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.     

Direct View on the Origin of High Li+ Transfer Impedance in All-Solid-State Battery

Large interfacial resistance plays a dominant role in the performance of all-solid-state lithium-ion batteries. However, the mechanism of interfacial resistance has been under debate. Here, the Li⁺ transport at the interfacial region is investigated to reveal the origin of the high Li⁺ transfer impedance in a LiCoO₂(LCO)/LiPON/Pt all-solid-state battery. Both an unexpected nanocrystalline layer and a structurally disordered transition layer are discovered to be inherent to the LCO/LiPON interface. Under electrochemical conditions, the nanocrystalline layer with insufficient electrochemical stability leads to the introduction of voids during electrochemical cycles, which is the origin of the high Li⁺ transfer impedance at solid electrolyte-electrode interfaces. In addition, at relatively low temperatures, the oxygen vacancies migration in the transition layer results in the formation of Co₃O₄ nanocrystalline layer with nanovoids, which contributes to the high Li⁺ transfer impedance. This work sheds light on the mechanism for the high interfacial resistance and promotes overcoming the interfacial issues in all-solid-state batteries.     

Lithiophilic MXene-Guided Lithium Metal Nucleation and Growth Behavior

The positive effects of a lithiophilic substrate on the electrochemical performance of lithium metal anodes are confirmed in several reports, while the understanding of lithiophilic substrate-guided lithium metal nucleation and growth behavior is still insufficient. In this study, the effect of a lithiophilic surface on lithium metal nucleation and growth behaviors is investigated using a large-area Ti₃C₂T_x MXene substrate with a large number of oxygen and fluorine dual heteroatoms. The use of the MXene substrate results in a high lithium-ion concentration as well as the formation of uniform solid–electrolyte-interface (SEI) layers on the lithiophilic surface. The solid–solid interface (MXene-SEI layer) significantly affects the surface tension of the deposited lithium metal nuclei as well as the nucleation overpotential, resulting in the formation of uniformly dispersed lithium nanoparticles ( ≈ 10–20 nm in diameter) over the entire MXene surface. The primary lithium nanoparticles preferentially coalesce and agglomerate into larger secondary particles while retaining their primary particle shapes. Subsequently, they form close-packed structures, resulting in a dense metal layer composed of particle-by-particle microstructures. This distinctive lithium metal deposition behavior leads to highly reversible cycling performance with high Columbic efficiencies >  99.0% and long cycle lives of over 1000 cycles.     

Nitrate Additives Coordinated with Crown Ether Stabilize Lithium Metal Anodes in Carbonate Electrolyte

Lithium metal anodes (LMAs) are promising for next-generation batteries but have poor compatibility with the widely used carbonate-based electrolytes, which is a major reason for their severe dendrite growth and low Coulombic efficiency (CE). A nitrate additive to the electrolyte is an effective solution, but its low solubility in carbonates is a problem that can be solved using a crown ether, as reported. A rubidium nitrate additive coordinated with 18-crown-6 crown ether stabilizes the LMA in a carbonate electrolyte. The coordination promotes the dissolution of NO₃⁻ ions and helps form a dense solid electrolyte interface that is Li₃N-rich which guides uniform Li deposition. In addition, the Rb (18-crown-6)⁺ complexes are adsorbed on the dendrite tips, shielding them from Li deposition on the dendrite tips. A high CE of 97.1% is achieved with a capacity of 1 mAh cm⁻² in a half cell, much higher than when using the additive-free electrolyte (92.2%). Such an additive is very compatible with a nickel-rich ternary cathode at a high voltage, and the assembled full battery with a cathode material loading up to 10 mg cm⁻² shows an average CE of 99.8% over 200 cycles, indicating a potential for practical use.     

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.     

Novel branched sulfonated polyimide membrane with remarkable vanadium permeability resistance and proton selectivity for vanadium redox flow battery application

A series of novel branched sulfonated polyimide (bSPI-x) membranes with 8% branched degree are developed for application in vanadium redox flow battery (VRFB). The sulfonation degrees of bSPI-x membranes are precisely regulated for obtaining excellent comprehensive performance. Among all bSPI-x membranes, the bSPI-50 membrane shows strong vanadium permeability resistance, which is as 8 times as that of commercial Nafion 212 membrane. At the same time, the bSPI-50 membrane has remarkable proton selectivity, which is four times as high as that of Nafion 212 membrane. The bSPI-50 membrane possesses slower self-discharge speed than Nafion 212 membrane. Furthermore, the bSPI-50 membrane achieves stable VRFB efficiencies during 200-time charge-discharge cycles at 120–180 mA cm^?2. Simultaneously, the bSPI-50 membrane exhibits excellent capacity retention compared with Nafion 212 membrane. All results imply that the bSPI-50 membrane possesses good application prospect as a promising alternative separator of VRFB.