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.     

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.     

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.     

Embedding carbon dots in Eu3+-doped metal-organic framework for label-free ratiometric fluorescence detection of Fe3+ ions

Fluorescent detection is a new spectroscopic measurement for ions sensing due to the advantages of real-time determination with high selectivity, accuracy, and low cost. However, chemosensors based on fluorescent detection are usually determined by absolute intensity from a monochromatic emission signal, which is easy to be fluctuated by the external environment, especially for Fe³⁺ detection in complex fluids. Herein, we rationally design a dual-emission Eu³⁺: CDs@ZIF-8 to construct a ratiometric fluorescent sensor with self-calibrating ability for Fe³⁺ determination. High efficient carbon dots (CDs) are embedded in europium ions (Eu³⁺)-doped MOF by simple stirring preparation at room temperature. The label-free ratiometric fluorescent probe (I_CDs@ZIF-8/I_Eu) exhibits simultaneous blue and red emission under the same excitation at 365 nm. Remarkably, Eu³⁺: CDs@ZIF-8 displays the superiority of high selectivity to Fe³⁺, which shows ratiometric fluorescence characteristics (I₀/I) in a range of 0-6 μmol\L with a low limit of detection (LOD) of 0.897 μmol\L. Besides, the CDs-MOF nanocomposite holds good aqueous dispersibility and low cytotoxicity, which shows great potential applications in medical aid including biological detection and clinical diagnosis.     

Molecular fingerprint and machine learning to accelerate design of high-performance homochiral metal–organic frameworks

Computational screening was employed to calculate the enantioseparation capabilities of 45 functionalized homochiral metal–organic frameworks (FHMOFs), and machine learning (ML) and molecular fingerprint (MF) techniques were used to find new FHMOFs with high performance. With increasing temperature, the enantioselectivities for (R,S)-1,3-dimethyl-1,2-propadiene are improved. The “glove effect” in the chiral pockets was proposed to explain the correlations between the steric effect of functional groups and performance of FHMOFs. Moreover, the neighborhood component analysis and RDKit/MACCS MFs show the highest predictive effect on enantioselectivities among the four ML classification algorithms with nine MFs that were tested. Based on the importance of MF, 85 new FHMOFs were designed, and a newly designed FHMOF, NO₂-NHOH-FHMOF, with high similarity to the optimal MFs achieved improved chiral separation performance, with enantioselectivities of 85%. The design principles and new chiral pockets obtained by ML and MFs could facilitate the development of new materials for chiral separation.     

Scaling analysis of hydrogen flow with carbon dioxide cushion gas in subsurface heterogeneous porous media

Subsurface hydrogen (H₂) storage in geological formations is of growing interest for decarbonization. However, there is a knowledge gap in understanding the multiphase flow involved in this process, which can have a significant impact on the recovery performance of H₂. Therefore, a full-compositional modeling study was conducted to analyze potential issues and to understand the fundamental hydrodynamic mechanisms of H₂ storage. We performed a range of 2D vertical simulations at the decametre scale with a very fine cell size (0.1 m) to observe the detailed flow behaviour of H₂ with carbon dioxide (CO₂) as cushion gas in various flow regimes. Issues such as viscous instability, capillary bypassing, gas trapping and gravity segregation are analysed here. To generalize our calculations, we have validated and applied the scaling theory in the context of subsurface H₂ storage. Since this study is focused on the hydrodynamic behaviour, three dimensionless groups, including aspect factor, capillary/viscous ratio and gravity/viscous ratio were identified to correlate recovery performance between various scales in a fixed heterogeneous system. It was found that H₂ could infiltrate the cushion gas in the proximity of the injectors, meaning that CO₂ is not displaced away from the injectors in a piston-like fashion. As a result, the purity of the back produced H₂ is much degraded, particularly in a viscous-dominated scenario. On the other hand, the injected H₂ mostly accumulates at the top forming a highly restricted mixing zone with CO₂ in the gravity-dominated case. The recovery performance is therefore much improved in this case. Although the gas distribution can be significantly altered by capillary forces leading to bypassed zones, the recovery performance of H₂ is hardly influenced. This is because the back-produced H₂ recovery is not dependent on the sweep efficiency of the gas. H₂ can be back produced following the same paths which were formed during injection.     

Responses to comments on the paper “two-dimensional Sc2C: A reversible and high capacity hydrogen storage material predicted by first-principles calculations”

A recent commentary by Santhosh and Ravindran on our paper (Int. J. Hydrogen Energy 2014, 39:10,606) demonstrated that the interaction between H₂ and MXene (Sc₂C and Ti₂C) phases are not Kubas-type and should be of weak physisorption, and thus made a conclusion that 2D Sc₂C and Ti₂C are not suitable for practical hydrogen storage applications. In this responses, we recalculated hydrogen adsorption on 2D Sc₂C and Ti₂C by using different exchange-correlation functionals. And based on the calculated results, bare MXenes (especially the Ti₂C) are suitable as hydrogen storage materials at temperatures of several tens degrees lower than room temperature. And the hydrogen adsorptions on the MXenes terminated with oxygen group were also investigated. Among the Ti₂C, Sc₂C and their oxygen-functional counterparts, the binding energy of H₂ on Sc₂CO₂ surface is the closest to the ideal range of 0.16–0.42 eV/H₂ at ambient conditions, and thus the Sc₂C with oxygen group is expected to be more suitable as hydrogen storage materials.     

一种分布式对象存储在巡检系统中的研究应用

随着通信技术的快速发展及智能设备的普及,网络规模、网元数量的不断增长,通信设备巡检任务成倍增加,给巡检系统的存储带来了巨大挑战,传统文件存储方式已无法满足要求。通过研究分布式对象存储,基于MinIO设计实现了一种新的巡检系统存储方案,可有效提升系统存储效率和可扩展性。该方案已经在江苏移动实际应用,支撑常态化巡检工作,对于网络运维及管理有较好的参考意义。     

Stable power supply of an independent power source for a remote island using a Hybrid Energy Storage System composed of electric and hydrogen energy storage systems

We propose a self-sustaining power supply system consisting of a “Hybrid Energy Storage System (HESS)” and renewable energy sources to ensure a stable supply of high-quality power in remote islands. The configuration of the self-sustaining power supply system that can utilize renewable energy sources effectively on remote islands where the installation area is limited is investigated. It is found that it is important to select renewable energy sources whose output power curve is close to the load curve to improve the efficiency of the system. The operation methods that can increase the cost-effectiveness of the self-sustaining power supply system are also investigated. It is clarified that it is important for increasing the cost effectiveness of the self-sustaining power supply system to operate the HESS with a smaller capacity of its components by setting upper limits on the output power of the renewable energy sources and cutting the infrequent generated power.