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Pseudoxanthoma elasticum (PXE) is a complex autosomal recessive disease caused by mutations of ABCC6 transporter and characterized by ectopic mineralization of soft connective tissues. Compared to the other ABC transporters, very few studies are available to explain the structural components and working of a full ABCC6 transporter, which may provide some idea about its physiological role in humans. Some studies suggest that mutations of ABCC6 in the liver lead to a decrease in some circulating factor and indicate that PXE is a metabolic disease. It has been reported that ABCC6 mediates the efflux of ATP, which is hydrolyzed in PPi and AMP; in the extracellular milieu, PPi gives potent anti-mineralization effect, whereas AMP is hydrolyzed to Pi and adenosine which affects some cellular properties by modulating the purinergic pathway. Structural and functional studies have demonstrated that silencing or inhibition of ABCC6 with probenecid changed the expression of several genes and proteins such as NT5E and TNAP, as well as Lamin, and CDK1, which are involved in cell motility and cell cycle. Furthermore, a change in cytoskeleton rearrangement and decreased motility of HepG2 cells makes ABCC6 a potential target for anti-cancer therapy. Collectively, these findings suggested that ABCC6 transporter performs functions that modify both the external and internal compartments of the cells.  相似文献   
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Palm fatty acid distillate (PFAD) is a rich source of vitamin E. As compared to other vegetable oil, PFAD has higher tocotrienol (70–80%) over tocopherol content, which makes it a valuable source for vitamin E extraction. Current vitamin E extraction methods are not sustainable due to the intensive usage of chemical and high operational cost. Hence, the present study investigated for the first time using dry fractionation process as a green and economical pretreatment method for separating solid fraction (stearin) and liquid fraction (olein) in order to concentrate vitamin E from PFAD in olein fraction. We examined the dry fractionation conditions: crystallization ending temperature (36–44 °C), cooling rate (0.3 and 1.5°C min−1), stirring speed (20–125 rpm), and holding time (0–60 min) on the composition of unsaturated and saturated fatty acids as well as vitamin E content in liquid fraction (olein) and solid fraction (stearin) using gas chromatography and high performance liquid chromatography, respectively. In most of these conditions, vitamin E was ultimately higher in olein fraction as compared to stearin fraction, which is correlated with the high degree of unsaturation. Under a cooling rate of 0.3°C min−1, 90 rpm stirring speed, and ending crystallization of 38 °C, the highest vitamin E rich olein fraction was attained with 1479 ± 10.51 ppm in 50 g olein fraction as compared to 1366 ± 7.94 ppm in 500 g of unfractionated PFAD.  相似文献   
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In the present investigation, systematic grinding experiments were conducted in a laboratory ball mill to determine the breakage properties of low-grade PGE bearing chromite ore. The population balance modeling technique was used to study the breakage parameters such as primary breakage distribution (Bi, j) and the specific rates of breakage (Si). The breakage and selection function values were determined for six feed sizes. The results stated that the breakage follows the first-order grinding kinetics for all the feed sizes. It was observed that the coarser feed sizes exhibit higher selection function values than the finer feed size. Further, an artificial neural network was used to predict breakage characteristics of low-grade PGE bearing chromite ore. The predicted results obtained from the neural network modeling were close to the experimental results with a correlation of determination R2 = 0.99 for both product size and selection function.  相似文献   
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《Ceramics International》2021,47(21):29646-29652
In the present study, the fatigue behavior and damage evolution of SiC/SiC minicomposites at elevated temperatures in oxygen-free environment are investigated which are important for their application and are still unclear. The high-temperature fatigue test platform is developed and the fatigue stress-life curve and the stress-strain response are obtained. The test result shows that the life of the material at elevated temperature is shorter than that at room temperature under the same stress level. Moreover, the hysteresis loop width and the residual strain increase with the increasing of the cycles while the hysteresis modulus decreases during the fatigue cycling. The evolution process of matrix cracks is observed using the real-time remote detection system. It is found that matrix cracking is insensitive to the cyclic loading which is similar to room temperature and is due to that the degeneration of the interfacial shear stress reduces the area of high stress in matrix. The fiber/matrix interfacial shear stress under different cycles is determined based on the fatigue modulus of each hysteresis loop. The result shows a fatigue enhancement phenomenon for the interface which is not observed at room temperature.  相似文献   
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Since solid, non-metallic inclusions influence considerably the quality of casted steel products, carbon-bonded alumina foam filters are used in secondary metallurgical treatments to remove these particles from steel melts. In order to attain a significant improvement of the filtration process, five different carbonaceous spinel compounds from the Fe-/Mg-/Mn-Al-O systems are applied on carbon-bonded alumina filters in this study and investigated with regard of their filtration efficiency. However, these spinel compounds decompose partially during sintering at 1400?°C under reducing atmosphere, wherefore the resulting coatings contain not only spinel compounds, but also oxidic and metallic components. The subsequent interaction with molten steel leads to the development of multicrystal structures on the filter surface, which stem from interfacial reactions between coating materials, molten steel, and inclusions. As a result of this procedure, a reduction of almost 60% alumina inclusions is measured with the aid of an automatic SEM, whereby spinel compounds from the Fe-Mn-Al-O system achieve highest filtration efficiencies.  相似文献   
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We investigate nonlinear phase dynamics of an ideal kink mode, induced by E × B flow. Here the phase is the cross phase(θ_c) between perturbed stream function of velocity■ and magnetic field ■, i.e. θ_c= θ_φ-θ_ψ. A dimensionless parameter, analogous to the Richardson number,■(γ_(kink): the normalized growth rate of the pure kink mode; ■: normalized E × B shearing rate) is defined to measure the competition between phase pinning by the current density and phase detuning by the flow shear. When R_i 1, θ_c is locked to a fixed value,corresponding to the conventional eigenmode solution. When R_i≤1, θ_c enters a phase slipping or oscillating state, corresponding to a nonmodal solution. The nonlinear phase dynamics method provides a more intuitive explanation of the complex dynamical behavior of the kink mode in the presence of E × B shear flow.  相似文献   
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本文论述了17型车钩钩体生产过程的工艺改进,介绍了17型车钩钩体尾部裂纹产生的原因及采取的措施。通过工艺上采用保温暗冒口提高凝固过程补缩性,同时采用空心销孔芯提高芯子的退让性,并加强车钩钩体生产过程质量控制等措施,成功地解决了17型车钩钩体尾部裂纹问题,产品废品率由4.3%降到0.23%.  相似文献   
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