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1.
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   
2.
向森 《电子测试》2021,(6):125-126
电路板在我们的日常生活中非常常见,这就使得印刷电路板的缺陷检测显得尤为重要。AOI作为新兴的检测PCB板缺陷的系统,在生产实际中正在被大家熟知并且应用。相较于传统的检测方式,AOI系统比较灵活,无论是在检测时间还是系统运算上,或者是对相关技术人员的要求相较于传统方式都比较有优势,本文就AOI系统在实际中的应用展开讨论,分析并且介绍了在实际应用中的具体细则。  相似文献   
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4.
A novel TiO2 thin film was prepared on the ceramic hollow fiber by the sol-gel method using poly(vinylpyrrolidone) (PVP) and polyvinyl alcohol (PVA) as additives. SEM images verified the formation of TiO2 layer with various thickness using different composition of titania sols. The effect of the PVP and PVA contents on the TiO2 sol properties, the separation and the antifouling performance of the ultrafiltration membranes were investigated thoroughly. When the contents of PVP and PVA were 1.0 wt% and 0.8 wt%, respectively, the resultant membrane showed a thickness of 0.55 μm with a pure water flux of 255 L m?2 h?1. In addition, the adherent foulant bovine serum albumin was applied to evaluate the antifouling performance. During the three fouling-recovery cycles, the flux recovery ratio and the flux decay ratio maintained about 99% and 30%. The BSA flux and rejection were still 169 L m?2 h?1 and 96.9% after the cycles, indicating a superior antifouling property.  相似文献   
5.
杨立宁  郑东昊  王立新  杨光 《化工进展》2022,41(11):5961-5967
以具有轻质高强优异性能的蜻蜓翅脉结构为设计灵感,在分析翅脉网格结构抗冲击原理的基础上,设计了传统和仿生两类对比结构。采用熔融挤出3D打印机成功制备了具有不同结构的连续碳纤维增强聚乳酸复合材料试样,并对不同结构复合材料试样的拉伸性能和抗冲击性能进行了测试和对比分析。研究分析结果表明:由于拉伸力方向上的连续碳纤维含量相对较少,限制了仿生结构复合材料抗拉强度的提高,但仿生结构的平均抗拉强度为传统结构的1.18倍;当仿生结构复合材料试样受到冲击力时,其内部六边形结构的连接角度会发生变化,从而极大消耗冲击能量,同时具有六边形网格结构的连续碳纤维可以有效阻碍裂纹的扩展,因此仿生结构的平均冲击韧性可以达到传统结构的2.46倍;仿生蜻蜓翅脉结构可以显著提高增材制造复合材料的综合力学性能,且对于抗冲击性能的提高具体突出效果。连续碳纤维增强树脂基复合材料的有效可行的仿生蜻蜓翅脉结构设计和增材制造,可极大扩展其在高冲击载荷领域中的相应应用。  相似文献   
6.
Borazine rings act as a pivotal part in siliconboroncarbonitride ceramics (SiBCN) for high-temperature stability and great resistance to crystallization. A detailed investigation of the ring formation mechanism will guide the design and synthesis of SiBCN to meet application requirements under extreme conditions. Boron trichloride (BCl3) and hexamethyldisilazane (HN(SiMe3)2) are common raw materials for the synthesis of precursors for SiBCN. In this paper, quantum chemical calculation was used to study the cyclization reaction mechanism between BCl3 and HN(SiMe3)2 to form trichloroborazine (TCBZ) at the MP2/6-31G (d,p) level of theory. We discussed the structure properties, reaction pathways, energy barriers, reaction rates, and other aspects in detail. The results show that BCl3 and HN(SiMe3)2 alternately participate in the reaction process, accompanied by the release of trimethylchlorosilane (TMCS), and that the entire reaction shows an absolute advantage in terms of energy. In the Step by step reaction, lower reaction barriers are formed due to the introduction of BCl3 with more heat released compared to that for the introduction of HN(SiMe3)2. The final single-molecule cyclization and TMCS elimination steps are found to be faster compared to all previous bimolecular reactions.  相似文献   
7.
Abstract

Different drying methods (spray drying (SD), vacuum drying (VD), microwave vacuum drying (MVD), and infrared vacuum drying (IFVD)) were applied in order to compare the hygroscopicity behavior of chicken powders. The hygroscopicity curves and glass transition temperature were used to evaluate the influence of ambient humidity and temperature on moisture absorption of powders. The results showed that the chicken powder dried by MVD had the lowest moisture absorption, followed by IFVD, VD, and SD. The hygroscopicity of SD chicken powders was different from other three kinds of chicken powders due to the physical properties of particles and the changes of protein secondary structure as detected by the Fourier transform-infrared spectrometer. For the three vacuum drying methods, the difference of protein secondary structure was the main reason of differences in hygroscopicity. Although MVD chicken powders were slightly inferior to SD chicken powders in taste, MVD chicken powders were the best in terms of smell and color as suggested by instrumental sensory parameter evaluations. It was found that MVD had a positive effect on reducing moisture absorption and maintaining sensory quality of chicken powders.  相似文献   
8.
The bromodomain and extra terminal (BET) family of bromodomain-containing proteins (BCPs) have been the subject of extensive research over the past decade, resulting in a plethora of high-quality chemical probes for their tandem bromodomains. In turn, these chemical probes have helped reveal the profound biological role of the BET bromodomains and their role in disease, ultimately leading to a number of molecules in active clinical development. However, the BET subfamily represents just 8/61 of the known human bromodomains, and attention has now expanded to the biological role of the remaining 53 non-BET bromodomains. Rapid growth of this research area has been accompanied by a greater understanding of the requirements for an effective bromodomain chemical probe and has led to a number of new non-BET bromodomain chemical probes being developed. Advances since December 2015 are discussed, highlighting the strengths/caveats of each molecule, and the value they add toward validating the non-BET bromodomains as tractable therapeutic targets.  相似文献   
9.
封隔器在石油天然气开采中起着非常重要的作用,而扩张式封隔器在裸眼井中广泛应用。本文采用有限元软件建立了裸眼封隔器与地层的模型,对胶筒在坐封过程中与井壁接触应力的变化进行了研究,并研究了在不同摩擦系数下接触压力的变化,结果表明,建立粗糙井壁面能够更加符合实际情况,胶筒肩部为应力集中的区域,地层与胶筒的接触应力会随着摩擦系数的增加而减小。研究结果为裸眼扩张式封隔器的设计和改进提供了理论依据。  相似文献   
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