Role of the HSP70 Co-Chaperone SIL1 in Health and Disease

Cell surface and secreted proteins provide essential functions for multicellular life. They enter the endoplasmic reticulum (ER) lumen co-translationally, where they mature and fold into their complex three-dimensional structures. The ER is populated with a host of molecular chaperones, associated co-factors, and enzymes that assist and stabilize folded states. Together, they ensure that nascent proteins mature properly or, if this process fails, target them for degradation. BiP, the ER HSP70 chaperone, interacts with unfolded client proteins in a nucleotide-dependent manner, which is tightly regulated by eight DnaJ-type proteins and two nucleotide exchange factors (NEFs), SIL1 and GRP170. Loss of SIL1′s function is the leading cause of Marinesco-Sjögren syndrome (MSS), an autosomal recessive, multisystem disorder. The development of animal models has provided insights into SIL1′s functions and MSS-associated pathologies. This review provides an in-depth update on the current understanding of the molecular mechanisms underlying SIL1′s NEF activity and its role in maintaining ER homeostasis and normal physiology. A precise understanding of the underlying molecular mechanisms associated with the loss of SIL1 may allow for the development of new pharmacological approaches to treat MSS.     

Benchmark experiment of large-angle scattering reaction cross section of iron at 14 MeV using two shadow bars – Comparison of experimental results with ENDF/B-VIII –

ABSTRACT

It is important to perform neutron transport simulations with accurate nuclear data in the neutronics design of a fusion reactor. However, absolute values of large-angle scattering cross sections vary among nuclear data libraries even for well-examined nuclide of iron. Benchmark experiments focusing on large-angle scattering cross sections were thus performed to confirm the correctness of nuclear data libraries. The series benchmark experiments were performed at a DT neutron source facility, OKTAVIAN of Osaka University, Japan, by the unique experimental system established by the authors’ group, which can extract only the contribution of large-angle scattering reactions. This system consists of two shadow bars, target plate (iron), and neutron detector (niobium). Two types of shadow bars were used and four irradiations were conducted for one experiment, so that contribution of room-return neutrons was effectively removed and only large-angle scattering neutrons were extracted from the measured four Nb reaction rates. The obtained experimental results were compared with calculations for five nuclear data libraries including JENDL-4.0, JEFF.-3.3, FENDL-3.1, ENDF/B- VII, and recently released ENDF/B-VIII. It was found from the comparison that ENDF/B-VIII showed the best result, though ENDF/B-VII showed overestimation and others are in large underestimation at 14 MeV.     

基于WebGL的3D立库可视化系统设计与实现

针对当前扩大化和普及化的仓储物流,提出了一种3D自动化立体仓库可视化系统的实现方案,用以解决仓储系统中遇到的数据不直观的问题。该系统采用JavaScript编程语言,以Three.js引擎为基础,构建了基于WebGL的三维自动化立体仓库的可视化系统,该系统能有效直观的进行仓储管理,适合现代化仓储企业的管理需求。     

Chicken nuggets-like core/shell nanosheet arrays as high performance flexible all-solid-state supercapacitor cathode and buckwheat shell as anode

The energy density of a flexible all-solid-state supercapacitor (ASC) requires new electrode material with special structure and morphology as a prerequisite for its secured improvement. In this paper, a new morphological exploration of chicken nuggets-like core/shell NiCo₂O₄/MnO₂ (NCM) nanosheet arrays on Ni foam was employed. The application of this special morphology aims to greatly improve the electrochemical performance of the cathode electrode. Additionally, Buckwheat Biochar (BBC) is utilized as the anode while the PVA/KOH thin film is prepared as the separator. The chicken nuggets-like core/shell NCM nanosheet arrays were obtained by a two-step hydrothermal method. A series of characterization methods were carried out to further support the core/shell's well-designed structure and precise composition. The tests exhibited excellent specific capacitance of 593.3 F g^?1 at 5 mA cm^?2 and outstanding cycling stability with a retention of 90% after 10000 cycles. Furthermore, the assembled NCM//BBC ASC device indicated a high specific capacitance (239 F g^?1 at the current density of 5 mA cm^?2), this is in due part of the unique architecture of NCM nanosheet arrays and interconnected special porous structure of the BBC and the thin film PVA/KOH. Hence, the assembled ASC device exhibited high energy density (an energy density of 58 Wh·kg^?1 at 3263 W kg^?1) and remarkable cycling stability.     

Progressive failure analysis of notched composite plate by utilizing macro mechanics approach

In this study, gradual and sudden reduction methods were combined to simulate a progressive failure in notched composite plates using a macro mechanics approach. Using the presented method, a progressive failure is simulated based on a linear softening law prior to a catastrophic failure, and thereafter, sudden reduction methods are employed for modeling a progressive failure. This combination method significantly reduces the computational cost and is also capable of simultaneously predicting the first and last ply failures (LPFs) in composite plates. The proposed method is intended to predict the first ply failure (FPF), LPF, and dominant failure modes of carbon/epoxy and glass/epoxy notched composite plates. In addition, the effects of mechanical properties and different stacking sequences on the propagation of damage in notched composite plates were studied. The results of the presented method were compared with experimental data previously reported in the literature. By comparing the numerical and experimental data, it is revealed that the proposed method can accurately simulate the failure propagation in notched composite plates at a low computational cost.     

Estimating long-term time-resolved indoor PM2.5 of outdoor and indoor origin using easily obtainable inputs

To evaluate the separate impacts on human health and establish effective control strategies, it is crucial to estimate the contribution of outdoor infiltration and indoor emission to indoor PM_2.5 in buildings. This study used an algorithm to automatically estimate the long-term time-resolved indoor PM_2.5 of outdoor and indoor origin in real apartments with natural ventilation. The inputs for the algorithm were only the time-resolved indoor/outdoor PM_2.5 concentrations and occupants’ window actions, which were easily obtained from the low-cost sensors. This study first applied the algorithm in an apartment in Tianjin, China. The indoor/outdoor contribution to the gross indoor exposure and time-resolved infiltration factor were automatically estimated using the algorithm. The influence of outdoor PM_2.5 data source and algorithm parameters on the estimated results was analyzed. The algorithm was then applied in four other apartments located in Chongqing, Shenyang, Xi'an, and Urumqi to further demonstrate its feasibility. The results provided indirect evidence, such as the plausible explanations for seasonal and spatial variation, to partially support the success of the algorithm used in real apartments. Through the analysis, this study also identified several further development directions to facilitate the practical applications of the algorithm, such as robust long-term outdoor PM_2.5 monitoring using low-cost light-scattering sensors.     

Development of a H2-permeable Pd60Cu40-based composite membrane using a reverse build-up method

A new reverse build-up method is developed to fabricate an economical H₂-permeable composite membrane. Sputtering and electroplating are used for the formation of a membrane comprised of a 3.7-μm-thick Pd₆₀Cu₄₀ (wt.%) alloy layer and a 13-μm-thick porous Ni support layer, respectively. The H₂-permeation measurements are performed under the flow of a gaseous mixture of H₂ and He at 300–320 °C and 50–100 kPa of H₂ partial pressure. The H₂/He selectivity values exceed 300. The activation energy at 300–320 °C is 10.9 kJ mol⁻¹. The H₂ permeability of the membrane is 1.25 × 10⁻⁸ mol m⁻¹ s⁻¹ Pa^−0.5 at 320 °C after 448 h. The estimated Pd cost of the proposed membrane is approximately 1/8 of the cost for a pure Pd₆₀Cu₄₀ membrane. This study demonstrates that the proposed method allows the facile production of low-cost, Pd-based membranes for H₂ separation.     

Effect of Newtonian heating/cooling on free convection in an annular permeable region in the presence of heat source/sink

The influence of Newtonian heating/cooling in the presence of heat source/sink has been investigated on laminar free convective flow in a vertical annular permeable region. The mathematical model for the problem has been considered as a boundary value problem consisting of two simultaneous ordinary differential equations. The boundary value problem has been transformed to nondimensional form. This has given rise to a number of parameters representing both geometrical and physical features of the problem. Closed‐form analytical solutions of the governing equations have been obtained for two different cases of internal heat generation/absorption. To assess the effects of governing parameters on the fluid velocity and temperature, a number of profiles of these field variables have been presented. The efficacy of the distinct processes on the field variables has been discussed extensively. The main outcome obtained in this study is that the velocity as well as temperature is enhanced in the case of the Newtonian heating while the opposite behavior occurs in the Newtonian cooling for both cases of source and sink. Furthermore, the influence of the governing parameters has been shown on the skin friction, volume flow rate, and the Nusselt number.     

High strain rate compressive response of the Cf/SiC composite

Carbon fiber reinforced ceramic owns the properties of lightweight, high fracture toughness, excellent shock resistance, and thus overcomes ceramic's brittleness. The researches on the advanced structure of astronautics, marine have exclusively evaluated the quasi-static mechanical response of carbon fiber reinforced ceramics, while few investigations are available in the open literature regarding elastodynamics. This paper reports the dynamic compressive responses of a carbon fiber reinforced silicon carbide (C_f/SiC) composite (CFCMC) tested by the material test system 801 machine (MTS) and the split Hopkinson pressure bar (SHPB). These tests were to determine the rate dependent compression response and high strain rate failure mechanism of the C_f/SiC composite in in-plane and out-plane directions. The in-plane compressive strain rates are from 0.001 to 2200?s^?1, and that of the out-plane direction are from 0.001 to 2400?s^?1. The compressive stress-strain curves show the C_f/SiC composite has a property of strain rate sensitivity in both directions while under high strain rate loadings. Its compressive stiffness, compressive stress, and corresponding strain are also strain rate sensitive. The compressive damage morphologies after high strain rate impacting show different failure modes for each loading direction. This study provides knowledge about elastodynamics of fiber-reinforced ceramics and extends their design criterion with a reliable evaluation while applying in the scenario of loading high strain rate.     

Competitive growth of Al2O3/YAG/ZrO2 eutectic ceramics during directional solidification: Effect of interfacial energy

The microstructure evolution and growth behavior of the Al₂O₃/Y₃Al₅O₁₂(YAG)/ZrO₂ ternary eutectic ceramics during directional solidification were well investigated. During directional solidification of the Al₂O₃/YAG/ZrO₂ ternary eutectic ceramics, {} Al₂O₃ paralleled with {001}ZrO₂ while they did not parallel with {001}YAG at the same time in the competitive growth stage. All of the interfaces parallel to each other finally. The area percentage of the Al₂O₃/ZrO₂ and YAG/ZrO₂ interfaces are 40.4 ± 0.2% and 30.8 ± 0.1%, respectively, higher than that of the Al₂O₃/YAG (28.8 ± 0.2%). The content of Al₂O₃ and YAG phases are 39.9% and 41.1%, respectively, almost double of that of ZrO₂. The interfaces of Al₂O₃/ZrO₂ and YAG/ZrO₂ are shorter and more dispersed than that of the Al₂O₃/YAG. It was found that the interfacial energy of Al₂O₃/ZrO₂ and YAG/ZrO₂ interfaces are lower than that of Al₂O₃/YAG. It can be concluded that interfacial energy plays a decisive role in affecting the crystallographic orientation and interfaces distribution in the Al₂O₃/YAG/ZrO₂ eutectic since the interfaces of Al₂O₃/ZrO₂ and YAG/ZrO₂ with lower interfacial energy can be formed more easily during directional solidification. Therefore, the contents of Al₂O₃/ZrO₂ and YAG/ZrO₂ interfaces are higher. This study can provide theoretical guidance for interface design of multi-phase materials.