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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.
当前,我国水泥工业在可燃废弃物应用技术方面都还处于一家一户、自制自用、效率极低的初级阶段。发达国家的替代燃料:“垃圾衍生燃料”RDF、“固体回收燃料”SRF、“次煤”Subcoal和“纸塑垃圾衍生燃料”RPF制成的原材料都是可燃废弃物,只是处理工艺技术不同或者由垃圾中分拣出的可燃废弃物不同,制成颗粒状衍生燃料的品质不同,这些都可以替代部分甚或替代全部化石燃料在水泥窑炉中应用。我国大力发展“替代燃料”产业,有助于水泥工业消纳更多的“可燃废弃物”,为改善环境尤其是城镇环境和面貌,为我国的节能减排和绿色高质量发展发挥更大的作用。 相似文献
3.
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
4.
AbstractDifferent 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
5.
一场新型冠状病毒肺炎疫情的爆发给染料行业的发展带来了影响,既有负面消极,也有积极影响。疫情将促进染料行业绿色发展,推动染料行业走向信息化、智能化时代,走向高质量发展,指出只要坚持绿色、创新和智能化,就能战胜疫情,推动中国染料行业稳健发展。 相似文献
6.
Michael A. Clegg Prof. Nicholas C. O. Tomkinson Dr. Rab K. Prinjha Dr. Philip G. Humphreys 《ChemMedChem》2019,14(4):362-385
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
7.
Gerd J. Hahn 《国际生产研究杂志》2020,58(5):1425-1441
The Fourth Industrial Revolution – also known as Industry 4.0 (i4.0) – comprises the digitalisation of the industrial sector. This paper uses the theoretical lens of supply chain innovation (SCI) to investigate the implications of i4.0 on supply chain management. For these purposes, the method of structured content analysis is applied to more than 200 use cases of i4.0-enabled SCI introduced by both established and startup companies. i4.0-enabled SCI manifests along three dimensions: process, technology, and business architecture. The key findings of this study can be summarised as follows: first, i4.0-enabled SCI extends the initial focus on productivity improvements in SC processes towards scalability and flexibility. Second, extant i4.0 solutions rely mostly on analytics and smart things while omitting smart people technology and the human-centric approach associated with the i4.0 paradigm. Third, established companies adopt i4.0 merely to sustain their existing business architectures while startup companies radically change their operating models, relying heavily on data analytics and the platform economy. Consequently, established companies pursue a problem-driven, engineering-based approach to SCI while startup companies follow an ‘asset-light’, business-driven approach. Lastly, there are two distinct approaches to digitalising operational SC processes: platform-based crowdsourcing of standard processes and on-demand provision of customised services. 相似文献
8.
Hot-dip galvanizing is a standard technology to produce coated steel strips. The primary objective of the galvanizing process is to establish a homogeneous zinc layer with a defined thickness. One condition to achieve this objective is a uniform transverse distance between the strip and the gas wiping dies, which blow off excessive liquid zinc. Therefore, a flat strip profile at the gas wiping dies is required. However, strips processed in such plants often exhibit residual curvatures which entail unknown flatness defects of the strip. Such flatness defects cause non-uniform air gaps and hence an inhomogeneous zinc coating thickness. Modern hot-dip galvanizing lines often use electromagnets to control the transverse strip profile near the gas wiping dies. Typically, the control algorithms ensure a flat strip profile at the electromagnets because the sensors for the transverse strip displacement are also located at this position and it is unfeasible to mount displacement sensors directly at the gas wiping dies. This brings along that in general a flatness defect remains at the gas wiping dies, which in turn entails a suboptimal coating.In this paper, a model-based method for a feedforward control of the strip profile at the position of the gas wiping dies is developed. This method is based on a plate model of the axially moving strip that takes into account the flatness defects in the strip. First, an estimator of the flatness defects is developed and validated for various test strips and settings of the plant. Using the validated mathematical model, a simulation study is performed to compare the state-of-the-art control approach (flat strip profile at the electromagnets) with the optimization-based feedforward controller (flat strip profile at the gas wiping dies) proposed in this paper. Moreover, the influence of the distance between the gas wiping dies and the electromagnets is investigated in detail. 相似文献
9.
10.
The development of a sustainable energy system throughout an enterprise is a complex task, which requires an agile holistic approach. Such an approach needs to include a variety of objectives including energy strategy formation and strategic decision-making, which are directly related to the analysis and management of the main areas of sustainable development:The economic, technological, environmental, and social. These multidimensional requirements of sustainability are often difficult to achieve within the enterprise, because these aspects are interrelated and influenced by various internal and external environment factors. This paper first reviews the main challenges for an energy system, and then demonstrates how a strategic agile enterprise architecture driven approach could effectively guide the sustainable energy system development. The study presented in this paper provides a holistic approach that contributes to the advancement and usage of literature dealing with issues of sustainable energy system development and agile enterprise architecture, which has not been discussed before to any great extent. 相似文献