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MPDZ variants associated with epilepsies and/or febrile seizures and the individualized genotype-phenotype correlation

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Genes & Diseases 2024年第3期11卷 124-127页

作者： Junxia Luo Yun Li Yong Lv Xin Li Bing Qin Chuanfang Cheng Xiaorong Liu weiping liao Jie Wang Zaifen Gao Department of Epilepsy Center Children's Hospital Affiliated to Shandong UniversityJinanShandong 250022China Department of Epilepsy Center Jinan Children's HospitalJinanShandong 250022China Department of Brain Function and Neuroelectrophysiology The Affiliated Nanhua HospitalHengyang Medical SchoolUniversity of South ChinaHengyangHunan 421001China Department of Pediatrics The First Affiliated Hospital of University of Science and Technology of China(Anhui Provincial Hospital)HefeiAnhui 230001China Department of Pediatrics The Second HospitalCheeloo College of MedicineShandong UniversityJinanShandong 250033China Epilepsy Center and Department of Neurosurgery The First Affliated Hospital of Jinan UniversityGuangzhouGuangdong 510630China Department of Cardiology The Second Afiliated Hospital of Guangzhou Medical UniversityGuangzhouGuangdong 510260China Institute of Neuroscience and Department of Neurology of the Second Affiliated Hospital of Guangzhou Medical University GuangzhouGuangdong 510260China Key Laboratory of Neurogenetics and Channelopathies of Guangdong Province and the Ministry of Education of China GuangzhouGuangdong 510260China

The multiple PDZ domain crumbs cell polarity complex component gene(MPDZ;MIM:603785),is highly expressed in the brain across the whole lifespan.It encodes the multiple PDZ domain protein,which is a member of the NMDAR signaling complex that may play a role in the control of AMPAR potentiation and synaptic plasticity in excitatory synapses."Previously,MPDZ variants have been demonstrated to be associated with autosomal recessive congenital hydrocephalus-2(HYC2;MIM:615219)which is commonly complicated by brain abnormalities and developmental delay.Seizures were reported in only one case.The association between MPDz and epilepsy requires clarification.

关键词： epilepsy febrile congenital

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Improvement of low temperature activity and stability of Ni catalysts with addition of Pt for hydrogen production via steam reforming of ethylene glycol

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Green Energy & Environment 2019年第3期4卷 300-310页

作者： Xingling Zhao Kai Wu weiping liao Yingxiong Wang Xiaoning Hou Mingshan Jin Zhanghuai Suo Hui Ge Institute of Applied Catalysis Yantai University30 Qingquan RoadYantai 264005ShangdongChina State Key Laboratory of Coal Conversion Institute of Coal ChemistryChinese Academy of SciencesTaiyuan 030001ShanxiChina

Hydrogen production by steam reforming of ethylene glycol(EG) at 300℃ was investigated over SiO2 and CeO2 supported Pt–Ni bimetallic catalysts prepared by incipient wetness impregnation methods. It was observed that impregnation sequence of Pt and Ni can affect the performance of catalysts apparently. Catalyst with Pt first and then Ni addition showed higher EG conversion and H2 yield owing to the Ni enrichment on the surface and the proper interaction between Pt and Ni. It was observed that although SiO2 supported catalysts exhibited better activity and H2 selectivity, CeO2 supported ones had better stability. This is attributed to the less coke formation on CeO2. Increasing Pt/Ni ratio enhanced the reaction activity, and Pt3–Ni7 catalysts with 3 wt% Pt and 7 wt% Ni showed the highest activity and stability. Ni surficial enrichment facilitated the C-C bond rupture and water gas shift reactions;and Pt addition inhibited methanation reaction. Electron transfer and hydrogen spillover from Pt to Ni suppressed carbon deposition. These combined effects lead to the excellent performance of Pt3–Ni7 supported catalysts.

关键词： Ethylene glycol Steam reforming Pt–Ni bimetallic catalyst Hydrogen production Synergistic effect

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Rational construction of metal–base synergetic sites on Au/Mg-beta catalyst for selective aerobic oxidation of 5-hydroxymethylfurfural

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Journal of Energy Chemistry 2021年第11期30卷 599-609,I0014页

作者： Zhiguo Zhu Xiongjie Gao Xiuming Wang Mengdie Yin Qingyao Wang Wanzhong Ren Bo Wang Hongying Lü weiping liao College of Chemistry and Chemical Engineering Yantai University30 Qingquan RoadYantai 264005ShandongChina Library Yantai University30 Qingquan RoadYantai 264005ShandongChina Shandong Provincial Key Laboratory of Chemical Energy Storage and Novel Cell Technology School of Chemistry and Chemical EngineeringLiaocheng UniversityLiaocheng 252059ShandongChina

The selective aerobic oxidation of biomass-derived 5-hydroxymethylfurfural to 2,5-furandicarboxylic acid (FDCA, a potential renewable substitution of fossil-based terephthalic acid to produce polyethylene 2,5-furandicarboxylate plastic) is an appealing transformation for constructing eco-friendly and sustainable chemical processes. Au supported catalysts have showed encouraging performances for this well-received conversion, whose catalytic behavior was greatly affected by the adopted support derived from the existence of metal-support interactions. Herein, a series of Mg-Beta zeolites were hydrothermally synthesized via developed structural reconstruction, which were employed as basic supports for Au catalysts to construct bifunctional catalysts. The relationship between structure (Au particle size, basicity within zeolites and Auδ+ contents) and FDCA yield was concretely established. The conclusion was made that the utilization of Mg-Beta zeolites with strong basicity as the support could not only improve the FDCA yield but also decrease the amount of additional base. Furthermore, the possible reaction mechanism was also proposed via tracking time-dependent variations of corresponding organics and controlled experiment. This work provides some guidance for rationally designing multifunctional catalysts in the view of integrating metal catalysts with metallosilicate zeolites, which was beneficial to the catalytic upgrading of organic compounds with multiple functional groups.

关键词： 5-Hydroxymethylfurfural Aerobic oxidation 2,5-Furandicarboxylic acid Bifunctional Au/Mg-Beta catalyst Structural reconstruction

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Synthesis and applications of mesoporous Cu-Zn-Al_2O_3 catalyst for dehydrogenation of 2-butanol

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Journal of Natural Gas Chemistry 2009年第2期18卷 179-182页

作者： Deren Fang Wanzhong Ren Zhongmin Liu Xiufeng Xu Lei Xu Hongying Lǔ weiping liao Huimin Zhang Dalian Institute of Chemical Physics Chinese Academy of Sciences Dalian 116023 Liaoning China Institute of Applied Catalysis of Yantai University Yantai 264005 Shandong China

A series of mesoporous Cu-Zn-Al2O3 materials have been synthesized at ambient temperature and their structure was characterized by XRD, N2 physical adsorption and TPR techniques. Their catalytic applications for the dehydrogenation of 2-butanol to methyl ethyl ketone （MEK） were evaluated in a fixed-bed flow reactor at atmospheric pressure. It is demonstrated from the XRD patterns that both the as-synthesized samples and calcined samples have the typical XRD patterns of meso-structured materials and the results of N20 chemical adsorption showed that Cu was embedded in the framework of the mesoporous materials and homogeneously dispersed in the mesoporous Cu-Zn-Al2O3 materials. The catalytic activity of 2-butanol dehydrogenation was varied in the order of CZA（10）〈 CZA（CP）〈 CZA（20）〈 CZA（30）; while the selectivity of MEK was increased in the order of CZA（CP）〈 CZA（10）〈 CZA（20）〈CZA（30）.

关键词： Cu-Zn-Al2O3 mesoporous materials dehydrogenation of 2-butanol catalyst

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广西气象培训师资建设特征与对策研究

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气象研究与应用 2024年第1期45卷 132-136页

作者：王君华廖伟平姜辅嫄王玉方陈瑾广西壮族自治区气象科学研究所南宁530022 广西壮族自治气象局机关服务中心南宁530022 广西壮族自治区气象局南宁530022

广西气象事业高质量发展对广西气象干部教育培训提出更高的要求。通过对广西气象系统92名专职和兼职教师基本信息及课件资源等资料进行统计分析,并开展调研,归纳出广西气象培训师资建设主要特征:以兼为主、来源广泛,结构合理、素质优良... 详细信息

广西气象事业高质量发展对广西气象干部教育培训提出更高的要求。通过对广西气象系统92名专职和兼职教师基本信息及课件资源等资料进行统计分析,并开展调研,归纳出广西气象培训师资建设主要特征:以兼为主、来源广泛,结构合理、素质优良,资源丰富、务实管用。发现存在几个问题:专业教师结构和能力仍存不足,兼职教师能力素质有待进一步提高,基于信息技术的师资库还未建立。分析造成现状的原因并提出针对性强的对策,以期发挥教育培训在气象干部队伍建设中的先导性、基础性、战略性作用,着力培养造就忠诚、干净、担当的高素质气象干部队伍。

关键词：广西气象教育培训师资建设

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Microfluidic separation of particles by synergistic effect of geometry-induced hydrodynamics and magnetic field

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Chinese Chemical Letters 2024年第2期35卷 380-385页

作者： Du Qiao Hongxia Li weiping Zhu Lili Zhu Danyang Zhao Honglin Li Key Laboratory for Precision & Non-traditional Machining Technology of Ministry of Education Dalian University of TechnologyDalian 116023China Shanghai Key Laboratory of New Drug Design School of PharmacyEast China University of Science and TechnologyShanghai 200237China Lingang Lab Shanghai 200031China

Microfluidic combined with magnetic field have been demonstrated to be the promising solutions for fast and low-damage particles separation.However,the difficulties in the precise layout of magnets and accurate prediction of particle trajectories lead to under and over separation of target particles.A novel particle separation lab-on-chip(LOC)prototype integrated with microstructures and micropolar arrays is designed and characterized.Meanwhile,a numerical model for the separation of magnetic particles by the synergistic effect of geometry-induced hydrodynamics and magnetic field is constructed.The effect of geometry and magnetic field layout on particle deflection is systematically analyzed to implement accurate prediction of particle trajectories.It is found that the separation efficiency of magnetic particles increased from 50.2%to 91.7%and decreased from 88.6%to 85.7%in the range of depth factors from 15µm to 27µm and width factors from 30µm to 60µm,respectively.In particular,the combined effect of the offset distance of permanent magnets and the distance from the main flow channel exhibits a significant difference from the conventional perception.Finally,the developed LOC prototype was generalized for extension to arbitrary systems.This work provides a new insight and robust method for the microfluidic separation of magnetic particles.

关键词： Microfluidic Particle separation Synergistic effect Hydrodynamics Magnetic field Numerical calculation

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Mechanism of nanoparticle aggregation in gas-liquid microfluidic mixing

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Chinese Chemical Letters 2024年第4期35卷 376-381页

作者： Hongxia Li Xiyang Wang Du Qiao Jiahao Li weiping Zhu Honglin Li Key Laboratory for Precision&Non-traditional Machining Technology of Ministry of Education Dalian University of TechnologyDalian 116023China Shanghai Key Laboratory of New Drug Design School of PharmacyEast China University of Science and TechnologyShanghai 200237China

Using gas-liquid segmented micromixers to prepare nanoparticles that have a homogeneous particle size, controllable shape, and monodispersity advantages. Although nanoparticle aggregation within a microfluid has been shown to be affected by the shear effect, the shear effect triggering conditions in gasliquid two-phase flow is unclear and the aggregation behavior of nanoparticles under the shear effect is difficult to predict, resulting in uncontrollable physical and chemical properties of nanoparticle aggregates. In this study, a numerical simulation of nanoparticle aggregation in gas-liquid two-phase flow under the shear effect is performed using the CFD-DEM method. Then, the effects of total flow rate,gas-liquid two-phase flow ratio, and particle volume fraction on particle aggregation were analyzed to achieve control of particle aggregation shape and size. Meanwhile, the triggering mechanism of the shear effect and the mechanism of the shear effect on the aggregation of nanoparticles were clarified. The results show that increasing the total flow rate or decreasing the gas-liquid two-phase flow rate ratio can induce the shear effect, which reduces the particle aggregation size and makes the morphology tend to be spherical. Moreover, increasing the particle volume fraction, and total flow rate or decreasing the gas-liquid two-phase flow rate ratio also increases the number of particle collisions and induce interparticle adhesion. Hence, particle adhesion and the shear effect compete with each other and together affect particle aggregation.

关键词： Microfluidic Gas-liquid segmented micromixer Nanoparticle aggregation Shear effect Numerical calculation

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某新建平台项目生产准备工作质量的提升探索与实践

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天津科技 2022年第6期49卷 17-19页

作者：廖维平中海石油(中国)有限公司天津分公司辽东作业公司天津300452

生产准备指的是在油(气)田开发项目从总体开发方案批准到本项目试运行前的时间内,生产人员为保障该项目的顺利试运行而做的各项准备工作。通过对某新建平台项目生产准备阶段在组建队伍、培训人员、参与设计、工程建造、收集技术资料、... 详细信息

生产准备指的是在油(气)田开发项目从总体开发方案批准到本项目试运行前的时间内,生产人员为保障该项目的顺利试运行而做的各项准备工作。通过对某新建平台项目生产准备阶段在组建队伍、培训人员、参与设计、工程建造、收集技术资料、编写试运行文件、调试运行设备、遗留问题整改等方面工作经验的归纳和提炼,总结出了一系列提升生产准备工作质量的方式和方法,以期为今后类似项目的生产准备工作提供借鉴。

关键词：生产准备经验总结探索提升

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“3+1”综合绩效评价方法在海上油田的应用

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天津科技 2023年第6期50卷 4-6,10页

作者：廖维平中海石油(中国)有限公司天津分公司辽东作业公司天津300452

海洋石油工业作为高风险、高技术、高投入行业,对安全、环保有着极高的要求,而高质量且完善的基础工作是海上油田安全、稳定生产的根基和前提。“3+1”综合绩效评价方法通过设计合理的综合绩效计算公式,建立员工正负面清单,着重对基础... 详细信息

海洋石油工业作为高风险、高技术、高投入行业,对安全、环保有着极高的要求,而高质量且完善的基础工作是海上油田安全、稳定生产的根基和前提。“3+1”综合绩效评价方法通过设计合理的综合绩效计算公式,建立员工正负面清单,着重对基础工作完成情况进行量化,综合考虑员工在创新创效等方面的亮点成绩和团队成员之间工作的认可度,同时对安全环保及廉洁自律有重大过错的员工实行一票否决,以最终树立注重基础、鼓励提升、倡导团结、敬畏规则的油田工作理念。

关键词：海洋石油绩效评价量化理念

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16p11.2 Deletion in Patients with Paroxysmal Kinesigenic Dyskinesia but without Intellectual Disability

16p11.2 Deletion in Patients with Paroxysmal Kinesigenic Dys...

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第六届CAAE脑电图与神经电生理大会

作者： Wen Li Yifan Wang Bin Li Bin Tang Hui Sun Jinxing Lai Na He Bingmei Li Heng Meng weiping liao Xiaorong Liu The Second Affiliated Hospital of Guangzhou Medical University

＜正＞Introduction:Mutations of the PRRT2 gene are the most common cause for paroxysmal kinesigenic dyskinesia.However,patients with negative PRRT2 mutations are not rare.The aim of this study is to determine whether copy number variant of PRRT2 gene is another potential pathogenic mechanism in the patients with paroxysmal kinesigenic dyskinesia with negative PRRT2 point and frameshift mutations.Methods:We screened PRRT2 copy number

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