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91.
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.  相似文献   
92.
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.  相似文献   
93.
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。  相似文献   
94.
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.  相似文献   
95.
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 PM2.5 in buildings. This study used an algorithm to automatically estimate the long-term time-resolved indoor PM2.5 of outdoor and indoor origin in real apartments with natural ventilation. The inputs for the algorithm were only the time-resolved indoor/outdoor PM2.5 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 PM2.5 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 PM2.5 monitoring using low-cost light-scattering sensors.  相似文献   
96.
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.  相似文献   
97.
Due to environmental and health aspects, aqueous ceramic slurries are preferred to traditional organic solvent systems in tape casting. An important obstacle associated with the high surface energy of water is poor wetting of aqueous ceramic slurries on polymeric tape carriers. Therefore, we measured the contact angles of an aqueous epoxy-based ceramic slurry on polyethylene terephtalate (PET), polypropylene (PP), polymethyl methacrylate (PMMA), and aluminium-coated polyethylene terephtalate (PET-Al) films and investigated approaches to improving their wetting. We evaluated the effect of plasma treatment of the tape carrier surface on the wetting behaviour and compared it with the effect of adding non-ionic amphiphilic surfactants to the ceramic slurry. The treatment of the tape carrier surface by low-temperature plasma substantially improved the wetting behaviour of aqueous ceramic slurry. The lowest contact angle of 31° was obtained on the PET film. Although the addition of non-ionic surfactants improved both the wetting behaviour of the slurry and the detachment of the polymeric carrier from the ceramic tape even better than the plasma treatment of the carrier surface did, the plasma-treated carriers still present a useful alternative to the surfactants. In the case of the plasma-treated PET carrier the surfactants could be fully eliminated and potential drawbacks related to the use of surfactants could be prevented.  相似文献   
98.
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.  相似文献   
99.
This study investigated the antifungal activity and the potential antifungal mechanisms of 2-hydroxy-4-methoxybenzaldehyde (HMB) against Aspergillus flavus (A. flavus). The minimum inhibitory concentration (MIC) of HMB in preventing spore germination was 70 μg mL−1. HMB at MIC disrupted cell wall integrity by reducing the number of septa by 86.66% (P < 0.05) in mycelia and increased cell membrane permeability by about 14-fold (P < 0.05) evidenced by propidium iodide (PI) staining. Furthermore, HMB at MIC inhibited respiration by 33.33%. These results revealed that the antifungal activity of HMB against A. flavus could be attributed to the damaged cell wall integrity, cell membrane permeability and respiration metabolism. What’s more, A. flavus was completely restrained in corn kernels due to HMB. Therefore, HMB could be applied as an effective antifungal agent.  相似文献   
100.
CsPbX3 (X = Cl, Br, I) perovskite quantum dots (QDs) represent bright and tunable photoluminescence, it is regrettable that the air instability and poor water resistant properties prevent their application in optoelectronic devices. At the same time, the toxicity of lead is also a major factor restricting its development. As a consequence, we demonstrate the partial replacement of Pb with Mn through conventional melt-quenching and heat-treatment method preparation of Mn-doped CsPb(Cl/Br)3 QD glass. Mn-doped CsPb(Cl/Br)3 QD glass exhibits high luminescent intensity like QDs. It is important that Mn-doped CsPb(Cl/Br)3 QD glass with Dual-Color maintained the same lattice structure like Mn-doped CsPb(Cl/Br)3 QDs, and highly homogeneous spectral characteristics of Mn luminescence. The intensity and position of this Mn-related emission are also tunable by altering the experimental parameters, such as the Pb-to-Mn feed ratio, annealing temperature. More importantly, the as-prepared orange Mn-doped CsPb(Cl/Br)3 QD glass was employed to fabricate white LEDs combined with a commercial Ce3+:Y3Al5O12 phosphor-in-glass (Ce-PiG) on top of a InGaN blue chip. And the constructed WLEDs generate a warm white with an optimal luminous efficacy (LE) of 67.00 lm/W, a high CRI of 81.4, and a low CCT of 4902 K.  相似文献   
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