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1.
Sialidosis, caused by a genetic deficiency of the lysosomal sialidase gene (NEU1), is a systemic disease involving various tissues and organs, including the nervous system. Understanding the neurological dysfunction and pathology associated with sialidosis remains a challenge, partially due to the lack of a human model system. In this study, we have generated two types of induced pluripotent stem cells (iPSCs) with sialidosis-specific NEU1G227R and NEU1V275A/R347Q mutations (sialidosis-iPSCs), and further differentiated them into neural precursor cells (iNPCs). Characterization of NEU1G227R- and NEU1V275A/R347Q- mutated iNPCs derived from sialidosis-iPSCs (sialidosis-iNPCs) validated that sialidosis-iNPCs faithfully recapitulate key disease-specific phenotypes, including reduced NEU1 activity and impaired lysosomal and autophagic function. In particular, these cells showed defective differentiation into oligodendrocytes and astrocytes, while their neuronal differentiation was not notably affected. Importantly, we found that the phenotypic defects of sialidosis-iNPCs, such as impaired differentiation capacity, could be effectively rescued by the induction of autophagy with rapamycin. Our results demonstrate the first use of a sialidosis-iNPC model with NEU1G227R- and NEU1V275A/R347Q- mutation(s) to study the neurological defects of sialidosis, particularly those related to a defective autophagy–lysosome pathway, and may help accelerate the development of new drugs and therapeutics to combat sialidosis and other LSDs. 相似文献
2.
Fanbo Meng Mingchang Zhang Jin Huang Wen Feng Lu Jun Min Xue Hao Wang 《Advanced functional materials》2021,31(9):2008280
The development of the Internet of things has prompted an exponential increase in the demand for flexible, wearable devices, thereby posing new challenges to their integration and conformalization. Additive manufacturing facilitates the fabrication of complex parts via a single integrated process. Herein, the development of a multinozzle, multimaterial printing device is reported. This device accommodates the various characteristics of printing materials, ensures high-capacity printing, and can accommodate a wide range of material viscosities from 0 to 1000 Cp. Complete capacitors, inclusive of the current collector, electrode, and electrolyte, can be printed without repeated clamping to complete the preheating, printing, and sintering processes. This method addresses the poor stability issue associated with printed electrode materials. Furthermore, after the intercalation of LiFePO4 with Na ions, X-ray photoelectron spectroscopy and X-ray diffraction results reveal that the Na ions permeate the interlayer structure of LiFePO4, enhancing the ion migration channels by increasing the ion transmission rate. A current rate of 2.5 mAh ensures >2000 charge/discharge cycles, while retaining a charge/discharge efficiency of 96% and a discharge capacity of 91.3 mAh g−1. This manufacturing process can provide conformal power modules for a diverse range of portable devices with various shapes, improving space utilization. 相似文献
3.
为分析地表径流速度对城市内涝的影响,采用一维地下排水管网与二维城区地形的动态耦合模型,选取大连市某排水区块作为研究区域,设置4种地表径流速度10种设计降雨场景,模拟分析在不同重现期设计降雨及不同地表径流速度下研究区的内涝积水特性。结果表明:随着地表径流速度降低,管道排水压力变小,管道排水达标率最高可提升48.05%,且降雨重现期越短,地表径流流速对管道排水压力的削减效果越明显;地表径流流速对检查井溢流量影响显著,随着地表径流速度降低,检查井溢流量峰值最高可减小2 750 m~3,峰现时间最长可滞后56 min,同时随着降雨重现期增长,地表径流流速对检查井溢流量的削减效果减弱;研究区低、高风险区淹没面积随地表径流速度降低,最高可分别减小1.64万、8.37万m~2,但中风险区淹没面积变化反复。 相似文献
4.
Gerasimov Sergey I. Ilyushin Mikhail A. Putis Sergey M. Dushenok Sergey A. Shugalei Irina V. Sudarikov Andrey M. 《含能材料》2022,30(4):370-378
In the present work it is found that the pyrotechnic composition VS-2 can be initiated with flash lamps IFC-500 and EVIS. VS-2 pyrotechnic composition contains 90% of mercury(Ⅱ) 5-hydrazinotetrazolate perchlorate and 10% of optically transparent copolymer of 2-methyl-5-vinyltetrazole and methacrylic acid (PVMT). We have found that the flash lamps make it possible to initiate combustion of VS-2 composition with its transition to detonation both in cylindrical charges placed in brass caps of 5 mm diameter and 2 mm high, and film charges with 10 mm×80 mm in size and surface weights of 60 mg·cm-2 and 90 mg·cm-2, showing ignition delay times 10 μs and 3 μs, respectively. We also measured detonation velocities for VS-2 composition film charges, which were 4375-4505 m·s-1 (of the charge being surface mass 60 mg·cm-2) and 4221-4281 m·s-1 (of the charge being surface mass 90 mg·cm-2) and their blasting action on the aluminum plate. The depths of the normal shock wave imprints at the charge-barrier interface were 0.6-0.7 mm (for surface mass of the film charges 60 mg·cm-2) and 1.2-1.3 mm (for surface mass of the film charges 90 mg·cm-2). 相似文献
5.
根据剪切解络-超滤过程中金属离子的动态浓度关系和质量守恒定律,采用CodeBlocks17.12软件编写程序,建立了剪切解络-超滤工艺关键指标预测模型,实现了金属离子浓度、回收率、体积稀释倍数等指标的预测,并用实验加以验证。依次在转速800 r/min、1 400 r/min、3 000 r/min下回收模拟废水中43 μg/L的Co2+、995 μg/L的Ni2+和88 μg/L的La3+,3种金属的回收率均达99.8%以上时,超滤过程体积稀释倍数的预测值分别为7.0、7.5、5.75,实验值分别为7.5、7.25、6.25,可见预测值与实验值基本一致。本模型对研究方案制定和调整有实际指导意义。 相似文献
6.
诱导式卫星欺骗干扰可诱导航空器逐渐偏离预定航迹,难以被发现,因此及时有效地检测干扰是飞行安全的保障。在现有紧组合导航体制基础上,设计了一种基于误差估值累加开环校正的紧组合导航结构,并证明了其性能与传统闭环校正紧组合导航性能等效。在此结构中,将紧组合导航系统与自适应序贯概率比检测方法结合,提出了一种基于误差估值累加开环校正的诱导式欺骗检测方法,融合紧组合导航信息与其他不受欺骗影响的导航信息,构建欺骗检测统计量进行诱导式欺骗检测。仿真结果表明,开环校正结构可避免随时间累加的惯性导航系统误差所导致的组合导航滤波器发散问题,同时欺骗检测方法可进一步提高算法对“最坏”情形下微小诱导式欺骗的检测效果。 相似文献
7.
寒区河道凌汛灾害河势“弯道效应”的量化评估十分重要。基于分形理论提出河道横断面-纵剖面-平面多维度河势分形维数计算方法及其物理机制,并探讨黄河内蒙古段不同维度河势演变分形特征及其与凌汛灾害的关联关系。结果表明,黄河内蒙古段不同维度河势均具有多尺度自相似分形特征,且具有多年记忆周期的长程相关性;冰坝(严重性冰塞)发生频次与河道主槽弯曲分形维数呈正相关指数型函数关系,与河相系数、深泓点高程和河段平均底坡分形维数负相关,与水深-面积分形维数正相关,总体表明冰坝灾害更易发生于主槽偏移摆动大、蜿蜒曲折、河湾发育程度高的宽浅型弯曲河道,研究成果可为凌汛期冰塞冰坝灾害易发河段诊断及预测提供重要理论依据。 相似文献
8.
《岩石力学与岩土工程学报(英文版)》2022,14(5):1556-1567
Wind erosion is one of the significant natural calamities worldwide, which degrades around one-third of global land. The eroded and suspended soil particles in the environment may cause health hazards, i.e. allergies and respiratory diseases, due to the presence of harmful contaminants, bacteria, and pollens. The present study evaluates the feasibility of microbially induced calcium carbonate precipitation (MICP) technique to mitigate wind-induced erosion of calcareous desert sand (Thar desert of Rajasthan province in India). The temperature during biotreatment was kept at 36 °C to stimulate the average temperature of the Thar desert. The spray method was used for bioaugmentation of Sporosarcina (S.) pasteurii and further treatment using chemical solutions. The chemical solution of 0.25 pore volume was sprayed continuously up to 5 d, 10 d, 15 d, and 20 d, using two different concentration ratios of urea and calcium chloride dihydrate viz 2:1 and 1:1. The biotreated samples were subjected to erosion testing (in the wind tunnel) at different wind speeds of 10 m/s, 20 m/s, and 30 m/s. The unconfined compressive strength of the biocemented crust was measured using a pocket penetrometer. The variation in calcite precipitation and microstructure (including the presence of crystalline minerals) of untreated as well as biotreated sand samples were determined through calcimeter, scanning electron microscope (SEM), and energy-dispersive X-ray spectroscope (EDX). The results demonstrated that the erosion of untreated sand increases with an increase in wind speeds. When compared to untreated sand, a lower erosion was observed in all biocemented sand samples, irrespective of treatment condition and wind speed. It was observed that the sample treated with 1:1 cementation solution for up to 5 d, was found to effectively resist erosion at a wind speed of 10 m/s. Moreover, a significant erosion resistance was ascertained in 15 d and 20 d treated samples at higher wind speeds. The calcite content percentage, thickness of crust, bulk density, and surface strength of biocemented sand were enhanced with the increase in treatment duration. The 1:1 concentration ratio of cementation solution was found effective in improving crust thickness and surface strength as compared to 2:1 concentration ratio of cementation solution. The calcite crystals formation was observed in SEM analysis and calcium peaks were observed in EDX analysis for biotreated sand. 相似文献
9.
Can Ercan S. Esra Safran Artuğ Türkmenoğlu Uğur Karabakal 《Journal of surfactants and detergents》2021,24(1):139-147
Garzan oil field is located at the south east of Turkey. It is a mature oil field and the reservoir is fractured carbonate reservoir. After producing about 1% original oil in place (OOIP) reservoir pressure started to decline. Waterflooding was started in order to support reservoir pressure and also to enhance oil production in 1960. Waterflooding improved the oil recovery but after years of flooding water breakthrough at the production wells was observed. This increased the water/oil ratio at the production wells. In order to enhance oil recovery again different techniques were investigated. Chemical enhanced oil recovery (EOR) methods are gaining attention all over the world for oil recovery. Surfactant injection is an effective way for interfacial tension (IFT) reduction and wettability reversal. In this study, 31 different types of chemicals were studied to specify the effects on oil production. This paper presents solubility of surfactants in brine, IFT and contact angle measurements, imbibition tests, and lastly core flooding experiments. Most of the chemicals were incompatible with Garzan formation water, which has high divalent ion concentration. In this case, the usage of 2-propanol as co-surfactant yielded successful results for stability of the selected chemical solutions. The results of the wettability test indicated that both tested cationic and anionic surfactants altered the wettability of the carbonate rock from oil-wet to intermediate-wet. The maximum oil recovery by imbibition test was reached when core was exposed 1-ethly ionic liquid after imbibition in formation water. Also, after core flooding test, it is concluded that considerable amount of oil can be recovered from Garzan reservoir by waterflooding alone if adverse effects of natural fractures could be eliminated. 相似文献
10.