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41.
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
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Tianxing Chen Tengteng Yao Hui Peng Andrew K. Whittaker Yao Li Shenmin Zhu Zhaoyang Wang 《Advanced functional materials》2021,31(45):2106079
The convenience of injectable hydrogels that can provide high loading of diverse phototherapy agents and further long-time retention at the tumor site has attracted tremendous interest in simultaneous photothermal and photodynamic cancer therapies. However, to incorporate the phototherapy agents into hydrogels, complex modifications are generally unavoidable. Moreover, these phototherapy agents usually suffer from low efficiency and work at different irradiation wavelengths outside the near infrared windows. Hence, a method for the fabrication of an injectable hydrogel for simultaneous photothermal therapy and photodynamic therapy, through the Schiff-base reaction between amido modified carbon dots (NCDs) and aldehyde modified cellulose nanocrystals is proposed. The NCDs act as both phototherapy agents and crosslinkers to form hydrogels. Significantly, the NCDs demonstrate an extremely high photothermal conversion efficiency of 77.6% which is among the highest levels for photothermal agents and a high singlet quantum yield of 0.37 under a single 660 nm light-emitting diode irradiation. The hydrogels are examined through in vitro and in vivo animal experiments which show nontoxic and effectively tumor inhibition. Thus, the strategy of direct reaction of phototherapy agents and the matrix not only provides new strategies for injectable hydrogel fabrication but paves a new road for advanced tumor treatment. 相似文献
44.
采用粉末冶金法将纳米单质铜(Cu0)、硅铁(FeSi)、四氧化三铁硅涂层(Fe3O4@SiO2)混合煅烧并制备出新型磁性硅铁载铜吸附剂MagFeSi-Cu0。实验研究不同烟气温度下MagFeSi-Cu0的汞吸附能力基础上,结合颗粒内扩散模型、准二阶动力学模型、Elovich模型及Bangham模型分析了MagFeSi-Cu0吸附Hg0过程的主要控制步骤。在此基础上,依据密度泛函理论(DFT)研究了不同反应温度下FeSi表面Cu0原子与Hg0原子的汞齐作用机制。研究结果表明,Bangham模型的拟合值与MagFeSi-Cu0汞吸附实验值拟合度最高,MagFeSi-Cu0表面痕量Hg0吸附由汞的外扩散和表面铜汞齐吸附共同控制;通过密度泛函计算,发现Cu0颗粒表面Cu-Hg齐吸附能为-0.534 eV,当烟气温度从80℃上升至200℃时,Hg0原子与单质Cu原子的吸附自由能从-22.47 kJ/mol下降至-13.96 kJ/mol,这些结果为深入了解Hg0在Cu(111)表面的反应机理提供了理论基础。 相似文献
45.
《Ceramics International》2022,48(21):31478-31490
Considering the great importance of nanocomposite based photo-active nanomaterials for a variety of electronics, photonics and photovoltaics application, it is always worth considering to synthesize new hetreostructure. This paper describes the sol-gel and hydrothermal synthesis of metal (holmium, barium, and cadmium) doped TiO2/CdS nanocomposites for photoanode applications. Various characterization techniques, including XRD, FTIR, UV–VIS, EDX, and SEM were used to examine the synthesized heterostructures. The band gap of pure TiO2 NPs is 3.10 eV, which was effectively decreased to 2.16 eV by doping and coupling with CdS. The nanomaterial's crystallinity, crystallite size, morphology and elemental composition were determined by XRD, SEM and EDX, respectively. As sensitizers, the organic dyes dithizone, carminic acid, and pyrocatechol violet were used. FTIR was used to analyze the effective dye grafting on the surface of nanomaterials. In the presence of hole conducting P3HT polymer as solid state electrolyte, the sensitized materials were evaluated for solid state dye-sensitized solar cells. Compared to the reference device, Cd–TiO2/CdS photosensitized using Pyrocatechol violet dye demonstrated the highest efficiency of 2.68% (0.82%). Other parameters of this device, including open circuit voltage (Voc) and short circuit current (Jsc), were determined to be 16.97 mA cm2 and 0.41V, respectively. 相似文献
46.
47.
Amyloid precursor protein (APP) is a type 1 transmembrane glycoprotein, and its homologs amyloid precursor-like protein 1 (APLP1) and amyloid precursor-like protein 2 (APLP2) are highly conserved in mammals. APP and APLP are known to be intimately involved in the pathogenesis and progression of Alzheimer’s disease and to play important roles in neuronal homeostasis and development and neural transmission. APP and APLP are also expressed in non-neuronal tissues and are overexpressed in cancer cells. Furthermore, research indicates they are involved in several cancers. In this review, we examine the biological characteristics of APP-related family members and their roles in cancer. 相似文献
48.
49.
持久蠕变强度外推是预测材料在高温高压环境下工作寿命的有效方法。目前常用的外推方法有等温法和参数法。这两种方法都是通过在实验室中采用较短时间的试验,获取试验数据后,通过Excel或者MATLAB等数据处理软件进行数据的拟合处理和外推计算。这些数据归纳统计和数学建模的过程有时需要耗费试验人员的大量精力。介绍了持久蠕变强度外推的软件实现方法,该功能包含在持久蠕变试验设备配备的软件中,试验完成后,软件直接根据试验结果进行数据拟合和持久蠕变强度的外推计算,无需试验数据的统计和建模,大大提高了持久蠕变强度外推计算的效率。 相似文献
50.
Carlos A. da Silva Henrique B. Pereira Maria E. S. Taqueda Zehbour Panossian 《工业材料与腐蚀》2021,72(11):1796-1807
In the offshore oil and gas industry, mainly focusing on the use of rigid or flexible pipes of subsea infrastructure applied to risers or flowlines, one of the greatest difficulties is the interpretation of the combined effects of the various correlated phenomena (hydrodynamic effects of intermittent flow, the effects of corrosivity of the environment in addition to variations in pressure, temperature, and dynamic loading). On the basis of this scenario, defining the degree of severity of each of the correlated system variables becomes of fundamental importance for establishing reliable criteria for selecting materials for subsea application. The established flow pattern directly affects the corrosion rate (or the pipe material mass loss), but the balance of other variables including possible changes in the physical and transported fluid chemical properties may increase the damage up to an order of magnitude, which is a piece of information normally not foreseen in design criteria. Therefore, to improve the understanding of the corrosion study influenced by multiphase flow, a testing loop was designed and assembled at the Corrosion and Protection Laboratory of the Institute for Technological Research, in which API X80 steel coupons were positioned in locations with a 0° and 45° inclinations. Tests were conducted by varying the partial pressure of the gaseous phase containing blends of CO2 and H2S with N2 balance, mixed with the liquid phase containing light oil and heavy oil in water with salinity (NaCl)-simulating oil well conditions with 80% water cut. The main objective of this study is to establish models that can predict the corrosion intensity in conditions close to those obtained experimentally. To achieve results, the multiple regression and Box–Cox transformation methods were applied. These models will make possible damage prediction and optimization of matrix parameters for the multiphase-loop test. 相似文献