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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.
Yi Qin Tao Xiong Ting Zhao Jianfeng Zhu Jun Yang Yi Liu Xingang Kong Xiaohan Li 《Ceramics International》2021,47(9):12641-12650
The strengthening method of multi-element M-site solid solution is a common approach to improve mechanical properties of MAX phase ceramic. However, the research on capability of multi-element A-site solid solution to improve mechanical properties has rarely been reported. Thereupon, quasi-high-entropy MAX phase ceramic bulks of Ti2(Al1?xAx)C and Ti3(Al1?xAx)C2 (A = Ga, In, Sn, x = 0.2, 0.3, 0.4) were successfully synthesized by in situ vacuum hot pressing via multi-elements solid solution. The multi-elements solid solution in single-atom thick A layer was confirmed by X-ray diffraction and X-ray photoelectron spectroscopy as well as by energy dispersive X-ray spectroscopy mappings. Effects of doped multi-elements contents on the phase, microstructure, mechanical properties, and high temperature tribological behaviors were studied. Results demonstrated that the Vickers hardness, anisotropic flexural strength, fracture toughness, and tribological properties of Ti–Al–C based MAX ceramics could be remarkably improved by constitution of quasi-high-entropy MAX phase in A layers. Moreover, the strengthening and wear mechanisms were also discussed in detail. This method of multi-element solid solution at A-site provides new way to enhance mechanical properties of other MAX phase ceramics. 相似文献
3.
《中国有色金属学会会刊》2021,31(10):3063-3074
The transient liquid phase (TLP) bonding of CoCuFeMnNi high entropy alloy (HEA) was studied. The TLP bonding was performed using AWS BNi-2 interlayer at 1050 °C with the TLP bonding time of 20, 60, 180 and 240 min. The effect of bonding time on the joint microstructure was characterized by SEM and EDS. Microstructural results confirmed that complete isothermal solidification occurred approximately at 240 min of bonding time. For samples bonded at 20, 60 and 180 min, athermal solidification zone was formed in the bonding area which included Cr-rich boride and Mn3Si intermetallic compound. For all samples, the γ solid solution was formed in the isothermal solidification zone of the bonding zone. To evaluate the effect of TLP bonding time on mechanical properties of joints, the shear strength and micro-hardness of joints were measured. The results indicated a decrement of micro-hardness in the bonding zone and an increment of micro-hardness in the adjacent zone of joints. The minimum and maximum values of shear strength were 100 and 180 MPa for joints with the bonding time of 20 and 240 min, respectively. 相似文献
4.
Yujing Zhang Pin Liu Kumara Raja Kandula Wenru Li Yanfeng Qin Haibo Zhang Guangzu Zhang 《Journal of the European Ceramic Society》2021,41(1):360-367
The applications of antiferroelectric (AFE) materials in miniaturized and integrated electronic devices are limited by their low energy density. To address the above issue, the antiferroelectricity of the reinforced material was designed to improve its AFE-ferroelectric (FE) phase transition under electric fields. In this present study, the composition of Zr4+ (0.72 Å) and Ti4+ (0.605 Å) at B-site of Pb0.97La0.02(ZrxSn0.05Ti0.95-x)O3 ceramics with orthogonal reflections are synthesized via the tape-casting method. These ceramics are modified to enhance their antiferroelectricity by reducing their tolerance factor. A recoverable energy storage density Wrec 12.1 J/cm3 was obtained for x = 0.93 under 376 kV/cm, which is superior value than reported until now in lead-based energy storage systems. Moreover, the discharge energy density can reach 10.23 J/cm3, and 90 % of which can be released within 5.66 μs. This work provides a new window and potential materials for further industrialization of pulse power capacitors. 相似文献
5.
为了建立一种准确性高、成本低的植物油中苯并(a)芘测定的样品前处理方法,基于GB 5009.7—2016以自制氧化铝柱为固相萃取柱,采用单因素试验对称样量、洗脱剂量、洗脱流速、吸附剂存放时间、吸附剂量这5个影响植物油中苯并(a)芘洗脱的因素进行研究,优化前处理条件,并对测定方法进行考察。结果表明:优化的前处理条件为称样量0.100 0 g、洗脱剂量120 mL、吸附剂(氧化铝)量22 g、洗脱流速1滴/2 s、吸附剂存放时间少于12周;方法检出限为0.2 μg/L,样品加标回收率为94.23%~100.00%,RSD为1.20%~7.36%;同一样品测定结果与SGS测定值接近,相对平均偏差为2.36%~3.50%。说明本试验方法测定结果准确,可应用于油脂企业植物油中痕量苯并(a)芘的测定。 相似文献
6.
针对煤矿进口锚杆机除尘马达频繁出现故障的情况,通过排查除尘装置的安装方式和监测马达工作时的压力状态,查找故障原因,并制定解决方案。通过马达外接单向阀,可满足马达进油口最低安全压力限制的要求,解决了马达频繁损坏的问题,保障了设备工作的稳定性,提高了工作效率。 相似文献
7.
Novel lead-free (1-x)Ba0·9Ca0·1Ti0·9Zr0·1O3-xSrNb2O6 ceramics were synthesized via a two-step high energy ball milling process. The evolution of microstructural properties, phase transformation, and energy storage characteristics was comprehensively investigated to assess the applicability of material in multi-layered ceramic capacitors. The substitution of SrNb2O6 (SNO) in Ba0·9Ca0·1Ti0·9Zr0·1O3 (BTCZ) has resulted in substantial improvement in materials density along with a small increase in the grain size of the synthesized ceramic. A thorough microstructural investigation indicates an excellent dispersibility and compatibility between BTCZ and SNO phases. With an increase in SNO substitution, a transition from typical ferroelectric to relaxor ferroelectric has been observed, which has led to a significantly slimmer ferroelectric loop along with frequency dispersive dielectric properties. The optimized composition (i.e., x = 0.10) exhibits an ultra-high recoverable energy density of 2.68 J/cm3 along with a moderately high energy efficiency of 83.4%. Further, SNO substituted samples have also shown an enhancement in breakdown strength. The improvement in energy storage performance and breakdown strength of SNO substituted BTCZ composites are mainly attributed to relatively homogeneous grain morphology, optimum grain size, microstructural density, and improved grain boundary interface. 相似文献
8.
为了探讨氮含量及固溶温度对21-6-9不锈钢组织和硬度的影响,分别在950、1000、1050和1100 ℃对3种不同氮含量的热轧态21-6-9不锈钢进行1 h固溶处理,通过光学显微镜观察其组织结构,结合Thermo-Calc热力学计算对试验钢的微观组织进行分析,并对其进行硬度测试。结果表明,0.20%~0.28%N的21-6-9不锈钢热轧后沿轧制方向析出铁素体,且钢中铁素体经950~1100 ℃固溶处理可消除,当N含量达到0.34%时,试验钢中不再出现铁素体。随着固溶处理温度的升高,21-6-9不锈钢的晶粒组织长大,硬度降低。N含量的增加可显著提高固溶态21-6-9不锈钢的硬度,其增加程度随固溶处理温度的升高而减弱。 相似文献
9.
William M. Pitts Martin Werrel Marco Fernandez Mary A. Long Evan A. Eisenberg James Filliben Cory D. Runyon 《火与材料》2021,45(1):127-154
Fire spread and growth on real‐scale four cushion mock‐ups of residential upholstered furniture (RUF) were investigated with the goal of identifying whether changes in five classes of materials (barrier, flexible polyurethane foam, polyester fiber wrap, upholstery fabric, and sewing thread), referred to as factors, resulted in statistically significant changes in burning behavior. A fractional factorial experimental design plus practical considerations yielded a test matrix with 20 material combinations. Experiments were repeated a minimum of two times. Measurements included fire spread rates derived from video recordings and heat release rates (HRRs). A total of 13 experimental parameters (3 based on the videos and 10 on the HRR results), referred to as responses, characterized the measurements. Statistical analyses based on Main Effects Plots (main effects) and Block Plots (main effects and factor interactions) were used. The results showed that three of the factors resulted in statistically significant effects on varying numbers of the 13 responses. The Barrier and Fabric factors had the strongest main effects with roughly comparable magnitudes. Foam was statistically significant for fewer of the responses and its overall strength was weaker than for Barrier and Fabric. No statistically significant main effects were identified for Wrap or Thread. Multiple two‐term interactions between factors were identified as being statistically significant. The Barrier*Fabric interaction resulted in the highest number of and strongest statistically significant effects. The existence of two‐term interactions means that it will be necessary to consider their effects in approaches designed to predict the burning behavior of RUF. 相似文献
10.
Alessio Zandona Bernd Rüdinger Joachim Deubener 《Journal of the American Ceramic Society》2021,104(2):1146-1155
Glass powder samples of cordierite composition (doped with 8 mol% TiO2) were heat-treated to produce a series of increasingly SiO2-enriched Mg-bearing quartz solid solutions (Qss). The obtained materials were then analyzed by X-ray diffraction: Rietveld structural refinements revealed that Mg-bearing Qss phases possess trigonal symmetry and a compositionally dependent intermediate structural arrangement between those of low and high quartz. High-temperature diffraction measurements were performed up to 700°C to characterize the thermal expansion behavior of the crystals. At SiO2-rich compositions, a reversible high-to-intermediate inversion of the quartz structure is observed, which shifts with increasing stuffing to lower temperatures than the conventional 573°C for pure quartz. Similarities and differences to the better-established Li-bearing Qss are discussed in the text. 相似文献