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The MORC2 p.S87L mutation reduces proliferation of pluripotent stem cells derived from a patient with the spinal muscular atrophy-like phenotype by inhibiting proliferation-related signaling pathways

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Neural Regeneration Research 2024年第1期19卷 205-211页

作者： Sen zeng Honglan Yang Binghao Wang Yongzhi Xie Ke Xu Lei liu Wanqian Cao Xionghao liu Beisha ta.g Mujun liu Ruxu Zhang Department of Neurology The Third Xiangya HospitalCentral South UniversityChangshaHunan ProvinceChina Department of Neurology The First Affiliated Hospital of Zhengzhou UniversityZhengzhouHenan ProvinceChina Department of Radiology The Third Xiangya HospitalCentral South UniversityChangshaHunan ProvinceChina Health Management Center The Third Xiangya HospitalCentral South UniversityChangshaHunan ProvinceChina Center for Medical genetics&Hunan Key Laboratory of Medical genetics School of Life SciencesCentral South UniversityChangshaHunan ProvinceChina National Clinical Research Center for geriatric Disorders Central South UniversityChangshaHunan ProvinceChina Department of Cell Biology School of Life SciencesCentral South UniversityChangshaHunan ProvinceChina

Mutations in the microrchidia CW-type zinc finger protein 2(MORC2)gene are the causative agent of Charcot-Marie-Tooth disease type 2Z(CMT2Z),and the hotspot mutation p.S87L is associated with a more seve re spinal muscular atrophy-like clinical *** aims of this study were to determine the mechanism of the severe phenotype caused by the MORC2 p.S87L mutation and to explore potential treatment *** cells were isolated from urine samples from a spinal muscular atrophy(SMA)-like patient[MORC2 p.S87L),a CMT2Z patient[MORC2 p.Q400R),and a healthy control and induced to generate pluripotent stem cells,which were then differentiated into motor neuron precursor ***-generation RNA sequencing followed by KEgg pathway enrichment analysis revealed that differentially expressed genes involved in the PI3K/Akt and MAP K/ERK signaling pathways were enriched in the p.S87L SMA-like patient group and were significantly downregulated in induced pluripotent stem *** proliferation was observed in the induced pluripotent stem cells and motor neuron precursor cells derived from the p.S87L SMA-like patient group compared with the CMT2Z patient group and the healthy control.g0/g1 phase cell cycle arrest was observed in induced pluripotent stem cells derived from the p.S87L SMA-like ***2 p.S87Lspecific antisense oligonucleotides(p.S87L-ASO-ta.geting)showed significant efficacy in improving cell prolife ration and activating the PI3K/Akt and MAP K/ERK pathways in induced pluripotent stem *** r,p.S87L-ASO-ta rgeting did not rescue prolife ration of motor neuron precursor *** findings suggest that downregulation of the PI3K/Akt and MAP K/ERK signaling pathways leading to reduced cell proliferation and g0/g1 phase cell cycle arrest in induced pluripotent stem cells might be the underlying mechanism of the severe p.S87L SMA-like phenotype.p.S87L-ASO-ta.geting treatment can alleviate disordered cell proliferation in the early stag

关键词： antisense oligonucleotides cell cycle arrest Charcot-Marie-Tooth disease 2Z induced pluripotent stem cells MAPK/ERK PI3K/Akt proliferation spinal muscular atrophy-like

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Development of a novel Eu^(3+)-doped tantalate red-emitting phosphor for w-LEDs application

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Journal of Rare Earths 2024年第8期42卷 1479-1488,I0003页

作者： Fan zeng Jingjing Ru Bing Zhao Feiyun guo Jianzhong Chen College of Chemistry and Materials Fujian Province University Key Laboratory of Green Energy and Environment CatalysisNingde Normal University.Ningde352100China School of Environment and Resources School of Carbon Neutral and Modern IndustryFujian Normal UniversityFuzhou350007China College of Information and Mechanical&Electrical Engineering Ningde Normal UniversityNingde352100China College of Chemistry Fuzhou UniversityFuzhou350108China

Two novel phosphors LiBa_(4(1-x))Eu_(4x)ta_(3)O_(12)(H-LBTO:xEu^(3+)) and Li_(0.25)Ba_(1-x)Eu_(x)ta_(0.75)O_(3)(C-LBTO:xEu^(3+))were prepared successfully by a molten salt *** transformation between these two structures was realized by changing the sintering temperature or changing the Eu^(3+) ions concentration,which was also demonstrated by the X-ray diffraction(XRD),scanning electron microscopy(SEM),diffuse reflectance spectra(DRS),and photoluminescence excitation(PLE) analyses,Both the sintering temperature and the Eu^(3+)ions doping concentration have significant impact on the formation of the crystal *** these phosphors sintered at 1023 K exhibit two major luminescence lines at 594 and 614 nm under nearUV light of 395 nm excitation,corresponding to Eu^(3+)ions typical transitions of ^(5)D_(0)→^(7)F_(1) and ^(5)D_(0)→^(7)F_(2).The optimum concentration of Eu^(3+) ions is 9 mol% for C-LBTO:xEu^(3+) samples and the quenching interaction type is the nearest-neighbor ion *** thermal stability of the C-LBTO:0.09Eu^(3+)sample was investigated in detail and the device application further suggests that C-LBTO:0.09Eu^(3+) can be used as a red phosphor for near-UV excited w-LEDs in lighting.

关键词： Phosphor tantalate LED device Rare earths

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Nb-and ta-doped(Hf,Zr,Ti)C multicomponent carbides with enhanced oxidation resistance at 2500℃

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Journal of Advanced Ceramics 2024年第3期13卷 332-344页

作者： Shiyan Chen Jinming Wang Zhaoke Chen Weilong Song Yi zeng Xingchao Li Tongqi Li Xiang Xiong State Key Laboratory of Powder Metallurgy Central South UniversityChangsha 410083China Aerospace Research Institute of Materials&Processing Technology China Academy of Launch Vehicle TechnologyBeijing 100076China

Enhancing oxidation resistance of multicomponent carbides above 2000℃is critical for their thermal protection *** this purpose,novel Nb-and ta-doped(Hf,Zr,Ti)C multicomponent carbides were designed to improve their oxidation resistance at 2500℃.The results demonstrated that Nb and ta doping reduced the oxidation rate constant by 16.67%and 25.17%,respectively,thereby significantly improving the oxidation resistance of(Hf,Zr,Ti)*** enhancement was attributed to the changes in oxycarbide composition and distribution within the oxide layer by adding Nb and *** to the different oxidation tendencies of the constituent elements,a distinctive structure was formed in which(Hf,Zr)O_(2)served as a skeleton,and various oxycarbides were dispersed throughout the oxide *** doped Nb and ta were retained within oxycarbides,retarding the diffusion of oxygen into the *** importantly,the addition of Nb and ta reduced the size of oxycarbides,decreasing both size and quantity of the pores in the oxide layer and facilitating the formation of a more effective oxygen barrier.

关键词： (Hf,Zr,Ti)C oxidation behaviour ultra-high-temperature oxidation Nb and ta doping oxycarbides

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Interfacial modification using the cross-linkable tannic acid for highly-efficient perovskite solar cells with excellent stability

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Journal of Energy Chemistry 2024年第4期91卷 236-244页

作者： Xing gao Lirong Rong Fei Wu Yen-Hung Lin Ye zeng Junhong tan Rongxing He Cheng Zhong Linna Zhu Chongqing Key Laboratory for Advanced Materials and Technologies of Clean Energy School of Materials&EnergySouthwest UniversityChongqing 400715China Hubei Key Lab on Organic and Polymeric Optoelectronic Materials Department of ChemistryWuhan UniversityWuhan 430072HubeiChina Key Laboratory of Luminescence Analysis and Molecular Sensing Ministry of EducationSchool of Chemistry and Chemical EngineeringSouthwest UniversityChongqing 400715China Department of Electronic and Computer Engineering The Hong Kong University of Science and TechnologyClear Water BayKowloon 999077Hong KongChina State Key Laboratory of Advanced Displays and Optoelectronics Technologies The Hong Kong University of Science and TechnologyClear Water BayKowloon 999077Hong KongChina

Although the performance of perovskite solar cells(PSCs)has been dramatically increased in recent years,stability is still the main obstacle preventing the PSCs from being *** device instability can be caused by a variety of reasons,including ions diffusion,surface and grain boundary defects,*** this work,the cross-linkable tannic acid(ta)is introduced to modify perovskite film through post-treatment *** numerous organic functional groups(–OH and C=O)in ta can interact with the uncoordinated Pb^(2+)and I^(-)ions in perovskite,thus passivating defects and inhibiting ions *** addition,the formed ta network can absorb a small amount of the residual moisture inside the device to protect the perovskite ***,ta modification regulates the energy level of perovskite,and reduces interfacial charge ***,following ta treatment,the device efficiency is increased significantly from 21.31%to 23.11%,with a decreased hysteresis ***,the treated device shows excellent air,thermal,and operational *** light of this,the readily available,inexpensive ta has the potential to operate as a multipurpose interfacial modifier to increase device efficiency while also enhancing device stability.

关键词： tannic acid Defect passivation lons diffusion Hydrophilic Stability Perovskite solar cells

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Hexafluoropropylene oxide trimer acid,a perfluorooctanoic acid alternative,induces cardiovascular toxicity in zebrafish embryos

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Journal of Environmental Sciences 2024年第5期139卷 460-472页

作者： Sujie Sun Li Zhang Xue Li Lu Zang Ling Huang Junquan zeng Zigang Cao Xinjun Liao Zilin Zhong Huiqiang Lu Jianjun Chen Jiangxi Engineering Laboratory of Zebrafish Modeling and Drug Screening for Human Diseases Jiangxi Key Laboratory of Developmental Biology of OrgansCenter for Clinical Research Center of the Affiliated Hospital of Jinggangshan UniversityJi’an 343009China Translational Research Institute of Brain and Brain-like Intelligence Shanghai Key Laboratory of Anesthesiology and Brain Functional ModulationDepartment of PediatricsShanghai Fourth People’s HospitalSchool of MedicineTongji UniversityShanghai 200434China State Environmental Protection Key Laboratory of Environmental Health Impact Assessment of Emerging Contaminants School of Environmental Sciences and EngineeringShanghai Jiao Tong UniversityShanghai 200240China Department of Interventional and Vascular Surgery Affiliated Hospital of Jinggangshan UniversityJi’an 343009China Department of Internal Medicine and Hematology Affiliated Hospital of Jinggangshan UniversityJi’an 343009China

As an increasingly used alternative to perfluorooctanoic acid(PFOA),hexafluoropropylene oxide trimer acid(HFPO-ta)has been widely detected in global water ***,little is known regarding its toxic effects on cardiovascular ***,zebrafish embryos were treated with egg water containing 0,60,120,or 240 mg/L *** showed that HFPO-ta treatment led to a significant reduction in both larval survival percentage and heart ***,HFPO-ta exposure caused severe pericardial edema and elongation of the sinus venous to bulbus arteriosus distance(SV-BA)in Tg(myl7:gFP)transgenic larvae,disrupting the expression of genes involved in heart development and thus causing abnormal heart *** sprouting angiogenesis was observed in the 120 and 240 mg/L exposed Tg(fli:gFP)transgenic ***-ta treatment also impacted the mRNA levels of genes involved in the vascular endothelial growth factor(VEgF)pathway and embryonic vascular ***-ta exposure significantly decreased erythrocyte number in Tg(gata1:DsRed)transgenic embryos and influenced gene expression associated with the heme metabolism ***-ta also induced oxidative stress and altered the transcriptional levels of genes related to cell cycle and apoptosis,inhibiting cell proliferation while promoting ***,HFPO-ta exposure may induce abnormal development of the cardiovascular and hematopoietic systems in zebrafish embryos,suggesting it may not be a suitable or safe alternative for PFOA.

关键词： Hexafluoropropylene oxide trimer acid Heart development Vascular development Hemopoiesis Zebrafish embryos

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Development of red phosphor Li_(8)CaLa_(2)ta_(2)O_(13):Eu^(3+)for WLEDs and anti-counterfeiting applications

ChemPhysMater

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ChemPhysMater 2024年第2期3卷 194-203页

作者： Jingjing Ru Fan zeng Bing Zhao Chenqing Ye Tonghui Zhong Feiyun guo Jianzhong Chen College of Chemistry and Materials Fujian Province University Key Laboratory of Green Energy and Environment CatalysisNingde Normal UniversityNingde 352100China School of Environment and Resources School of Carbon Neutral and Modern IndustryFujian Normal UniversityFuzhou 350007China College of Information and Mechanical&Electrical Engineering Ningde Normal UniversityNingde 352100China College of Chemistry Fuzhou UniversityFuzhou 350108China

A set of novel red phosphors Li_(8)CaLa_(2)ta_(2)O_(13):Eu^(3+)(LCLTO:xEu^(3+))were successfully prepared using a solid-state reaction *** properties of the prepared samples,including phase purity,elemental composition,and morphology,were systematically investigated using X-ray diffraction,scanning electron microscopy,and diffuse reflectance spectroscopy *** 610 nm maximum emission peak is attributed to the^(5)D_(0)→^(7)F_(2)transition of Eu^(3+)ion under 394 nm *** all the LCLTO:xEu^(3+)phosphors,LCLTO:0.6Eu^(3+)showed the strongest emission intensity because of the concentration quenching effect of the electric dipole-dipole in-teraction among the Eu^(3+)ions,which was also demonstrated by the decay ***,the emission intensity of the optimal LCLTO:0.6Eu^(3+)phosphor,which exhibited a high internal quantum efficiency of 49.30%and excellent color purity of 96.79%,was approximately 2.29 times higher than that of commercial Y203:Eu^(3+)red *** thermal stability of the LCLTO:0.6Eu^(3+)sample with good color stability was meticulously *** fabricated white-light-emitting diode(WLED)exhibited a superior color-rendering index of Ra=82 and chromaticity coordinates of(0.3260,0.3639),suggesting that LCLTO:0.6Eu^(3+)has potential applicability in developing efficient and high-quality ***,the prepared LCLTO:0.6Eu^(3+)/PDMS composite film demonstrated exceptional flexural resistance and chemical stability,indicating considerable promise for practical anti-counterfeitingapplications.

关键词： Phosphor tantalate Luminescence LED device

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基于LTCC的3D异质集成低通滤波器

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微波学报 2024年第4期40卷 69-73页

作者：曾竞涛沈斌徐金旭沓世我章秀银华南理工大学电子与信息学院广州510641 广东风华高新科技股份有限公司肇庆526060 新型电子元器件关键材料与工艺国家重点实验室肇庆526060

在射频应用中,低温共烧陶瓷(LTCC)技术是关键技术路线之一,然而使用单一介质的LTCC技术的性能很局限。为此,文中对基于LTCC的3D异质集成技术进行研究,通过调节流延配方、烧结速度、烧结温度、保温时间等工艺参数,设计异质匹配共烧的烧... 详细信息

在射频应用中,低温共烧陶瓷(LTCC)技术是关键技术路线之一,然而使用单一介质的LTCC技术的性能很局限。为此,文中对基于LTCC的3D异质集成技术进行研究,通过调节流延配方、烧结速度、烧结温度、保温时间等工艺参数,设计异质匹配共烧的烧结行为控制、界面相容性和组织性能调控方案,开发出稳定可靠的LTCC集成工艺,给出基于LTCC异质集成的器件仿真设计、3D集成制备方法,并制作了一款共烧界面结合完好、性能优越的LTCC异质集成低通滤波器进行验证。验证结果表明,该异质集成技术的瓷粉性能指标和可加工特性满足使用要求,预期能够得到广泛应用。

关键词：低温共烧陶瓷 3D异质集成低通滤波器

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Plume-lithosphere interaction in the Comei Large Igneous Province: Evidence from two types of mafic dykes in gyangze, south Tibet, China

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China geology 2024年第1期7卷 80-90页

作者： Ya-ying Wang Ling-sen zeng Li-e gao Li-long Yan Ling-hao Zhao Jia-hao gao Ying-long Di guang-xu Li Yi-hong Tian Institute of geology Chinese Academy of Geological SciencesBeijing 100037China National Research Center for geoanalysis Chinese Academy of Geological SciencesBeijing 100037China China Nonferrous Metals(guilin)geology and Mining Co. LtdGuilin 541004China

Two suites of mafic dykes,T1193-A and T1194-A,outcrop in gyangze area,southeast *** are in the area of Comei LIP and have indistinguishable field occurrences with two other dykes in gyangze,T0902 dyke with 137.7±1.3 Ma zircon age and T0907 dyke with 142±1.4 Ma zircon age reported by Wang YY et al.(2016),indicating coeval formation *** all the four diabase dykes into consideration,two different types,OIB-type and weak enriched-type,can be ***“OIB-type”samples,including T1193-A and T0907 dykes,show OIB-like geochemical features and have initial Sr-Nd isotopic values similar with most mafic products in Comei Large Igneous Provinces(LIP),suggesting that they represent melts directly generated from the Kerguelen mantle ***“weak enriched-type”samples,including T1194-A and T0902 dykes,have REEs and trace element patterns showing withinplate affinity but have obvious Nb-ta-Ti negative *** show uniform lowerεNd(t)values(−6‒−2)and higher 87Sr/86Sr(t)values(0.706‒0.709)independent of their MgO variation,indicating one enriched mantle *** their closely spatial and temporal relationship with the widespread Comei LIP magmatic products in Tethyan Himalaya,these“weak enriched-type”samples are consistent with mixing of melts from mantle plume and the above ancient Tethyan Himalaya subcontinental lithospheric mantle(SCLM)in different *** weak enriched mafic rocks in Comei LIP form one special rock group and most likely suggest large scale hot mantle plume-continental lithosphere *** process may lead to strong modification of the Tethyan Himalaya lithosphere in the Early Cretaceous.

关键词： OIB type diabase Comei Large Igneous Province Weakly enriched diabase Plume-lithosphere interaction Nb-ta-Ti negative anomaly Kerguelen plume geological survey engineering Tibet Plateau

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Direct synthesis of large-area Al-doped graphene by chemical vapor deposition:Advancing the substitutionally doped graphene family

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Nano Research 2022年第2期15卷 1310-1318页

作者： Sami Ullah Yu liu Maria Hasan Wenwen zeng Qitao Shi Xiaoqin Yang Lei Fu Huy Q.ta Xueyu Lian Jingyu Sun Ruizhi Yang Lijun liu Mark H.Rummeli College of Energy Soochow Institute for Energy and Materials Innovations Soochow UniversitySuzhou 215006China Key Laboratory of Advanced Carbon Materials and Wearable Energy Technologies of Jiangsu Province Soochow UniversitySuzhou 215006China NUTECH School of Applied Sciences&Humanities and Computer Engineering Department National University of TechnologyIslamabad 42000Pakistan Centre of Polymer and Carbon Materials Polish Academy of SciencesM.Curie-Sklodowskiej 34Zabrze 41-819Poland School of Energy and Power Engineering Xian Jiaotong UniversityXian 710049China College of Chemistry and Molecular Science Wuhan UniversityWuhan 430072China Institute for Complex Materials IFW Dresden 20 Helmholtz Strasse Dresden 01069Germany Institute of Environmental Technology VSB-Technical University of Ostrava17.listopadu 15Ostrava 70833Czech Republic

graphene doping continues to gather momentum because it enables graphene properties to be tuned,thereby affording new properties to,improve the performance of,and expand the application potential of *** can be chemically doped using various methods such as surface functionalization,hybrid composites(e.g.,nanoparticle decoration),and substitution doping,wherein C atoms are replaced by foreign ones in the graphene *** works have predicted that graphene could be substitutionally doped by aluminum(Al)atoms,which could hold promise for exciting applications,including hydrogen storage and evolution,and *** theoretical predictions suggest that Al substitutionally doped graphene(AIg)could serve as a material for gas sensors and the catalytic decomposition of undesirable ***,fabricating Al substitutionally doped graphene has proven challenging until ***,we demonstrate how controlled-flow chemical vapor deposition(CVD)implementing a simple solid precursor can yield high-quality and large-area monolayer AIg,and this synthesis is unequivocally confirmed using various characterization methods including local electron energy-loss spectroscopy(EELS).Detailed high-resolution transmission electron microscopy(HRTEM)shows numerous bonding configurations between the Al atoms and the graphene lattice,some of which are not theoretically ***,the produced AIg shows a CO_(2) capturability superior to those of other substitutionally doped graphenes.

关键词： aluminum-doped graphene single-solid precursor chemical vapor deposition carbon dioxide capture energy storage catalytic applications

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基于区块链的白盒化光网络故障检测

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光通信技术 2024年第2期48卷 72-77页

作者：曾创展蔡晨贾农姚耀刘伟杨辉 61932部队北京100000 北京邮电大学信息光子学与光通信国家重点实验室北京100876

针对白盒化通信网设备异常检测问题,提出了基于区块链的白盒化光网络故障检测方案。首先,设计了面向数据收集的区块链架构和基于区块链的数据收集与存储方案;然后,又设计了基于自编码器的数据异常检测算法;最后,将自编码器模型部署至区... 详细信息

针对白盒化通信网设备异常检测问题,提出了基于区块链的白盒化光网络故障检测方案。首先,设计了面向数据收集的区块链架构和基于区块链的数据收集与存储方案;然后,又设计了基于自编码器的数据异常检测算法;最后,将自编码器模型部署至区块链共识设备中,形成完整的基于区块链的异常检测机制。实验结果表明:在不同设备的采集数据下,所提方案可以快速实现数据上链存储,且能准确、快速地识别设备的异常数据,实现网络设备的实时异常检测。

关键词：白盒化光网络异常检测区块链自编码器

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