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Molecular medicine of fragile X syndrome: based on known molecular mechanisms

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World Journal of Pediatrics 2016年第1期12卷 19-27页

作者： shi-yu luo Ling-Qian Wu Ran-Hui Duan State Key Laboratory of Medical Genetics&School of Life Sciences Central South UniversityChangshaChina

Background:Extensive research on fragile X mental retardation gene knockout mice and mutant Drosophila models has largely expanded our knowledge on mechanism-based treatment of fragile X syndrome(FXS).In light of these findings,several clinical trials are now underway for therapeutic translation to *** sources:Electronic literature searches were conducted using the PubMed database and *** search terms included"fragile X syndrome","FXS and medication","FXS and therapeutics"and"FXS and treatment".Based on the publications identified in this search,we reviewed the neuroanatomical abnormalities in FXS patients and the potential pathogenic mechanisms to monitor the progress of FXS research,from basic studies to clinical ***:The pathological mechanisms of FXS were categorized on the basis of neuroanatomy,synaptic structure,synaptic transmission and fragile X mental retardation protein(FMRP)loss of *** neuroanatomical abnormalities in FXS were described to motivate extensive research into the region-specific pathologies in the brain responsible for FXS behavioural ***-directed molecular medicines were classified according to their target pathological mechanisms,and the most recent progress in clinical trials was ***:Current mechanism-based studies and clinical trials have greatly contributed to the development of FXS pharmacological *** examining the extent to which these treatments provided a rescue effect or FMRP compensation for the developmental impairments in FXS patients may help to improve the effi cacy of treatments.

关键词： clinical trial fragile X syndrome mechanism-based treatment

Influence of platform position on stray grain nucleation in Ni-based single-crystal dummy blade clusters

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China Foundry 2021年第5期18卷 442-449页

作者： Zhen-yu Yang Chen-guang Liu Song-song Hu Su-jie Zheng yu-shi luo Shen-long Dai Science and Technology on Advanced High Temperature Structural Materials Laboratory Beijing Institute of Aeronautical MaterialsBeijing 100095China School of Materials Science and Engineering Xiangtan UniversityXiangtan 411105HunanChina

Stray grains are the most severe of the solidification defects that occur in the industrial single-crystal blade preparation *** this study,a single-crystal dummy blade cluster with different crystal orientations controlled by the seeding method was prepared,and the influence of the position of the circular platform(relative to the sample and furnace body)on stray grain nucleation was *** show that the microstructure of the circular platforms could be divided into the center,expansion,and stray grain *** inside of the circular platform facing the center of the cluster is more prone to stray grain formation than the outside of the circular platform facing the furnace *** an increase in the distance between the circular platform and the bottom of the dummy blade cluster,the stray grain region expands,whereas the expansion region *** stray grain is slightly aggravated with increase of the ***,the mechanism underlying the influence of platform position on the formation of stray grains in single-crystal dummy blade clusters is discussed based on the temperature evolution during directional solidification.

关键词： Ni-based single-crystal superalloy directional solidification stray grain nucleation dummy blade cluster

Creep property and microstructural evolution of DD11 alloy under high temperature and low stress

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Rare Metals 2024年第2期43卷 810-816页

作者： Yan-Fei Liu Jiang-Bo Sha yun-Song Zhao Chen-Guang Liu yuan-yuan Guo Jian Zhang yu-shi luo School of Materials Science and Engineering Beihang UniversityBeijing100191China Science and Technology on Advanced High Temperature Structural Materials Laboratory Beijing Institute of Aeronautical MaterialsBeijing100095China

In this paper,the interrupted and ruptured creep tests were carried out in a novel second generation single crystal superalloy named DD11 at 1100℃/130 *** alloy exhibited typical creep curve including primary,steady,and tertiary three creep *** microstructural evolution at different stages of the creep were analyzed by scanning electron microscopy(SEM)and transmission electron microscopy(TEM).The results show that theγ′phases transform into rafted structure at the early stage of the steady creep and keep stable during the steady creep *** the creep goes on,the rafted structure further coarsens and the topological inversion *** addition,at the primary creep,the dislocations mainly move in theγmatrix and pile up in theγ/γ′interface since the matrix channels widen *** formation of the regular interfacial dislocation networks occurs at the early stage of the steady *** the low stress,the dominated deformation mechanism during steady creep stage is the climbing of the〈010〉type edge ***,the effect of the deformation mechanism on creep property was discussed in detail.

关键词： DD11 alloy Creep property Interrupted creep test Rafted structure Deformation mechanism

Tilting homeostatic and dyshomeostatic microglial balance in health and disease:transforming growth factor-beta1 as a critical protagonist

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Neural Regeneration Research 2025年第10期20卷 2895-2897页

作者： Nicolas Hugues yu luo Department of Molecular and Cellular Biosciences University of CincinnatiCincinnatiOHUSA Neuroscience Graduate Program University of CincinnatiCincinnatiOHUSA Division of Immunobiology Cincinnati Children’s Hospital Medical CenterUniversity of Cincinnati College of MedicineCincinnatiOHUSA

Adult microglia,by continuously sensing changes in their environment and communicating with nearly all brain cell types,are considered to be the immune sentinels of the *** the healthy central nervous system(CNS),microglia display a unique molecular homeostatic signature(i.e.,Tmem119,P2ry12,Sall1,Siglech,Gpr34,and Hexb)(Figure 1A).

关键词： Figure signature critical

A study of the ameliorating effects of carnitine on hepatic steatosis induced by total parenteral nutrition in rats

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World Journal of Gastroenterology 1999年第4期5卷 40-43页

作者： LIANG Li Jian, YIN Xiao yu, luo shi Min, ZHENG Jin Fang, LU Ming De and HUANG Jie Fu

AIM To investigate the effects of carnitine on ameliorating hepatic steatosis induced by total parenteral nutrition (TPN) in animal model. METHODS Eighteen normal Wistar rats and 19 cirrhotic Wistar rats induced by carbon tetrachloride were randomly divided into three groups, i.e., free access to food and drink (group A), TPN (group B) and TPN+carnitine (group C) for one week, respectively. Hepatic function, histology and its fat content were determined on the 7th day. RESULTS Hepatic triglyceride (TG) and cholesterol (CHO) contents were significantly higher in groups B and C than in group A, and significantly lower in group C than in group B in both normal and cirrhotic rats (all P <0 05). Histopathological examinations revealed that hepatic steatosis was more severe in group B than in group C in both normal and cirrhotic rats. CONCLUSION Carnitine can ameliorate hepatic steatosis associated with TPN in both non cirrhotic and cirrhotic rats.

关键词： carnitine hepatic steatosis total parenteral nutrition rats

收费情形下一类弹性需求混合交通均衡的效率损失

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交通运输系统工程与信息 2018年第1期18卷 102-107页

作者：余孝军高世玉罗玲玲贵州财经大学数统学院贵阳550025

收费是降低交通均衡效率损失的一种重要方法,本文对收费情形下多用户类弹性需求交通均衡分配的效率损失进行了研究.首先,构建该类交通均衡分配在两类不同出行决策准则下的变分不等式模型.然后,通过解析推导法分别界定了其在不同出行决... 详细信息

收费是降低交通均衡效率损失的一种重要方法,本文对收费情形下多用户类弹性需求交通均衡分配的效率损失进行了研究.首先,构建该类交通均衡分配在两类不同出行决策准则下的变分不等式模型.然后,通过解析推导法分别界定了其在不同出行决策准则下的效率损失上界,并探讨了它们与网络参数的关系.最后,给出了数值算例.结果表明:两类效率损失上界都与路段出行时间函数、路段收费向量、出行者的出行时间价值系数、用户均衡时社会总收益与社会总剩余之比相关;费用度量出行决策准则下的效率损失上界还与系统最优时的社会总收益与均衡时社会总剩余之比有关.

关键词：城市交通效率损失弹性需求多用户类混合交通均衡分配收费

STUDIES ON THE LOW-TEMPERATURE SYNTHESIS OF METHANOL BY CARBONYLATION AND HYDROGENOLYSIS

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Journal of Energy Chemistry 1992年第3期 222-234页

作者： WU yu-Tang luo shi-Zhong JIA Zhao-Xia LIANG Yan Chengdu Institute of Organic Chemistry,Academia Sinica,Chengdu,Sichuan 610015 Received January 8,1992

The present process may be less energy intensive compared withthe existing processes.A proprietary amide aleohol additive can markedly in-hibit the deactivation of CHONa catalyst for methanol ***-time yield（STY）of the methyl formate（MF）is 625g·1hafter 40htime on stream with theadditive,while the yield is only 190g·1hwithout the additive at the same reaction *** using four types of tubu-lar reactors without any mechanico-rotary parts and hige speed circulation ofthe reactants for the carbonylation at P=5MPa,a 95% single pass conversion of CO and 645g·1hof STY of MF were obtained.A novel Cu-ZnO/AlOcatalyst for MF hydrogenolysis to methanol is reported in this *** Cu-ZnO/AlOcatalyst is characterized by the resistance to poison-ing of high concentration CO up to 16.2v%.

关键词： STUDIES ON THE LOW-TEMPERATURE SYNTHESIS OF METHANOL BY CARBONYLATION AND HYDROGENOLYSIS CO MF ZnO high

The interaction between KIF21A and KANK1 regulates dendritic morphology and synapse plasticity in neurons

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Neural Regeneration Research 2025年第1期20卷 209-223页

作者： shi-Yan Sun Lingyun Nie Jing Zhang Xue Fang Hongmei luo Chuanhai Fu Zhiyi Wei Ai-Hui Tang Hefei National Research Center for Physical Sciences at the Microscale CAS Key Laboratory of Brain Function and DiseaseMinistry of Education Key Laboratory for Membrane-less Organelles and Cellular DynamicsDivision of Life Sciences and MedicineUniversity of Science and Technology of ChinaHefeiAnhui ProvinceChina Institute of Artificial Intelligence Hefei Comprehensive National Science CenterHefeiAnhui ProvinceChina CAS Center for Excellence in Molecular Cell Sciences Division of Life Sciences and MedicineUniversity of Science and Technology of ChinaHefeiAnhui ProvinceChina Department of Neurobiology School of Life SciencesSouthern University of Science and TechnologyShenzhenGuangdong ProvinceChina Brain Research Center Southern University of Science and TechnologyShenzhenGuangdong ProvinceChina

Morphological alterations in dendritic spines have been linked to changes in functional communication between neurons that affect learning and ***-4 KIF21A helps organize the microtubule-actin network at the cell cortex by interacting with KANK1;however,whether KIF21A modulates dendritic structure and function in neurons remains *** this study,we found that KIF21A was distributed in a subset of dendritic spines,and that these KIF21A-positive spines were larger and more structurally plastic than KIF21A-negative ***,the interaction between KIF21A and KANK1 was found to be critical for dendritic spine morphogenesis and synaptic *** of either KIF21A or KANK1 inhibited dendritic spine morphogenesis and dendritic branching,and these deficits were fully rescued by coexpressing full-length KIF21A or KANK1,but not by proteins with mutations disrupting direct binding between KIF21A and KANK1 or binding between KANK1 and *** down KIF21A in the hippocampus of rats inhibited the amplitudes of long-term potentiation induced by high-frequency stimulation and negatively impacted the animals’cognitive *** together,our findings demonstrate the function of KIF21A in modulating spine morphology and provide insight into its role in synaptic function.

关键词： actin cytoskeleton dendrite KANK1 KIF21A microtubule spine morphology spine synaptic plasticity talin1

Effect of Bi/Si Ratio of BiBSi Glass on Its Structure, Properties and Laser Sealing Shear Strength for Vacuum Glazing

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Journal of Wuhan University of Technology(Materials Science) 2025年第1期40卷 13-24页

作者： LIU Wei JIAO Jinxu luo Dusha ZHOU Junjie shi Lifen WANG Weiwei LI Changqing WANG Peng XIONG Dehua LI Hong State Key Laboratory of Silicate Materials for Architectures Wuhan University of TechnologyWuhan 430070China Silica-Based Materials Laboratory of Anhui Province CNBM Research Institute for Advanced Glass Materials Group Co.LtdBengbu 233018China State Key Laboratory of Advanced Technology for Float Glass CNBM Research Institute for Advanced Glass Materials Group Co.LtdBengbu 233018China

The low-melting glass of Bi2O_(3)-B2O_(3)-SiO_(2)(BiBSi)system was used for the first time for laser sealing of vacuum *** the condition of constant boron content,how the structure and properties vary with Bi/Si ratio in low-melting glass was *** addition,the relationships between laser power,low-melting glass solder with different Bi/Si ratios and laser sealing shear strength were *** results show that a decrease in the Bi/Si ratio can cause a contraction of the glass network of the low-melting glass,leading to an increase of its characteristic temperature and a decrease of its coefficient of thermal *** laser sealing,the copper ions in the low-melting glass play an endothermic role.A change in the Bi/Si ratio will affect the valence state transition of the copper ions in the low-melting *** absorbance of the low-melting glass does not follow the expected correlation with the Bi/Si ratio,but shows a linear correlation with the content of divalent copper *** greater the concentration of divalent copper ions,the greater the absorbance of the low-melting glass,and the lower the laser power required for laser *** shear strength of the low melting glass solder after laser sealing was tested,and it was found that the maximum shear strength of Z1 glass sample was the highest up to 2.67 MPa.

关键词： Bi2O_(3)-B2O_(3)-SiO_(2)(BiBSi) Bi/Si ratio low-melting glass laser sealing vacuum glazing