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Drosophila models used to simulate human ATP1A1 gene mutations that cause Charcot-Marie-Tooth type 2 disease and refractory seizures

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Neural Regeneration Research 2025年第1期20卷 265-276页

作者： Yao Yuan Lingqi Yu Xudong Zhuang Dongjing Wen Jin He jingmei hong Jiayu Xie Shengan Ling Xiaoyue Du Wenfeng Chen Xinrui Wang Institute of Life Sciences College of Biological Science and EngineeringFuzhou UniversityFuzhouFujian ProvinceChina NHC Key Laboratory of Technical Evaluation of Fertility Regulation for Non-human Primate(Fujian Maternity and Child Health Hospital) FuzhouFujian ProvinceChina Medical Research Center Fujian Maternity and Child Health HospitalCollege of Clinical Medicine for Obstetrics&Gynecology and PediatricsFujian Medical UniversityFuzhouFujian ProvinceChina Department of Neurology and Institute of Neurology of The First Affiliated Hospital Institute of Neuroscienceand Fujian Key Laboratory of Molecular NeurologyFujian Medical UniversityFuzhouFujian ProvinceChina

Certain amino acids changes in the human Na^(+)/K^(+)-ATPase pump,ATPase Na^(+)/K^(+)transporting subunit alpha 1(ATP1A1),cause Charcot-Marie-Tooth disease type 2(CMT2)disease and refractory *** develop in vivo models to study the role of Na^(+)/K^(+)-ATPase in these diseases,we modified the Drosophila gene homolog,Atpα,to mimic the human ATP1A1 gene mutations that cause *** located within the helical linker region of human ATP1A1(I592T,A597T,P600T,and D601F)were simultaneously introduced into endogenous Drosophila Atpαby CRISPR/Cas9-mediated genome editing,generating the Atpα^(TTTF)*** addition,the same strategy was used to generate the corresponding single point mutations in flies(Atpα^(I571T),Atpα^(A576T),Atpα^(P579T),and Atpα^(D580F)).Moreover,a deletion mutation(Atpα^(mut))that causes premature termination of translation was generated as a positive *** these alleles,we found two that could be maintained as homozygotes(Atpα^(I571T)and Atpα^(P579T)).Three alleles(Atpα^(A576T),Atpα^(P579)and Atpα^(D580F))can form heterozygotes with the Atpαmut *** found that the Atpαallele carrying these CMT2-associated mutations showed differential phenotypes in *** heterozygous for Atpα^(TTTF)mutations have motor performance defects,a reduced lifespan,seizures,and an abnormal neuronal *** Drosophila models will provide a new platform for studying the function and regulation of the sodium-potassium pump.

关键词： ATP1A1 Atpα bang-sensitive paralysis Charcot-Marie-Tooth disease type 2 CRISPR/Cas9 homology-directed repair Na^(+)/K^(+)-ATPase point mutation seizures sodium pump

17p11.2拷贝数变异周围神经病的基因、临床及电生理分析

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福建医科大学学报 2022年第6期56卷 535-539页

作者：洪净梅张小蓉高缘张雨朵何瑾福建医科大学附属第一医院神经内科福州350005

目的分析17p11.2拷贝数变异相关遗传性周围神经病的基因、临床、电生理特点和表型-基因型特点。方法收集2001年10月—2021年11月115例就诊福建医科大学附属第一医院神经内科门诊患者,通过多重连接依赖的探针扩增(MLPA)确定为17p11.2拷... 详细信息

目的分析17p11.2拷贝数变异相关遗传性周围神经病的基因、临床、电生理特点和表型-基因型特点。方法收集2001年10月—2021年11月115例就诊福建医科大学附属第一医院神经内科门诊患者,通过多重连接依赖的探针扩增(MLPA)确定为17p11.2拷贝数变异相关遗传性周围神经病,分析其基因、临床和电生理特点。结果115例17p11.2拷贝数变异相关遗传性周围神经病中,90例为17p11.2拷贝数重复变异,诊断为腓骨肌萎缩症1A型(CMT1A);25例为17p11.2拷贝数缺失变异,诊断为遗传性压力易感性周围神经病(HNPP)。2组患者的发病年龄比较,差别无统计学意义(P=0.318);2组患者在起病形式上,均以肌无力或同时出现肌无力及感觉异常或单纯性感觉异常起病,均以单纯性肌无力起病占多数;CMT1A患者肌萎缩、步态异常及畸形的发生率较HNPP患者高(P<0.001)。58例CMT1A患者及18例HNPP患者进行电生理检测,结果显示HNPP患者的神经传导检出率明显高于CMT1A患者(P<0.001),且波幅均较CMT1A患者高(P<0.0001)。结论17p11.2拷贝数变异相关遗传性周围神经病临床表现不一致,可分为CMT1A、HNPP,临床上经常出现误诊、漏诊。对于临床特征不典型的、神经电生理检查呈脱髓鞘表现的患者,排除获得性病因后,应检测17p11.2拷贝数变异甚至全外显子测序以明确诊断,为疾病诊断及遗传咨询提供依据。

关键词： 17p11.2腓骨肌萎缩症1A型遗传性压力易感性周围神经病 PMP22基因

Drip-drop hovering control for mixed propulsion system

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Drip-drop hovering control for mixed propulsion system

第36届中国控制会议

作者： Jiadong Ren jingmei Huang Qingshuang Zeng hong Zhu School of Astronautics Harbin Institute of Technology Shanghai Institute of Spaceflight Control Technology Shanghai Key Laboratory of Space Intelligent Control Technology

ISBN: (纸本)9781538629185

In order to satisfy the hovering technology demand for spacecraft on-orbit service task etc.,based on the description method of relative orbit parameters,two parameters of semi-major axis error and eccentricity error are used for deep study of the forming of drip-drop hovering configuration in plane,and the forming condition and configuration design method are also ***’s more,considering the evolution property under the electric-chemical integration propulsion system,the design method for the two parameters of in-plane *** results show that,the guidance scheme is correct and efficacious,the total burn-up for hovering approximates open-loop optimal performance,and the demand for engine is low,therefore it is a feasible engineering technology approach.

关键词： relative kinematics relative orbit parameter error drip-drop hovering electric propulsion

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高碱煤热烟气强化水洗脱碱机制及动力学分析

工程热物理学报 2024年第10期45卷 3209-3216页

作者：王祥曦程小爱李显郭丽魏博李建钟梅刘景梅马凤云罗光前姚洪新疆大学化工学院煤炭清洁转化与化工过程自治区重点实验室乌鲁木齐830000 华中科技大学能源与动力工程学院煤燃烧国家重点实验室武汉430074

基于低p H浸洗促进高碱煤中碱金属和碱土金属(AAEM)的增溶原理,将电厂烟气(CO_(2))作为酸源并将余热资源化利用,团队前期开发出一种针对高碱煤的热烟气强化水洗脱碱技术(FG-WL)。本文对不同形态AAEM的脱除及关键参数的影响规律和机制进... 详细信息

基于低p H浸洗促进高碱煤中碱金属和碱土金属(AAEM)的增溶原理,将电厂烟气(CO_(2))作为酸源并将余热资源化利用,团队前期开发出一种针对高碱煤的热烟气强化水洗脱碱技术(FG-WL)。本文对不同形态AAEM的脱除及关键参数的影响规律和机制进行了较详细讨论,通过动力学计算确定AAEM不同时间段的控制步骤和参数。结果表明,FG-WL的温度从25℃升至60℃,高碱煤120 min的脱钠率从80.7%升至94.7%,40 min的脱钙率从32.5%提升至38.7%;处理0~40 min的碱金属(Na、K)脱除主体为水溶态,且为固膜扩散控制,40 min后以醋酸铵溶态为主,且为化学反应控制;碱土金属(Ca、Mg)的全程以醋酸铵溶态脱除为主并伴随部分盐酸溶态,且为固体膜控制。本研究可为此方法的工艺优化和参数调节提供一定指导。

关键词：高碱煤热烟气洗煤碱金属和碱土金属浸出动力学

高碱煤热烟气强化水洗脱碱及其燃烧结渣沾污研究

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工程热物理学报 2024年第8期45卷 2509-2516页

作者：程小爱王祥曦马金荣程延峰胡振中马剑飞魏博李建钟梅刘景梅马凤云罗光前姚洪新疆大学化工学院煤炭清洁转化与化工过程自治区重点实验室乌鲁木齐830000 华中科技大学能源与动力工程学院煤燃烧国家重点实验室武汉430074 新疆新业能源化工有限责任公司五家渠831300

新疆高碱煤强结渣沾污特性是影响其大规模燃烧发电的瓶颈问题。本研究基于CO_(2)溶于水形成的碳酸可有效脱除煤中水不溶碱金属碱土金属的原理,提出以热烟气为CO_(2)来源的高碱煤热烟气强化水洗脱碱方法。通过对多种典型新疆高碱煤的实... 详细信息

新疆高碱煤强结渣沾污特性是影响其大规模燃烧发电的瓶颈问题。本研究基于CO_(2)溶于水形成的碳酸可有效脱除煤中水不溶碱金属碱土金属的原理,提出以热烟气为CO_(2)来源的高碱煤热烟气强化水洗脱碱方法。通过对多种典型新疆高碱煤的实验研究发现,Na的脱除率达到90%以上,Ca的脱除率达到45%,显著优于普通水洗对Na和Ca的脱除效果。洗后煤灰的碱酸比显著降低,灰熔点提高了150℃左右,大部分煤的结渣沾污倾向从严重结渣变为了中等结渣,或从中等结渣变为了不结渣。所以,热烟气强化水洗对高碱煤脱碱及抑制其燃烧过程的结渣沾污具有明显效果。另外,本方法简单可行,而且为电厂烟气(CO_(2))提供了一条新的资源化利用途径,具有明显的经济价值和社会意义。

关键词：高碱煤热烟气洗煤结渣沾污

Network prediction of surgical complication clusters:a prospective multicenter cohort study

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Science China(Life Sciences) 2023年第7期66卷 1636-1646页

作者： Xiaochu Yu Peng Wu Zixing Wang Wei Han Yuguang Huang Shijie Xin Qiang Zhang Shengxiu Zhao hong Sun Guanghua Lei Taiping Zhang Luwen Zhang Yubing Shen Wentao Gu hongwei Li jingmei Jiang Peking Union Medical College Hospital Chinese Academy of Medical SciencesBeijing100730China Institute of Basic Medical Sciences Chinese Academy of Medical Sciences/School of Basic MedicinePeking Union Medical CollegeBeijing100005China The First Hospital of China Medical University Shenyang110001China Qinghai Provincial People’s Hospital Xining810007China Xiangya Hospital of Central South University Changsha410008China Research Department PaodingAIBeijing100083China

Complicated relationships exist in both occurrence and progression of surgical complications,which are difficult to account for using a separate quantitative method such as prediction or *** of 51,030 surgical inpatients were collected from four academic/teaching hospitals in a prospective cohort study in *** relationship between preoperative factors,22 common complications,and death was *** input from 54 senior clinicians and following a Bayesian network approach,a complication grading,cluster-visualization,and prediction(GCP)system was designed to model pathways between grades of complication and preoperative risk factor *** the GCP system,there were 11 nodes representing six grades of complication and five preoperative risk factor clusters,and 32 arcs representing a direct *** critical targets were pinpointed on the *** status was a fundamental cause widely associated(7/32 arcs)with other risk factor clusters and *** Society of Anesthesiologists(ASA)score≥3 was directly dependent on all other risk factor clusters and influenced all severe *** III complications(mainly pneumonia)were directly dependent on4/5 risk factor clusters and affected all other grades of *** of grade,complication occurrence was more likely to increase the risk of other grades of complication than risk factor clusters.

关键词： surgical complication network system complication grading complication prediction complication management

A Spatio-temporal Based Parallel Insertion Algorithm for Solving Dial-A-Ride Problem 26th

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A Spatio-temporal Based Parallel Insertion Algorithm for Sol...

第26届中国控制与决策会议

作者： jingmei Guo Chao Liu Institute of System Engineering School of Information Science and EngineeringNortheastern University Institute of Systems Science Northeastern University

ISBN: (纸本)9781479937097

A static dial-a-ride problem with time windows is investigated, without wait while carrying passengers and maximum ride time constraints as the service quality. A parallel insertion heuristic based on the spatial and temporal aspects is presented. The proposed algorithm firstly sorts the requests based on the earliest pickup time and decentralization index alternately and initializes the routes with seed requests. Then a insertion-rejected-reinsertion operation is performed, in which the spatial aspect as the granular is also considered. Simulation results indicate that the proposed algorithm reduces costs effectively, compared with the algorithms only considering the temporal aspect and no seed requests. Also, sensitivity analysis indicates that the required vehicle number is insensitive to the granular extent and the initial vehicle number.

关键词： Dial-A-Ride Problem Spatio-Temporal Parallel Insertion Algorithm Rejected-Reinsertion

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Image J软件在象牙鉴定中的应用

东北林业大学学报 2010年第6期38卷 129-130,132页

作者：胡红金煜周晶梅东北林业大学哈尔滨150040

引入Image J软件应用于猛犸象牙、现生象牙外缘施氏角的度量,发现100°与115°是识别猛犸象牙和现生象牙的分界点,同前人研究结果相符。该软件在象牙的物种鉴定中有很强的应用前景,不仅能降低人为主观因素给检验结果造成的影响,又可减... 详细信息

引入Image J软件应用于猛犸象牙、现生象牙外缘施氏角的度量,发现100°与115°是识别猛犸象牙和现生象牙的分界点,同前人研究结果相符。该软件在象牙的物种鉴定中有很强的应用前景,不仅能降低人为主观因素给检验结果造成的影响,又可减少全手工操作所需的时间,提高工作效率。

关键词：象牙物种鉴定施氏角 Image J软件

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反应温度对脱沥青油临氢热转化反应的影响

石油学报（石油加工） 2016年第5期32卷 1020-1029页

作者：洪琨马凤云刘景梅钟梅新疆大学化学化工学院煤炭洁净转化与化工过程自治区重点实验室新疆乌鲁木齐830046

以塔河常压渣油(THAR)为原料,正己烷为溶剂分离获得C6-沥青质(As)和脱沥青油(DAO),考察了反应温度对DAO临氢热转化性能及其产物中次生沥青质(As*)结构的影响。结果表明,与THAR相比,DAO热转化活性较高,残炭值较低,且热转化反应产物中轻... 详细信息

以塔河常压渣油(THAR)为原料,正己烷为溶剂分离获得C6-沥青质(As)和脱沥青油(DAO),考察了反应温度对DAO临氢热转化性能及其产物中次生沥青质(As*)结构的影响。结果表明,与THAR相比,DAO热转化活性较高,残炭值较低,且热转化反应产物中轻质油收率在410℃之前迅速增加,随后趋于平缓,而焦炭收率的增幅显著变大。与As相比,As*中芳环γ位(Hγ)和芳环β位氢(Hβ)含量较少,芳环α位氢(Hα)含量、芳环氢(HA)含量和芳香度(fA)较高。随反应温度提高,As*中Hγ含量和Hβ含量逐渐减小,Hα含量、HA含量和fA逐渐增加;同时,As*结构中芳香片层间距(dm)呈减小趋势,而芳香薄片直径(La)、芳香片层堆砌高度(Lc)及堆砌层数(Mc)均逐渐增大。As*中与芳环γ、β位相连的烷基侧链发生断裂脱落,促使芳核在横向和纵向上均发生缩聚,致使As*中迫位缩合结构、Hα含量和HA含量增加,dm减小,L_a、L_c及Mc变大。

关键词：脱沥青油临氢热转化次生沥青质分子结构