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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
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Zhijiang Li Yingping Zheng Liqin Cao Lei Jiao Yanfei Zhong Caiyi Zhang 《Color research and application》2020,45(4):656-670
Image color clustering is a basic technique in image processing and computer vision, which is often applied in image segmentation, color transfer, contrast enhancement, object detection, skin color capture, and so forth. Various clustering algorithms have been employed for image color clustering in recent years. However, most of the algorithms require a large amount of memory or a predetermined number of clusters. In addition, some of the existing algorithms are sensitive to the parameter configurations. In order to tackle the above problems, we propose an image color clustering method named Student's t-based density peaks clustering with superpixel segmentation (tDPCSS), which can automatically obtain clustering results, without requiring a large amount of memory, and is not dependent on the parameters of the algorithm or the number of clusters. In tDPCSS, superpixels are obtained based on automatic and constrained simple non-iterative clustering, to automatically decrease the image data volume. A Student's t kernel function and a cluster center selection method are adopted to eliminate the dependence of the density peak clustering on parameters and the number of clusters, respectively. The experiments undertaken in this study confirmed that the proposed approach outperforms k-means, fuzzy c-means, mean-shift clustering, and density peak clustering with superpixel segmentation in the accuracy of the cluster centers and the validity of the clustering results. 相似文献
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介绍用已知年龄和烧结温度的古陶片穆斯堡尔超精细参数与年龄和烧结温度的实验关系曲线作基础,应用计算机模拟程序,自动求出未知陶片的年龄和最高烧结温度的方法。 相似文献
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堇青石基玻璃陶瓷的制备与展望 总被引:2,自引:0,他引:2
介绍了制备堇青石基玻璃陶瓷的三种方法:熔融法、烧结法和溶胶-凝胶法,综述了玻璃组成、添加剂和成核剂对堇青石基玻璃陶瓷的烧结及性能的影响,并对其未来的发展进行了展望。 相似文献
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Masami Ikeda Susumu Fukumoto Hiroshi Takao Shinya Ohtsuka Eiichi Haginomori Masayuki Hikita 《Electrical Engineering in Japan》2002,141(4):16-24
This paper describes the dielectric breakdown characteristics of oil and oil‐impregnated paper for very fast transient (VFT) voltages. Blumlein circuits generate VFT voltages of 60 and 300 ns in a pulse width that simulates disconnecting switching surges in gas‐insulated switch gears. We measured the breakdown voltages of needle‐to‐plane, plane‐to‐plane oil gaps and several pieces of paper between plane electrodes for VFT and lightning impulse voltages. The measured data were formulated in V‐t characteristics and Weibull probability distributions. The inclination n of V‐t characteristics of insulating paper is 150, which is less than n = 13.7 of the plane‐to‐plane oil gap in the VFT time range. The shape parameters of Weibull distribution obtained in this study show that the scattering of breakdown voltages of paper is much less than that of oil. © 2002 Wiley Periodicals, Inc. Electr Eng Jpn, 141(4): 16–24, 2002; Published online in Wiley InterScience ( www.interscience.wiley.com ). DOI 10.1002/eej.10043 相似文献
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钢包钢水保温覆盖剂的开发 总被引:2,自引:0,他引:2
研制了钢包钢水新型保温覆盖剂,主要成分为(%);15-25C,20-35SiO2,10-20Al2O3,2-5Fe2O3,15-25CaO,2-5MgO,新覆盖剂铺展性与保温性能良好。经转炉钢水160t钢包生产使用,统计数据表明,镇静15min后钢水降温速度由原碳化稻壳覆盖剂1.33℃/min降低到0.93℃/min。 相似文献
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