS),where MESS appears in both dependent and error *** empirical likelihood(EL)ratio statistics are established for the parameters of the MESS *** is shown that the limiting distributions of EL ratio statistics follow chi-square distributions,which are used to construct the confidence regions of model *** experiments are conducted to compare the performances of confidence regions based on EL method and normal approximation method.

关键词： MESS empirical likelihood con dence region

Bilayer Compounded Polytetrafluoroethylene Membrane for Enhanced Oil-Water Emulsion Separation

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Chinese Journal of Polymer Science 2024年第6期42卷 838-850页

作者： Yu-Liang yang Tai-Ran Zhang Yan-Ting Han Shao-Yun Guo qin-Gong rong Jia-Bin Shen State Key Laboratory of Polymer Materials Engineering Polymer Research Institute of Sichuan UniversitySichuan Provincial Engineering Laboratory of Plastic/Rubber Complex Processing TechnologyChengdu610065China West China Hospital/West China School of Nursing Sichuan UniversityChengdu610041China Shandong Senrong New Materials Co. Ltd.Zibo256401China

In order to achieve efficient and durable oil-water emulsion separation,the membranes possessing high separation efficiency and mechanical strength attract extensive attention and are in great *** present study,a kind of polytetrafluoroethylene(PTFE)-based bilayer membrane was fabricated by electrospinning fibrous PTFE(fPTFE)on an expanded PTFE(ePTFE)*** morphological observation revealed that the fibrous structure of the fPTFE layer could be tailored by controlling the formulation of spinning *** addition of appropriate polyoxyethylene(PEO)would make the fibers in the fPTFE layer finer and more *** a result,the compounded membrane exhibited a small pore size of approximately 1.25µm and a substantial porosity nearing 80%.This led to super-hydrophobicity,characterized by a high water contact angle(WCA)of 149.8°,and facilitated rapid oil *** water-in-oil emulsion separation experiment further confirmed that the compounded membrane not only had a high separation efficiency closing 100%,but such an outstanding separation capacity could be largely retained,either through multiple cycles of use or through strong acid(pH=1),strong alkali(pH=12),or high-temperature(100°C)***,the mechanical behavior of the bilayer membrane was basically contributed by that of each layer in terms of their volume *** significantly,the poor creep resistance of fPTFE layer was suppressed by compounding with ePTFE ***,this study has laid the groundwork for a novel approach to create PTFE-based compounded membranes with exceptional overall characteristics,showing promise for applications in the realm of emulsion separation.

关键词： ePTFE Electrospinning membrane Bilayer compounding Emulsion separation

Application of texture signatures based on multiparameter-magnetic resonance imaging for predicting microvascular invasion in hepatocellular carcinoma:Retrospective study

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World Journal of Gastrointestinal Oncology 2024年第4期16卷 1309-1318页

作者： Hai-yang Nong Yong-Yi Cen Mi qin Wen-Qi qin You-Xiang Xie Lin Li Man-rong Liu Ke Ding Department of Radiology The Third Affiliated Hospital of Guangxi Medical UniversityNanning 530031Guangxi Zhuang Autonomous RegionChina Department of Radiology The Affiliated Hospital of Youjiang Medical University for NationalitiesBaise 533000Guangxi Zhuang Autonomous RegionChina Department of Radiology The First Affiliated Hospital of Guangxi University of Traditional Chinese MedicineNanning 530031Guangxi Zhuang Autonomous RegionChina Department of Hepatobiliary Surgery The Third Affiliated Hospital of Guangxi Medical UniversityNanning 530031Guangxi Zhuang Autonomous RegionChina Department of Ultrasound The Third Affiliated Hospital of Guangxi Medical UniversityNanning 530031Guangxi Zhuang Autonomous RegionChina

BACKGROUND Despite continuous changes in treatment methods,the survival rate for advanced hepatocellular carcinoma(HCC)patients remains low,highlighting the importance of diagnostic methods for *** To explore the efficacy of texture analysis based on multi-parametric magnetic resonance(MR)imaging(MRI)in predicting microvascular invasion(MVI)in preoperative *** This study included 105 patients with pathologically confirmed HCC,categorized into MVI-positive and MVI-negative *** employed Original Data Analysis,Principal Component Analysis,Linear Discriminant Analysis(LDA),and Non-LDA(NDA)for texture analysis using multi-parametric MR images to predict preoperative *** effectiveness of texture analysis was determined using the B11 program of the MaZda4.6 software,with results expressed as the misjudgment rate(MCR).RESULTS Texture analysis using multi-parametric MRI,particularly the MI+PA+F dimensionality reduction method combined with NDA discrimination,demonstrated the most effective prediction of MVI in *** accuracy in the pulse and equilibrium phases was 83.81%.MCRs for the combination of T2-weighted imaging(T2WI),arterial phase,portal venous phase,and equilibrium phase were 22.86%,16.19%,20.95%,and 20.95%,*** area under the curve for predicting MVI positivity was 0.844,with a sensitivity of 77.19%and specificity of 91.67%.CONCLUSION Texture analysis of arterial phase images demonstrated superior predictive efficacy for MVI in HCC compared to T2WI,portal venous,and equilibrium *** study provides an objective,non-invasive method for preoperative prediction of MVI,offering a theoretical foundation for the selection of clinical therapy.

关键词： Magnetic resonance imaging Hepatocellular carcinoma Texture analysis Microvascular invasion

Rapid development of double-hit mRNA antibody cocktail against orthopoxviruses

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Signal Transduction and Targeted Therapy 2024年第4期9卷 1768-1776页

作者： Hang Chi Suo-Qun Zhao Ru-Yi Chen Xing-Xing Suo rong-rong Zhang Wen-Hui yang Dong-Sheng Zhou Min Fang Bo Ying Yong-Qiang Deng Cheng-Feng qin State Key Laboratory of Pathogen and Biosecurity Beijing Institute of Microbiology and EpidemiologyAMMS100071BeijingChina College of Life Sciences Inner Mongolia Agricultural UniversityHohhot010018Inner MongoliaChina School of Life Sciences Henan UniversityKaifeng475004HenanChina Suzhou Abogen Biosciences Co. LtdSuzhou215123JiangsuChina Research Unit of Discovery and Tracing of Natural Focus Diseases Chinese Academy of Medical Sciences100071BeijingChina

The Orthopoxvirus genus,especially variola virus(VARV),monkeypox virus(MPXV),remains a significant public health threat *** development of therapeutic antibodies against orthopoxviruses is largely hampered by the high cost of antibody engineering and manufacturing ***-encoded antibodies have emerged as a powerful and universal platform for rapid antibody ***,by using the established lipid nanoparticle(LNP)-encapsulated mRNA platform,we constructed four mRNA combinations that encode monoclonal antibodies with broad neutralization activities against *** vivo characterization demonstrated that a single intravenous injection of each LNP-encapsulated mRNA antibody in mice resulted in the rapid production of neutralizing *** importantly,mRNA antibody treatments showed significant protection from weight loss and mortality in the vaccinia virus(VACV)lethal challenge mouse model,and a unique mRNA antibody cocktail,Mix2a,exhibited superior in vivo protection by targeting both intracellular mature virus(IMV)-form and extracellular enveloped virus(EEV)-form *** summary,our results demonstrate the proof-of-concept production of orthopoxvirus antibodies via the LNP-mRNA platform,highlighting the great potential of tailored mRNA antibody combinations as a universal strategy to combat orthopoxvirus as well as otheremerging viruses.

关键词： proof universal neutral

Vermiform Ni@CNT derived from one-pot calcination of Ni-MOF precursor for improving hydrogen storage of MgH_(2)

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Transactions of Nonferrous Metals Society of China 2024年第8期34卷 2629-2644页

作者： Zi-yin DAI Bing ZHANG Hideo KIMURA Li-rong XIAO rong-han LIU Cui NI Chuan-xin HOU Xue-qin SUN Yu-ping ZHANG Xiao-yang yang rong-hai YU Wei DU Xiu-bo XIE School of Environmental and Material Engineering Yantai UniversityYantai 264005China Key Laboratory of Aerospace Materials and Performance Ministry of EducationSchool of Materials Science and EngineeringBeihang UniversityBeijing 100191China

The Ni-coated carbon nanotubes(Ni@CNT)composite was synthesized by the facile“filtration+calcination”of Ni-based metal−organic framework(MOF)precursor and the obtained composite was used as a catalyst for MgH_(2).MgH_(2)was mixed evenly with different amounts of Ni@CNT(2.5,5.0 and 7.5,wt.%)through ball *** MgH_(2)−5wt.%Ni@CNT can absorb 5.2 wt.%H_(2)at 423 K in 200 s and release about 3.75 wt.%H_(2)at 573 K in 1000 *** its dehydrogenation and rehydrogenation activation energies are reduced to 87.63 and 45.28 kJ/mol(H_(2)).The in-situ generated Mg_(2)Ni/Mg_(2)NiH4 exhibits a good catalytic effect due to the provided more diffusion channels that can be used as“hydrogen pump”.And the presence of carbon nanotubes improves the properties of MgH_(2)to some extent.

关键词： Mg-based hydrogen storage material activation energy Ni-loaded carbon nanotubes catalyst mechanism

Demonstration of normally-off p-channel GaN transistor with high threshold voltage and low subthreshold swing based on single p-GaN

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Science China(Information Sciences) 2024年第09期67卷 348-349页

作者： yangfeng LI Zian DONG Shuai CHEN qin WANG Tong LI Shulin CHEN Kun ZHENG Jie ZHANG Guojian DING yang WANG Haiqiang JIA rong yang Lei LIAO Changsha Semiconductor Technology and Application Innovation Research Institute College of Semiconductors (College of Integrated Circuits) Hunan University Hunan San'an Semiconductor Co. Ltd. Songshan Lake Materials Laboratory

GaN outperforms silicon in applications with high power and high frequency owing to the high critical electric field and high electron mobility. However, the hole-based GaN transistors which are pivotal to the GaN-based CMOS circuits and high-side switching are still under investigation. Two-dimensional hole gas(2DHG) has been used to demonstrate the p-channel GaN field-effect-transistors(FETs) [1–5].

关键词： MOSFETS

Global status and trends of metabolomics in diabetes: A literature visualization knowledge graph study

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World Journal of Diabetes 2024年第5期15卷 1021-1044页

作者： Hong Li Liu Li Qiu-qing Huang Si-Yao yang Jun-Ju Zou Fan Xiao qin Xiang Xiu Liu rong Yu College of Chinese Medicine Hunan University of Chinese MedicineChangsha 410208Hunan ProvinceChina College of International Education Hunan University of Chinese MedicineChangsha 410208Hunan ProvinceChina Department of Science and Technology Hunan University of Chinese MedicineChangsha 410208Hunan ProvinceChina Hunan Key Laboratory of TCM Prescription and Syndromes Translational Medicine Hunan University of Chinese MedicineChangsha 410208Hunan ProvinceChina College of Graduate Hunan University of Chinese MedicineHunan ChangshaHunan ProvinceChina

BACKGROUND Diabetes is a metabolic disease characterized by hyperglycemia,which has increased the global medical burden and is also the main cause of death in most *** To understand the knowledge structure of global development status,research focus,and future trend of the relationship between diabetes and metabolomics in the past 20 *** The articles about the relationship between diabetes and metabolomics in the Web of Science Core Collection were retrieved from 2002 to October 23,2023,and the relevant information was analyzed using CiteSpace6.2.2R(CiteSpace),VOSviewer6.1.18(VOSviewer),and Bibliometrix software under R *** A total of 3123 publications were included from 2002 to *** the past two decades,the number of publications and citations in this field has continued to *** United States,China,Germany,the United Kingdom,and other relevant funds,institutions,and authors have significantly contributed to this *** Reports and PLoS One are the journals with the most publications and the most *** keyword co-occurrence and cluster analysis,the closely related keywords are"insulin resistance","risk","obesity","oxidative stress","metabolomics","metabolites"and"biomarkers".Keyword clustering included cardiovascular disease,gut microbiota,metabonomics,diabetic nephropathy,molecular docking,gestational diabetes mellitus,oxidative stress,and insulin *** detection analysis of keyword depicted that"Gene","microbiota","validation","kidney disease","antioxidant activity","untargeted metabolomics","management",and"accumulation"are knowledge frontiers in recent *** The relationship between metabolomics and diabetes is receiving extensive *** nephropathy,diabetic cardiovascular disease,and kidney disease are key diseases for future research in this *** microbiota,molecular docking,and untargeted metabolomics are key research directions in the *** a

关键词： Diabetes Metabolomics Bibliometric CiteSpace VOSviewer

Making waste profitable: Efficient recovery of metallic iron from jarosite residues

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Chinese Journal of Chemical Engineering 2024年第7期71卷 66-76页

作者： Songshan Zhou Yunhui Han rong Huang Yin Huang qingyuan Dong Haiyin Gang Jinchuan qin Xi Yu Xiangfei Zeng Wenxing Cao Jiqin Wang Shaoqin Chen rong Wang Mengjun Chen Key Laboratory of Solid Waste Treatment and Resource Recycle(SWUST) Ministry of EducationSouthwest University of Science and TechnologyMianyang 621010China School of Environment Key Laboratory of Yellow River and Huai River Water Environment and Pollution ControlMinistry of EducationHenan Normal UniversityXinxiang 453007China School of National Defense Science and Technology College SWUST Ministry of EducationSouthwest University of Science and TechnologyMianyang 621010China

To address the hazardous by-product of zinc smelting and resource utilization of jarosite residue,this study applies an electric field-assisted hot acid treatment to completely recycle iron(Fe).This innovative approach aims to enhance the leaching efficiency of Fe from jarosite *** introduction of an electric field changes the charge distribution on the surface of the particles to enhance ions and electrons exchange and promotes the collision between particles to strengthen reaction *** on the above,the leaching efficiency of Fe in jarosite under sulfuric acid attack has improved *** result shows that Fe leaching efficiency reaches 98.83%,which is increased by 28%under the optimal experimental conditions:current density of 30 mA·cm^(-2),H_(2)SO_(4) concentration of 1.5 mol·L^(-1),solid-liquid ratio of 70 g·L^(-1),temperature of 80℃ and time of 12 *** kinetics calculations show that the apparent activation energy is 16.97 kJ·mol^(-1) and the leaching of jarosite residue is controlled by a mixture of chemical reaction and diffusion,as well as the temperature and concentration of the leaching solution have an influence on *** work provides a feasible idea for the efficient leaching of Fe from jarosite residue.

关键词： Jarosite residue Electric filed Leaching kinetics