Modeling Sialidosis with Neural Precursor Cells Derived from Patient-Derived Induced Pluripotent Stem Cells

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 NEU1^G227R and NEU1^V275A/R347Q mutations (sialidosis-iPSCs), and further differentiated them into neural precursor cells (iNPCs). Characterization of NEU1^G227R- and NEU1^V275A/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 NEU1^G227R- and NEU1^V275A/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.     

Influence of temperature on acoustic emission source location accuracy in underground structure

An acoustic emission (AE) experiment was carried out to explore the AE location accuracy influenced by temperature. A hollow hemispherical specimen was used to simulate common underground structures. In the process of heating with the flame, the pulse signal of constant frequency was stimulated as an AE source. Then AE signals received by each sensor were collected and used for comparing localization accuracy at different temperatures. Results show that location errors of AE keep the same phenomenon in the early and middle heating stages. In the later stage of heating, location errors of AE increase sharply due to the appearance of cracks. This provides some beneficial suggestions on decreasing location errors of structural cracks caused by temperature and improves the ability of underground structure disaster prevention and control.     

Achieving excellent energy storage density of Pb0.97La0.02(ZrxSn0.05Ti0.95-x)O3 ceramics by the B-site modification

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 Pb_0.97La_0.02(Zr_xSn_0.05Ti_0.95-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 W_rec 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.     

不同盐沼湿地类型脱氮除磷效能及影响因素分析

采用沉水植物表面流湿地(沉水组)、挺水植物表面流湿地(挺水组)和浮床湿地(浮床组)3种盐沼湿地对长江口近岸低污染水体进行脱氮除磷效能的研究。结果表明,HRT为3 d时,水组、挺水组、浮床组对NO3^--N的去除率在高温时段分别为79.9%±13.2%、71.8%±15.2%、77.2%±13.2%,中温时段分别为39.4%±13.7%、31.5%±8.5%、18.4%±16.6%,低温时段分别为15.6%±14.6%、19.7%±8.6%、2.%5±8.6%。沉水组和挺水组对TP的去除率受温度影响较小,分别为66.4%±32.4%、55.5%±29.4%;而浮床组除磷效果受温度影响较大。当HRT缩短为1.5 d时,3组湿地系统在高温时段仍可达到相近的脱氮除磷效果,在中低温时段脱氮除磷效果都有不同程度的下降。     

Microstructure induced ultra-high energy storage density coupled with rapid discharge properties in lead-free Ba0.9Ca0.1Ti0.9Zr0.1O3–SrNb2O6 ceramics

Novel lead-free (1-x)Ba_0·9Ca_0·1Ti_0·9Zr_0·1O₃-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 Ba_0·9Ca_0·1Ti_0·9Zr_0·1O₃ (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.     

一种新型的室温快速固化胶黏剂的研制

本文开发了一种新型的方舱夹芯板用室温固化高强度环氧结构胶黏剂,验证了其物化特性、相关力学性能和环境适应性。结果表明此胶黏剂具有优良性能,可以满足方舱用大板胶黏剂的使用需求。     

Thiol-Functionalized Covalent Organic Frameworks as Thermal History Indictor for Temperature and Time History Monitoring

Various products, including foods and pharmaceuticals, are sensitive to temperature fluctuations. Thus, temperature monitoring during production, transportation, and storage is critical. Facile indicators are required to monitor temperature conditions via color changes in real time. This study aimed to prepare and apply thiol-functionalized covalent organic frameworks (COFs) as a novel indicator for monitoring thermal history and temperature abuse. The COFs underwent obvious color changes from bright yellow to purple after exposure to different temperatures for varying durations. The reaction kinetics are analyzed under isothermal conditions, which reveal that the order of reaction rates is k_−20°C < k_4°C < k_20°C < k_35°C < k_55°C. The activation energy (E_a) of the COFs is calculated using the Arrhenius equation as 50.71 kJ moL⁻¹. The COFs are capable of sensitive color changes and offer a broad temperature tracking range, thereby demonstrating their application potential for the monitoring of temperature and time exposure history during production, transportation, and storage. This excellent performance thermal history indicator also shows promise for expanding the application field of COFs.     

Superior Low-Temperature Reversible Adhesion Based on Bio-Inspired Microfibrillar Adhesives Fabricated by Phenyl Containing Polydimethylsiloxane Elastomers

Gecko-inspired microfibrillar adhesives have achieved great progress in microstructure design and adhesion improvement over the past two decades. Space applications nowadays show great interest in this material for the characteristics of reversible adhesion and universal van der Waals interactions. However, the impact of harsh environment of space on the performance of microfibrillar adhesives, especially the extreme low temperature, is rarely addressed. Herein, microfibrillar adhesives fabricated by phenyl containing polydimethylsiloxane (p-PDMS) elastomers with superior low-temperature reversible adhesion is proposed. p-PDMS elastomers are synthesized through one-pot anionic ring-opening copolymerization, and the resulting elastomers become non-crystallizable with excellent low-temperature elasticity. Low-temperature adhesion tests demonstrate that the adhesion strength of microfibrillar adhesives fabricated by p-PDMS elastomers can be well maintained to as low as −120 °C. In contrast, the adhesion strength of pure PDMS microfibrillar adhesive reduces more than 50% below its crystallization temperature. The low-temperature cyclic adhesion tests further demonstrate that p-PDMS microfibrillar adhesives exhibit superior reversible adhesion compared to that of PDMS microfibrillar adhesives, owing to the sustainable conformal contact and even distribution of loads over repeated cycles. This study provides a new fabrication strategy for microfibrillar adhesives, and is beneficial for the practical application of microfibrillar adhesives.