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A novel mutation of LIM2 causes autosomal dominant membranous cataract in a Chinese family

International Journal of Ophthalmology(English edition) 2020年第10期13卷 1512-1520页

作者： Rui Pei Peng-Fei Liang Wei Ye ji Li ji-yuan ma jian Zhou Department of Ophthalmology Xijing HospitalFourth Military Medical UniversityXi’an 710032Shaanxi ProvinceChina Department of Otolaryngology Xijing HospitalFourth Military Medical UniversityXi'an 710032Shaanxi ProvinceChina

AIM:To identify mutations in the genes of a fourgeneration Chinese family with congenital membranous cataracts and investigate the morphologic changes and possible functional damage underlying the role of the mutant ***:Whole exome analysis of thirteen members of a four-generation pedigree affected with congenital membranous cataracts was performed;co-segregation analysis of identified variants was validated by Sanger *** members underwent detailed physical and complete eye *** physical changes caused by the mutation were analyzed in silico through homology *** lens fiber block from a patient was observed under a scanning electron microscope(SEM).Cell membrane proteins and cytoplasmic proteins from the human lenses donated by one patient with cataract in this family and from the dislocated lens resulted from the penetrating ocular trauma of a patient unrelated with this family were extracted,and the expression and localization of MP20 and Cx46 were detected by Western blot(WB)assay in these ***:A novel LIM2 heterozygous mutation(c.388 C>T,p.R130 C)was identified with congenital membranous cataracts inherited by an autosomal dominant(AD)*** and amblyopia were observed in all patients of this family,and exotropia and long axial length were observed in most patients.A/B ultrasound scan and ultrasound biomicroscopy revealed obvious thin crystalline lenses from 1.7 to 2.7 mm in central thickness in all cataract *** bioinformatic analysis showed that the mutation was deleterious to the physiological function of LIM2-encoded ***,by SEM,ultrastructure of the cataract nucleus showed that lens fiber cells(LFCs)remained morphologic characteristics of immature fiber cells,including flap cell surface with straight edges and lacking normal ball-and-socket joint boundaries,which implied that the differentiation of LFCs might be *** of MP20 and Cx46 in the cytoplasm was observe

关键词： LIM2 MP20 congenital cataract missense mutation differentiation

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Long-term follow-up of HCV patients with sustained virological response after treatment with pegylated interferon plus ribavirin

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Hepatobiliary & Pancreatic Diseases International 2021年第2期20卷 137-141页

作者： yuan-ji ma Ling-Yao Du Li-Bo Yan Juan Liao Xing Cheng Wu-Wei Xie Hong Tang Center of Infectious Diseases West China Hospital of Sichuan UniversityChengdu 610041China

Background:The progress of liver diseases may not stop after viral *** study aimed to provide data on long-term prognosis of patients with hepatitis C virus(HCV)infection who underwent pegylated interferon plus ribavirin(PR)regimen and achieved a sustained virological response 24 weeks post-treatment(SVR24).Methods:Responders to the PR regimen in our hospital from January 2011 to June 2014 were enrolled and prospectively followed *** characteristics were *** incidence of hepatocellular carcinoma(HCC),progression of liver disease(increase in liver stiffness or occurrence of decompensated complication),and HCV recurrence was all *** accumulative and annualized incidence rates(AIRs)of these adverse events were analyzed,and the risk factors were also ***:In total,151 patients reached a median follow-up time of 103 *** them,two had an incidence of HCC during the surveillance with AIR of 0.68%(95%CI:0.00-1.63%).Six patients showed progression of liver disease with AIR of 2.05%(95%CI:0.42%-3.68%).Three patients who had risky behaviors encountered HCV *** cirrhotic patients faced higher risk of poor prognosis than non-cirrhotic patients,including HCC and progression of liver disease(AIR:6.17%vs.1.42%,P=0.039).Conclusions:The incidence of HCC and progression of liver disease was evident in PR responders during the long-term follow-up period,but the risk level was *** responders were more vulnerable to develop HCC post SVR24 compared with non-cirrhotic *** recurrence was rare in responders with SVR24 who had corrected their risky behaviors.

关键词： Hepatitis C virus Sustained virological response Hepatocellular carcinoma

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Hemoperitoneum in cirrhotic patients without abdominal trauma or tumor

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Hepatobiliary & Pancreatic Diseases International 2011年第6期10卷 644-648页

作者： yuan-ji ma,En-Qiang Chen,jia-jie Lu,Ming-Zhen Tan and Hong Tang Center of Infectious Diseases,West China Hospital,Sichuan University,Chengdu 610041,China Center of Infectious Diseases West China Hospital Sichuan University Chengdu 610041 China

BACKGROUND:Hemoperitoneum is associated with several emergency conditions and is especially evident when it occurs in patients with liver *** study aimed to assess the clinical characteristics of cirrhotic patients who did not have abdominal trauma or tumor but who developed ***:We reviewed the clinical records of 1276 consecutive cirrhotic patients with hemoperitoneum at our center between January 2007 and December *** was confirmed by abdominal ***:Of the 1276 cirrhotic patients,19 were found to have hemoperitoneum,but only 6 did not have abdominal trauma or *** occurrence of spontaneous hemoperitoneum in the cirrhotic patients was therefore 0.5%.Hemoperitoneum can occur spontaneously in severely decompensated cirrhotic patients with intra-abdominal collateral vessels and high scores on the model for end-stage liver disease and Child-Pugh-Turcotte *** patients presented with abdominal distension,abdominal pain,increased abdominal girth and hemodynamic instability with a significant drop in the hemoglobin *** patients died of hemorrhagic shock within 24 hours,and the other 3 died of hepatic encephalopathy or spontaneous bacterial peritonitis after 5 to 10 days because of further decompensation of the ***:Hemoperitoneum can occur in cirrhotic patients who do not have abdominal trauma or *** mainly occurs in severely decompensated end-stage cirrhotic *** patients with hemoperitoneum have a poor prognosis.

关键词： liver cirrhosis portal pressure hemoperitoneum abdominal paracentesis

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FK506 contributes to peripheral nerve regeneration by inhibiting neuroinflammatory responses and promoting neuron survival

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Neural Regeneration Research 2025年第7期20卷 2108-2115页

作者： Yuhui Kou Zongxue jin Yusong yuan Bo ma Wenyong Xie Na Han Department of Trauma and Orthopedics Peking University People’s HospitalBeijingChina National Center for Trauma Medicine Peking University People’s HospitalBeijingChina Key Laboratory of Trauma and Neural Regeneration(Peking University) Ministry of EducationBeijingChina Trauma Medicine Center Peking University People’s HospitalBeijingChina Department of Orthopedics China-Japan Friendship HospitalBeijingChina

FK506(Tacrolimus)is a systemic immunosuppressant approved by the *** and Drug ***506 has been shown to promote peripheral nerve regeneration,however,its precise mechanism of action and its pathways remain *** this study,we established a rat model of sciatic nerve injury and found that FK506 improved the morphology of the injured sciatic nerve,increased the numbers of motor and sensory neurons,reduced inflammatory responses,markedly improved the conduction function of the injured nerve,and promoted motor function *** findings suggest that FK506 promotes peripheral nerve structure recovery and functional regeneration by reducing the intensity of inflammation after neuronal injury and increasing the number of surviving neurons.

关键词： FK506 inflammation motor neurons nerve regeneration neuron peripheral nerve injury sensory neurons

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局部枸橼酸抗凝在血浆吸附联合血浆置换治疗高危出血倾向肝衰竭患者中的应用

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世界华人消化杂志 2018年第3期26卷 165-173页

作者：马元吉陈芳许艳白浪唐红四川大学华西医院感染性疾病中心四川省成都市610041

目的分析局部枸橼酸抗凝(regional citrate anticoagulation,RCA)在血浆吸附联合血浆置换(plasma adsorption plus plasma exchange,PA+PE)治疗高危出血倾向肝衰竭中的可行性与安全性.方法收集2016-10/2017-07四川大学华西医院感染性疾病中心收治的采用RCA行PA+PE治疗的高危出血倾向肝衰竭患者的临床资料,回顾性分析治疗效果与枸橼酸蓄积、电解质及酸碱变化.当血总钙(Ca tot)与离子钙(Ca ion)的比值(Ca tot/Ca ion)≥2.5时,判定发生了枸橼酸蓄积.结果 7例患者存活2例,死亡5例.共完成24例次R C A的PA+PE治疗.PA治疗期间体外Ca ion浓度为0.28 mmol/L±0.09 mmol/L.45.8%(11/24)例次治疗在PA治疗期间发生了枸橼酸蓄积;至PE治疗末,全部例次治疗均出现了枸橼酸蓄积.治疗后2 h,仍有41.7%(10/24)例次治疗存在枸橼酸蓄积,但已较PE治疗末明显减少(P<0.01);至第2天晨,全部例次治疗均不再存在枸橼酸蓄积.治疗期间及治疗后无新发出血,原有出血未恶化;主要不良反应为碱中毒和一过性低Ca ion血症及高Ca tot血症.结论肝衰竭患者仍具有一定程度的代谢枸橼酸和耐受枸橼酸蓄积的能力.RCA在PA联合PE治疗肝衰竭患者中可能具有一定的可行性及安全性.

关键词：肝衰竭血浆吸附血浆置换枸橼酸抗凝

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虚拟作战战场环境的研究与实现

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系统仿真学报 2003年第7期15卷 969-972,1053页

作者：李宁彭晓源马继峰冯勤北京航空航天大学先进仿真技术航空科技重点实验室北京100083

介绍了构建以解放战争某著名战役为背景的虚拟战场环境的关键技术,包括虚拟战场环境的结构组成、仿真模型、仿真管理和想定生成、场景管理、虚拟士兵的行为表示、实时战场环境声音仿真、人机交互方式等。在此基础上,介绍了已研制完成的... 详细信息

介绍了构建以解放战争某著名战役为背景的虚拟战场环境的关键技术,包括虚拟战场环境的结构组成、仿真模型、仿真管理和想定生成、场景管理、虚拟士兵的行为表示、实时战场环境声音仿真、人机交互方式等。在此基础上,介绍了已研制完成的基于多PC机联网具有沉浸与交互特征的多用户同时参与的含虚拟士兵的虚拟作战演示与观众参与系统。

关键词：虚拟现实虚拟战场环境虚拟士兵人机交互

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Locally Enhanced Flow and Electric Fields Through a Tip Effect for Efficient Flow‑Electrode Capacitive Deionization

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Nano-Micro Letters 2025年第2期17卷 1-17页

作者： Ziquan Wang Xiangfeng Chen yuan Zhang jie ma Zhiqun Lin Amor Abdelkader maria‑magdalena Titirici Libo Deng College of Chemistry and Environmental Engineering Shenzhen UniversityShenzhen 518060People’s Republic of China Research Center for Environmental Functional materials College of Environmental Science and EngineeringTongji University1239 Siping RoadShanghai 200092People’s Republic of China Department of Chemical and Biomolecular Engineering National University of SingaporeSingapore 117585Singapore Department of Engineering Faculty of Science and TechnologyBournemouth UniversityTalbot CampusFern BarrowPooleEngland BH125BBUK Institut de Chimie de Nice UniversitéCôte d’AzurUMR CNRS 727228 Av.Valrose06108 NiceFrance Department of Chemical Engineering Imperial College LondonSouth Kensington CampusExhibition RdLondon SW72AZUK

Low-electrode capacitive deionization(FCDI)is an emerging desalination technology with great potential for removal and/or recycling ions from a range of ***,it still suffers from inefficient charge transfer and ion transport kinetics due to weak turbulence and low electric intensity in flow electrodes,both restricted by the current ***,a new tip-array current collector(designated as T-CC)was developed to replace the conventional planar current collectors,which intensifies both the charge transfer and ion transport *** effects of tip arrays on flow and electric fields were studied by both computational simulations and electrochemical impedance spectroscopy,which revealed the reduction of ion transport barrier,charge transport barrier and internal *** the voltage increased from 1.0 to 1.5 and 2.0 V,the T-CC-based FCDI system(T-FCDI)exhibited average salt removal rates(ASRR)of 0.18,0.50,and 0.89μmol cm^(-2) min^(-1),respectively,which are 1.82,2.65,and 2.48 folds higher than that of the conventional serpentine current collectors,and 1.48,1.67,and 1.49 folds higher than that of the planar current ***,with the solid content in flow electrodes increased from 1 to 5 wt%,the ASRR for T-FCDI increased from 0.29 to 0.50μmol cm^(-2) min^(-1),which are 1.70 and 1.67 folds higher than that of the planar current ***,a salt removal efficiency of 99.89%was achieved with T-FCDI and the charge efficiency remained above 95%after 24 h of operation,thus showing its superior long-term stability.

关键词： Flow-electrode Capacitive deionization Current collector Tip effect Desalination

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mainchain conformation ordering self-assembly tailored room temperature phosphorescence emission

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Science China Chemistry 2025年第01期 329-336页

作者： Yuzhu Li Yumeng Gao Feng Tian Yu Wang yuan Yao Shaoliang Lin Xiang ma Binbin Xu Shanghai Key Laboratory of Advanced Polymeric materials Key Laboratory for Ultrafine Materials of Ministry of Education School of Materials Science and Engineering East China University of Science and Technology Shanghai Synchrotron Radiation Facility Shanghai Advanced Research Institute Chinese Academy of Sciences Key Laboratory for Advance materials and Feringa Noble Prize Scientist Joint Research Centre Frontiers Science for Materiobiology and Dynamic Chemistry School of Chemistry and Molecular Engineering East China University of Science & Technology

Developing organic room-temperature phosphorescence （RTP） materials with a wide range of tunable-emission is significant in expanding the applications of RTP ***,we rationally designed amphiphilic alternating copolymers poly(1,2-bis（4-alkoxyphenyl）ethane-1,2-dione-alt-（ethylene glycol）x)(P（Bz-alt-EGx）) that combined the advantage of alternating benzil and EGxskeleton,and tunable mainchain *** a mainchain conformation ordering assembly （COA） process,P（Bz-alt-EGx） self-assembled into well-defined platelets,and such assemblies emitted blue RTP at ca.425 nm due to the ordered folded-chain conformation and confined *** contrast,traditional self-assembly （TA） of P（Bz-alt-EGx） obtained less ordered aggregates,such as asymmetric worms,vesicles,and rod-like *** resultant less ordered aggregates exhibited yellow-green RTP at ca.550 *** wide-tuning RTP emission in solution （between blue and yellow-green,over125 nm） is realized using the P（Bz-alt-EGx） alternating copolymers under the treatment of different self-assembly *** study uncovers a new strategy to tailor the RTP emission through different self-assembly pathways and holds great promise for the fabrication of advanced optical materials.

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Decreased levels of phosphorylated synuclein in plasma are correlated with poststroke cognitive impairment

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Neural Regeneration Research 2025年第9期20卷 2598-2610页

作者： Yi Wang Yuning Li Yakun Gu Wei ma Yuying Guan Mengyuan Guo Qianqian Shao Xunming ji jia Liu Department of Clinical Laboratory Beijing Bo’ai HospitalChina Rehabilitation Research CenterCapital Medical UniversityBeijingChina Beijing Institute of Brain Disorders Laboratory of Brain DisordersMinistry of Science and TechnologyCollaborative Innovation Center for Brain DisordersBeijing Advanced Innovation Center for Big Data-based Precision MedicineCapital Medical UniversityBeijingChina Department of Neurosurgery Xuanwu HospitalCapital Medical UniversityBeijingChina

Poststro ke cognitive impairment is a major secondary effect of ischemic stroke in many patients;however,few options are available for the early diagnosis and treatment of this *** aims of this study were to(1)determine the specific relationship between hypoxic andα-synuclein during the occur of poststroke cognitive impairment and(2)assess whether the serum phosphorylatedα-synuclein level can be used as a biomarker for poststro ke cognitive *** found that the phosphorylatedα-synuclein level was significantly increased and showed pathological aggregation around the cerebral infa rct area in a mouse model of ischemic *** addition,neuronalα-synuclein phosphorylation and aggregation were observed in the brain tissue of mice subjected to chronic hypoxia,suggesting that hypoxia is the underlying cause ofα-synuclein-mediated pathology in the brains of mice with ischemic *** phosphorylatedα-synuclein levels in patients with ischemic stroke were significantly lower than those in healt hy subjects,and were positively correlated with cognition levels in patients with ischemic ***,a decrease in serum high-density lipoprotein levels in stroke patie nts was significantly correlated with a decrease in phosphorylatedα-synuclein *** ischemic stroke mice did not show significant cognitive impairment or disrupted lipid metabolism 14 days after injury,some of them exhibited decreased cognitive function and reduced phosphorylatedα-synuclein *** together,our results suggest that serum phosphorylatedα-synuclein is a potential biomarker for poststroke cognitive impairment.

关键词： biomarker high-density lipoprotein ischemic stroke phosphorylatedα-synuclein poststroke cognitive impairment

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Regulation and function of endoplasmic reticulum autophagy in neurodegenerative diseases

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Neural Regeneration Research 2025年第1期20卷 6-20页

作者： Xiu-Yun Zhao De-En Xu Ming-Lei Wu ji-Chuan Liu Zi-Ling Shi Quan-Hong ma Department of Neurology and Clinical Research Center of Neurological Disease the Second Affiliated Hospital of Soochow UniversitySuzhouJiangsu ProvinceChina Institute of Neuroscience&jiangsu Key Laboratory of Translational Research and Therapy for Neuro-Psycho-Diseases Soochow UniversitySuzhouJiangsu ProvinceChina Department of Neurology Jiangnan University Medical CenterWuxiJiangsu ProvinceChina

The endoplasmic reticulum,a key cellular organelle,regulates a wide variety of cellular *** reticulum autophagy,one of the quality control systems of the endoplasmic reticulum,plays a pivotal role in maintaining endoplasmic reticulum homeostasis by controlling endoplasmic reticulum turnover,remodeling,and *** this review,we briefly describe the endoplasmic reticulum quality control system,and subsequently focus on the role of endoplasmic reticulum autophagy,emphasizing the spatial and temporal mechanisms underlying the regulation of endoplasmic reticulum autophagy according to cellular *** also summarize the evidence relating to how defective or abnormal endoplasmic reticulum autophagy contributes to the pathogenesis of neurodegenerative *** summary,this review highlights the mechanisms associated with the regulation of endoplasmic reticulum autophagy and how they influence the pathophysiology of degenerative nerve *** review would help researchers to understand the roles and regulatory mechanisms of endoplasmic reticulum-phagy in neurodegenerative disorders.

关键词： autophagy endoplasmic reticulum endoplasmic reticulum autophagy endoplasmic reticulum quality control system endoplasmic reticulum receptors endoplasmic reticulum-associated degradation neurodegeneration neurodegenerative disease selective autophagy unfolded protein response

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