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.     

Effect of wind condition on unintended hydrogen release in a hydrogen refueling station

Ambient condition, especially the wind condition, is an important factor to determine the behavior of hydrogen diffusion during hydrogen release. However, only few studies aim at the quantitative study of the hydrogen diffusion in a wind-exist condition. And very little researches aiming at the variable wind condition have been done. In this paper, the hydrogen diffusion in different wind condition which including the constant wind velocity and the variable wind velocity is investigated numerically. When considering the variable wind velocity, the UDF (user defined function) is compiled. Characteristics of the FGC (flammable gas cloud) and the HMF (hydrogen mass fraction) are analyzed in different wind condition and comparisons are made with the no-wind condition. Results indicate that the constant wind velocity and the variable wind velocity have totally different effect for the determination of hydrogen diffusion. Comparisons between the constant wind velocity and the variable wind velocity indicate that the variable wind velocity may cause a more dangerous situation since there has a larger FGC volume. More importantly, the wind condition has a non-negligible effect when considering the HMF along the radial direction. As the wind velocity increases, the distribution of the HMF along the radial direction is not Gaussian anymore when the distance between the release hole and the observation line exceeds to a critical value. This work can be a supplement of the research on the hydrogen release and diffusion and a valuable reference for the researchers.     

宰前倒挂应激对鸭血凝胶特性的影响

宰前倒挂因能有效提高屠宰效率成为肉鸭宰杀前必经的一个工艺过程，但倒挂往往会加剧肉鸭的应激程度，影响其血液品质，已成为工厂生产中亟待解决的问题。因此，本实验旨在借助血液学与凝血相关指标探究宰前倒挂应激对鸭血凝胶特性的影响。在屠宰前，将40 只樱桃谷鸭随机分为2 个处理组：对照组和宰前倒挂组（宰前倒挂2.5 min），屠宰后采集血样并制备血凝块，随后分析血液指标（应激指标、血液学指标、凝血指标）以及鸭血凝胶特性。结果表明，宰前倒挂组鸭血中皮质酮激素及促肾上腺皮质激素的水平均高于对照组，说明实验所建立宰前倒挂模型成立。倒挂应激后，红细胞计数、血小板计数、血红蛋白质量浓度增加，红细胞比容、平均红细胞体积及平均血小板体积增大；凝血酶原的水平极显著上升（P＜0.01），组织因子的水平显著上升（P＜0.05）。宰前倒挂最终导致鸭血凝胶化加快，鸭血凝胶体系中水分迁移速率增大，结合水与自由水比例减少，不易流动水比例增大，质构特性和保水性变差。研究揭示了宰前倒挂应激通过影响鸭血的血液学指标和凝血系统从而改变其凝胶行为及凝胶特性，可为工厂改善应激对鸭血凝胶品质的影响提供理论依据。     

Combination of vegetable soup and glucan demonstrates synergistic effects on macrophage-mediated immune responses

Vegetable soup (VS), a plant-based functional food, has been used as a traditional folk medicine and is attracting attention for its ability to enhance the immune response. β-Glucan, a well-established and effective immunomodulator, has synergistic effects when used in combination with some bioactive compounds. In the present study, we aimed to evaluate the synergistic immunomodulatory effects of the combination of VS and β-glucan on macrophage-mediated immune responses. β-Glucan was demonstrated to synergistically enhance the VS-stimulated immune response, including the production of interleukin-6, tumor necrosis factor-α, and nitric oxide, mainly through the mitogen-activated protein kinase pathway in macrophages. In addition, this combination has the potential for further development in functional foods with immune-enhancing activity.Supplementary InformationThe online version contains supplementary material available at 10.1007/s10068-021-00888-x.     

Modeling unaccounted-for gas among residential natural gas consumers using a comprehensive fuzzy cognitive map

Residential natural gas consumption depends on several factors. Available tools and methods to identify, categorize, and validate effective factors have some limitations, making consumption modeling more complex. Once a comprehensive model of effective consumption factors is developed for residential gas consumers, it can predict consumption. In addition, such a model could be used to verify the accuracy of measuring devices in order to reduce unaccounted for gas (UFG). The key factors affecting residential gas consumption were identified based on previous studies and their mutual effects were analyzed using a fuzzy cognitive mapping (FCM) method. The most significant factors and their effects on natural gas consumption in the residential sector were determined. In this study, for the first time, the expected consumption for each consumer was estimated using a consumption index. Generally, if the estimated consumption is significantly different from the amount recorded by the meter, it could suggest a potential source of UFG. The proposed method was applied to the data collected from the residential gas consumers of a small region in Iran (Dasht-e Arjan region, Fars province), and the results demonstrate the effectiveness of the proposed method.     

H_2S制硫酸装置外部安全防护距离设计

以某酸性气制硫酸装置酸性气缓冲罐为例,进行外部安全防护距离设计,结合当地多种气象,使用PHAST软件和LEAK软件进行H_2S气体扩散和爆炸计算,扩散结果应用于周边人员集中场所的防毒设计以及企业的应急救援,H_2S爆炸计算结果应用于周边人员集中场所建构筑物的抗爆设计,并和《石油化工工厂布置设计规范》(GB 50984-2014)中的建议值进行比对。