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Analysis of Phenotypes Associated with Deficiency of PAX6 Haplotypes in Chinese Aniridia Families

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当代医学科学（英文） 2024年第4期44卷 820-826页

作者： Xiao-lu HAO Ran CHEN Wei LIU Bao-ke hou Ling-hui QU Zhao-hui LI Da-jiang WANG xin JIN hou-bin HUANG Senior Department of Opthalmology the Third Medical Center of Chinese PLA General HospitalBeijing 100039China Bolin Eye Care Group Beijing 100098China Department of Ophthalmology Hainan Hospital of Chinese PLA General HospitalSanya 572000China Department of Ophthalmology the74th Army Group HospitalGuangzhou 510318China

Objective:To examine the clinical phenotype and genetic deficiencies present in Chinese aniridia families with PAX6 haplotype ***:A comprehensive questionnaire and ophthalmological assessments were administered to both affected patients and unaffected *** clinical feature analysis included the evaluation of visual acuity,intraocular pressure,slit-lamp anterior segment examination,fundus photography,and spectral domain optical coherence *** identify the mutation responsible for aniridia,targeted next-generation sequencing was used as a beneficial ***:A total of 4 mutations were identified,consisting of two novel frameshift mutations(c.314delA,p.K105Sfs*33 and c.838_845dup AACACACC,p.S283Tfs*85),along with two recurring nonsense mutations(c.307C＞T,p.R103X and c.619A＞T,p.K207*).Complete iris absence,macular foveal hypoplasia,and nystagmus were consistent in these PAX6 haplotype-deficient Chinese aniridia families,while corneal lesions,cataracts,and glaucoma exhibited heterogeneity both among the families and within the same ***:In our study,two novel PAX6 mutations associated with aniridia were identified in Chinese families,which expanded the phenotypic and genotypic spectrum of PAX6 *** also analyzed the clinical characteristics of PAX6 haplotype deficiency in Chinese aniridia families.

关键词： PAX6 aniridia genotype haplotype deficiency mutation phenotype

浅海环境中用于目标深度属性判别的线谱起伏特征量分析

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物理学报 2024年第13期73卷 175-189页

作者：邓玉鑫刘雄厚杨益新西北工业大学航海学院西安710072 陕西省水下信息技术重点实验室西安710072

浅海环境中目标辐射线谱的起伏特征可用于水面、水下目标深度属性判别.已有研究未给出表征水面、水下线谱起伏差异的物理量性质,导致利用不同物理量进行目标深度属性判别时性能不佳.针对这一问题,本文从声源深度起伏所导致的线谱起伏出... 详细信息

浅海环境中目标辐射线谱的起伏特征可用于水面、水下目标深度属性判别.已有研究未给出表征水面、水下线谱起伏差异的物理量性质,导致利用不同物理量进行目标深度属性判别时性能不佳.针对这一问题,本文从声源深度起伏所导致的线谱起伏出发,采用微分方法与统计方法对线谱起伏规律进行分析,研究了线谱起伏产生的物理机理,得到了适合用于水面、水下目标深度属性判别的物理量.首先,分别推导了复声压实部、声功率与声压幅值关于声源深度的导数关系式,得到了以上三种物理量在声源深度起伏时的起伏大小;其次,利用仿真实验数据从接收深度、声源水平距离、线谱频率等层面对理论推导进行验证,并对线谱的归一化起伏特征进行了分析.最后,利用海试数据验证了相关结论.结果表明,当声源的深度起伏时,水面、水下线谱的起伏差异源自各阶模态的相互作用.声源频率、声源水平距离、接收深度三种因素决定了模态间相互作用的性质.声压幅值适用于构建目标深度属性判别特征量,可用于表征低频线谱的强度起伏差异.数值仿真和海试数据处理结果表明,相较于声压实部和声功率,利用声压幅值构建的归一化水面、水下目标深度属性判别特征量具有更优性能.

关键词：线谱起伏深度属性判别浅海环境简正波

Efficient piezo-catalytic dye degradation using piezoelectric 6H-SiC under harsh conditions

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Rare Metals 2024年第7期43卷 3173-3184页

作者： Lin-Lin Zhou Tao Yang Kang Wang Lai-Pan Zhu En-Hui Wang Kuo-Chih Chou Hai-Long Wang xin-Mei hou Institute for Carbon Neutrality University of Science and Technology BeijingBeijing 100083China Institute of Steel Sustainable Technology Liaoning Academy of MaterialsShenyang 110000China Beijing Institute of Nanoenergy and Nanosystems Chinese Academy of SciencesBeijing 101400China School of Materials Science Engineering Zhengzhou UniversityZhengzhou 450001China Beijing Advanced Innovation Center for Materials Genome Engineering Beijing University of Science and TechnologyBeijing 100083China

The development of novel piezoelectric catalysts against harsh conditions is indeed crucial for improving the piezo-catalytic degradation efficiency of colored organic dyes in *** this work,6H-SiC nanoparticles(NPs)are utilized to piezo-catalytic degrade rhodamine B(RhB)and methylene blue(MB)under ultrasonic vibration for the first *** degradation efficiency of RhB and MB reaches 98.8%and 98.7%within 80 *** piezoelectricity of 6H-SiC is comprehensively analyzed by the piezoresponse force microscope(PFM)and finite element method(FEM).The strong oxidizing active free radicals generated by the continuous piezoelectric polarized electric field of 6H-SiC,i.e.,·O_(2)^(-)and·OH,induce the decomposition reactions of colored organic dyes in *** the dyes are proven to degrade to harmless or less-harmful products gradually during the piezo-catalysis process by high-performance liquid chromatography tandem mass spectrometry(HPLC-MS).Moreover,RhB is also decomposed efficiently by 6HSiC NPs under acidic and alkaline *** results prove the feasibility of 6H-SiC for decomposing common water pollutants under harsh conditions and provide a new perspective for water purification.

关键词： Piezo-catalytic degradation 6H-SiC Rhodamine B(RhB) Methylene blue(MB) Harsh conditions

Dual-Confined Bead-Like CoSe_(2)@NC@NCNFs Bifunctional Catalyst Boosting Rechargeable Zinc-Air Batteries

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Chinese Journal of Chemistry 2024年第4期42卷 397-405页

作者： Kuixing Ding Jiugang Hu Liming Zhao Huanan Yu Shan Cai yi Yang Jun Tan Hongshuai hou Xiaobo Ji College of Chemistry and Chemical Engineering Central South UniversityChangshaHunan410083 China College of Materials and Chemistry China Jiliang UniversityHangzhouZhejiang310018 China College of Standardization China Jiliang UniversityHangzhouZhejiang310018 China

Rationally developing efficient and durable bifunctional catalysts toward oxygen reduction reaction(ORR)and oxygen evolution reaction(OER)is critical for rechargeable zinc-air batteries(ZABs).Herein,a bead-like CoSe_(2)@NC@NCNFs bifunctional catalyst was designed and fabricated by confining cubic CoSe_(2)nanoparticles to three-dimensional(3D)porous MOFs-derived nitrogen-doped carbon(NC)and one-dimensional(1D)N-doped carbon nanofibers(NCNFs)through a facile encapsulate *** 1D/3D continuous network structure contributes to the improvement of specific surface area and electronic conductivity,while the strong synergistic effect between CoSe_(2)sites and Co-Nx-C sites can effectively enhance electron/mass transfer and reduce the diffusion *** as-constructed CoSe_(2)@NC@NCNFs catalyst exhibits high catalytic activity and stability toward ORR/OER with a high half-wave potential of 0.80 V(***)in ORR and a low overpotential of 280 mV at 10 mA·cm^(−2)in *** encouragingly,the rechargeable ZABs with CoSe_(2)@NC@NCNFs cathode deliver high peak power densities(126.8 mW·cm^(−2)),large specific capacities(763.1 mA·h·g^(−1)),and robust charge-discharge cycling stability over 240 *** study provides a facile strategy for designing efficient bifunctional catalysts for rechargeable energy conversion applications.

关键词： Dual-confined Electrospinning Cobalt selenide Bifunctional catalysts Rechargeable zinc-air batteries

Tongxinluo Activates PI3K/AKT Signaling Pathway to Inhibit Endothelial Mesenchymal Transition and Attenuate Myocardial Fibrosis after Ischemia-Reperfusion in Mice

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Chinese Journal of Integrative Medicine 2024年第7期30卷 608-615页

作者： WEI Ya-ru hou Yun-long yiN Yu-jie LI Zhen LIU yi HAN Ning-xin WANG Zi-xuan LIU Lu WANG Xiao-qi HAO Yuan-jie MA Kun GU Jiao-jiao JIA Zhen-hua Graduate School Hebei University of Chinese MedicineShijiazhuang050090China Shijiazhuang yiling Pharmaceutical New Drug Evaluation Center Shijiazhuang050035China Hebei Institute of Integrated Traditional and Western Medicine Shijiazhuang050035China National Key Laboratory for Innovation and Transformation of Luobing Theory Shijiazhuang050035China Department of Cardiology Hebei Yiling HospitalShijiazhuang050091China Graduate School Hebei Medical UniversityShijiazhuang050017China

Objective To investigate the potential role of Tongxinluo(TXL)in attenuating myocardial fibrosis after myocardial ischemia-reperfusion injury(MIRI)in *** A MIRI mouse model was established by left anterior descending coronary artery ligation for 45 *** to a random number table,66 mice were randomly divided into 6 groups(n=11 per group):the sham group,the model group,the LY-294002 group,the TXL group,the TXL+LY-294002 group and the benazepril(BNPL)*** day after modeling,TXL and BNPL were administered by *** injection of LY-294002 was performed twice a week for 4 consecutive *** was used to measure cardiac function in *** staining was used to evaluate the degree of myocardial fibrosis in *** and quantitative analysis of endothelial mesenchymal transition(EndMT)after MIRI was performed by immunohistochemistry,immunofluorescence staining and flow cytometry,*** protein expressions of platelet endothelial cell adhesion molecule-1(CD31),α-smoth muscle actin(α-SMA),phosphatidylinositol-3-kinase(PI3K)and phospho protein kinase B(p-AKT)were assessed using Western *** TXL improved cardiac function in MIRI mice,reduced the degree of myocardial fibrosis,increased the expression of CD31 and inhibited the expression ofα-SMA,thus inhibited the occurrence of EndMT(P0.05).In addition,the use of the PI3K/AKT pathway-specific inhibitor LY-294002 to block this pathway and combination with TXL intervention,eliminated the protective effect of TXL,further supporting the protective effect of *** TXL activated the PI3K/AKT signaling pathway to inhibit EndMT and attenuated myocardial fibrosis after MIRI in mice.

关键词： myocardial fibrosis endothelial mesenchymal transition myocardial ischemia-reperfusion injury phosphatidylinositol-3-kinase/protein kinase B(PI3K/AKT)pathway

Fusarium mycotoxins:The major food contaminants

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mLife 2024年第2期3卷 176-206页

作者： Zheng Qu Xianfeng Ren Zhaolin Du Jie hou Ye Li Yanpo Yao yi An Agro‐Environmental Protection Institute Ministry of Agriculture and Rural AffairsTianjinChina Institute of Quality Standard and Testing Technology for Agro‐Products Shandong Academy of Agricultural SciencesJinanChina

Mycotoxins,which are secondary metabolites produced by toxicogenic fungi,are natural food toxins that cause acute and chronic adverse reactions in humans and *** genus Fusarium is one of three major genera of mycotoxin‐producing ***,fumonisins,and zearalenone are the major Fusarium mycotoxins that occur *** mycotoxins have the potential to infiltrate the human food chain via contamination during crop production and food processing,eventually threatening human *** occurrence and development of Fusarium mycotoxin contamination will change with climate change,especially with variations in temperature,precipitation,and carbon dioxide *** address these challenges,researchers have built a series of effective models to forecast the occurrence of Fusarium mycotoxins and provide guidance for crop *** mycotoxins frequently exist in food products at extremely low levels,thus necessitating the development of highly sensitive and reliable detection *** successful detection methods have been developed to meet the requirements of various situations,and an increasing number of methods are moving toward highthroughput *** Fusarium mycotoxins cannot be completely eliminated,numerous agronomic,chemical,physical,and biological methods can lower Fusarium mycotoxin contamination to safe levels during the preharvest and postharvest *** theoretical innovations and technological advances have the potential to facilitate the development of comprehensive strategies for effectively managing Fusarium mycotoxin contamination in the future.

关键词： climate change Fusarium mycotoxin management strategy mycotoxin detection

Transcriptome-Wide Identification and Functional Analysis of PgSQE08-01 Gene in Ginsenoside Biosynthesis in Panax ginseng ***.

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Phyton-International Journal of Experimental Botany 2024年第2期93卷 313-327页

作者： Lei Zhu Lihe hou Yu Zhang Yang Jiang yi Wang Meiping Zhang Mingzhu Zhao Kangyu Wang College of Life Science Jilin Agricultural UniversityChangchun130118China Jilin Engineering Research Center Ginseng Genetic Resources Development and Utilization Changchun130118China Jilin Academy of Vegetable and Flower Sciences Changchun130033China

Panax ginseng *** an important plant species used in traditional Chinese medicine,whose primary active ingredient is a *** biosynthesis is not only regulated by transcription factors but also controlled by a variety of structural ***,the molecular mechanism underlying ginsenoside biosynthesis has always been a topic in the discussion of ginseng secondary *** epoxidase(SQE)is a key enzyme in the mevalonic acid pathway,which affects the biosynthesis of secondary metabolites such as *** ginseng transcriptome,expression,and ginsenoside content databases,this study employed bioinformatic methods to systematically analyze the genes encoding SQE in *** first selected six PgSQE candidates that were closely involved in ginsenoside biosynthesis and then identified PgSQE08-01 to be highly associated with ginsenoside ***,we constructed the overexpression vector pCAMBIA3301-PgSQE08-01 and the RNAi vector pART27-PgSQE08-01 and transformed ginseng adventitious roots using Agrobacterium rhizogenes,to obtain positive hairy-root ***,quantitative reverse transcriptionpolymerase chain reaction and high-performance liquid chromatography were used to determine the expression of relevant genes and ginsenoside content,***,we focused on the function of PgSQE08-01 gene,which was noted to be involved in ginsenoside ***,these findings not only provided a molecular basis for the identification of important functional genes in ginseng but also enriched genetic resources for the biosynthesis of ginsenosides using synthetic biology.

关键词： Panax ginseng pgSQE08-01 gene squalene epoxidase ginsenoside ginseng hairy roots

Highly efficient electrosynthesis of H_(2)O_(2)in acidic electrolyte on metal-free heteroatoms co-doped carbon nanosheets and simultaneously promoting Fenton process

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Chinese Chemical Letters 2024年第7期35卷 514-518页

作者： Xiaodan Wang yingnan Liu Zhibin Liu Zhongjian Li Tao Zhang yi Cheng Lecheng Lei Bin Yang Yang hou Key Laboratory of Biomass Chemical Engineering of Ministry of Education College of Chemical and Biological EngineeringZhejiang UniversityHangzhou 310058China Institute of Zhejiang University-Quzhou Quzhou 324000China Donghai Laboratory Zhoushan 316000China Ningbo Institute of Materials Technology&Engineering University of Chinese Academy of SciencesNingbo 315200China School of Biological and Chemical Engineering NingboTech UniversityNingbo 315100China Zhejiang Hengyi Petrochemical Research Institute Co. Ltd.Hangzhou 310058China

Recently electrochemical synthesis of H_(2)O_(2)through oxygen reduction reaction(ORR)via 2e^(-)pathway is considered as a green and on-site ***,it still remains a big challenge for fabricating novel metal-free catalysts under acidic solutions,since it suffers from high overpotential due to the intrinsically week*OOH ***,a co-doped carbon nanosheet(O/N–C)catalyst toward regulating O and N content was synthesized for improving the selectivity and activity of H_(2)O_(2)electrosynthesis *** O/N–C exhibits outstanding 2e-ORR performance with low onset potential of 0.4 V(***)and a selectivity of 92.4%in 0.1 mol/L HClO_(4)*** in situ electrochemical impedance spectroscopy(EIS)tests reveals that the N incorporation contributes to the fast ORR *** density functional theory(DFT)calculations demonstrate that the binding strength of*OOH was optimized by the co-doping of oxygen and nitrogen at certain content,and the O/N–C–COOH site exhibits a lower theoretical overpotential for H_(2)O_(2)formation than O–C–COOH ***,the promoted kinetics for typical organic dye degradation in simultaneous electron-Fenton process on O/N–C catalyst was demonstrated particularly for broadening its environmental application.

关键词： Oxygen reduction Metal-free catalyst H_(2)O_(2)electrosynthesis N doping Carbon materials

Preparation of Laser Cladding Coating Undercooling Cu-based Alloy and Co on Non-equilibrium Solidification Structure

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Journal of Wuhan University of Technology(Materials Science) 2024年第2期39卷 463-472页

作者：田徐铭 CAO Shichao hou Kai hou Xiaopeng WANG Hongfu 张煜 Department of Outpatient Xinzhou People’s HospitalXinzhou 034099China School of Mechanical Engineering North University of ChinaTaiyuan 030051China Department of Neurosurgery Xinzhou People’s HospitalXinzhou 034099China Department of Orthopedics Xinzhou People’s HospitalXinzhou 034099China

The effect of the gradient content of Co element on the solidification process of Cu-based alloy under deep under cooling conditions was *** non-equilibrium solidification structure of the under cooled alloy samples were *** is found that the rapidly solidified alloy has undergone twice grain refinement during the undercooling *** and significance of the maximum undercooling refinement structure of Cu60Ni35Co5 at T=253 K were ***-density defects were observed,such as dislocations,stacking faults networks,and twinning *** standard FCC diffraction pattern represents that it is still a single-phase *** on the metallographic diagram,EBSD and TEM data analysis,it is illustrated that the occurrence of grain refinement under high undercooling is due to stress induced *** addition,the laser cladding technology is used to coat Co-based alloy(Stellite12) coating on 304 stainless steel substrate;the microstructure of the coating cross-section was *** was found that the microstructure of the cross-section is presented as columnar crystals,planar crystals,and disordered growth direction,so that the coating has better hardness and wear *** electrochemical corrosion of the substrate and coating,it can be seen that the Co and Cr elements present in the coating are more likely to form a dense passivation film,which improved the corrosion resistance of the coating.

关键词： non-equilibrium solidification structure undercooling recrystallization laser cladding coating