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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.
一类Feistel密码的线性分析 总被引:5,自引:0,他引:5
该文提出一种新的求取分组密码线性偏差上界的方法,特别适用于密钥线性作用的Feistel密码.该分析方法的思路是,首先对密码体制线性偏差进行严格的数学描述,分别给出密码线性偏差与轮函数F及S盒的线性偏差的数学关系;然后通过求取线性方程组最小重量解,确定密码线性偏差的上界. 相似文献
3.
提出了一种用双边带深侧槽的小尺寸圆形紧凑拉伸试样评定核压力容器(RPV)钢断裂韧性的单试作试验方法,给出了用该方法测定的两个厂家生产的核压力容器用A508CL3钢的断裂韧性参数,还与Charpy试样的试验结果及大尺寸标准试样的试验结果进行了比较。研究结果表明:用双边带深侧槽的小尺寸R-CT试样测得的断裂韧性值比相同恻槽深度预制疲劳裂纹Charpy试样的测试值更接近有效断裂韧性值,所以,用于核压力容器断裂韧性的监测是可行的。 相似文献
4.
One hypothesis for the transcontinental and intra-Great Lakes basin transfer of round gobies (Neogobius melanostomus) has been that round gobies were pumped into the ballast water of ships. During June 2005 in Lake Erie, we obtained evidence of a vertical migration of round goby larvae, when we collected 167 round goby larvae in surface ichthyoplankton net tows at night and zero during day. These results complemented similar findings from the Muskegon River estuary of Lake Michigan during 2003 and 2004, documenting diel vertical migration for the first time in larval round gobies. We suggest vertical migration behavior may have allowed larval round gobies to be transported to and within the Great Lakes via ballast water and dispersed in the Great Lakes via advection of 6.5–8.5-mm long larvae at the surface. Based on our results, if ballast water was only taken on near the surface during daylight hours from May through September when larval round gobies were present, it would have mitigated the spread of round gobies throughout the Great Lakes. 相似文献
5.
Ionic Strength Effects on Heat-induced Gelation of Myofibrils and Myosin from Fast- and Slow-twitch Rabbit Muscles 总被引:1,自引:0,他引:1
The effects of ionic strength on myofibrils and myosin from rabbit fast-twitch Psoas major (PM) and slow-twitch Semimembranosus proprius (SMp) muscles before and after heating were studied by electron microscopy and thermal scanning rheometry. The direct suspension of proteins in low ionic strength (0.2M KCl; pH 6.0) led to very weak gels, whereas a gradual lowering of the ionic strength (by dialysis against 0.2M KCl; pH 6.0) of 0.6M KCl protein solutions induced strand-type networks at low temperature and strong heat-induced gels. As shown by transmission and scanning electron micrographs, in low ionic strength, SMp myosins formed shorter filaments before heating and thinner and shorter structures in heat-induced gels, as well as a lower gel porosity than PM myosins. 相似文献
6.
《Displays》2021
Immersion in virtual reality is still linked to symptoms of visual fatigue such as eye strain, dizziness, and overall discomfort. Studies have investigated visual fatigue through pre- and post-immersion tests of the visual function. In this work, we extend on our previous study and derive a visual fatigue likelihood metric using biomechanical analysis. Previously, we have investigated the effect of VR on the vergence system during immersion. The proposed visual fatigue metric exhibited a significant correlation to vergence angle variability which was previously linked to vergence accommodation conflict in VR. We also discuss subjective feedback and its relationship with the proposed visual fatigue metric. 相似文献
7.
Sajjad Zare Akbar Ghaffarpour Rahbar 《Journal of Network and Computer Applications》2012,35(1):459-468
Congestion is one of the most important challenges in optical networks. In a Passive Optical Network (PON), the Optical Line Terminal (OLT) is a bottleneck and congestion prone. In this paper, a framework is proposed with Forward Error Correction (FEC) at the IP layer combined with Weighted Round Robin (WRR) at the scheduling level to overcome packet-loss due to congestion in the OLT in order to achieve efficient video multicasting over PON. In the FEC scheme, Reed-Solomon (RS(n,k)) with erasure coding is used, where (n−k) erroneous symbols per n symbol blocks can be corrected. In our framework, an Internet Protocol TeleVision (IPTV) service provider uses the mentioned RS coding and generates redundant packets from regular IPTV packets in such a way that an Optical Network Unit (ONU) can recover lost packets from received packets, thus resulting in a better video quality. Simulation results show that using the proposed framework, an ONU can recover many lost packets and achieve better video quality under different traffic loads for its users. For instance, the proposed method can reduce packet loss rate by almost 55% and 10% under traffic load 0.9, respectively, compared with the Round Robin (RR) and WRR methods under symmetric traffic load. When High Receivers Queue (HRQ) traffic (i.e., traffic received by many users) is twice Low Receivers Queue (LRQ) traffic (i.e., traffic received by a small number of users), this reduction is almost 86% and 30% under traffic load 0.9. Finally, when LRQ traffic is twice HRQ traffic, the reduction in packet loss rate is almost 70% and 91% at traffic load 0.5. 相似文献
8.
Svetlana V. Klinova Boris A. Katsnelson Ilzira A. Minigalieva Oksana P. Gerzen Alexander A. Balakin Ruslan V. Lisin Ksenia A. Butova Salavat R. Nabiev Oleg N. Lookin Leonid B. Katsnelson Larisa I. Privalova Daniil A. Kuznetsov Vladimir Ya. Shur Ekaterina V. Shishkina Oleg H. Makeev Irene E. Valamina Vladimir G. Panov Marina P. Sutunkova Larisa V. Nikitina Yuri L. Protsenko 《International journal of molecular sciences》2021,22(7)
Subchronic intoxication was induced in outbred male rats by repeated intraperitoneal injections with lead oxide (PbO) and/or cadmium oxide (CdO) nanoparticles (NPs) 3 times a week during 6 weeks for the purpose of examining its effects on the contractile characteristics of isolated right ventricle trabeculae and papillary muscles in isometric and afterload contractions. Isolated and combined intoxication with these NPs was observed to reduce the mechanical work produced by both types of myocardial preparation. Using the in vitro motility assay, we showed that the sliding velocity of regulated thin filaments drops under both isolated and combined intoxication with CdO–NP and PbO–NP. These results correlate with a shift in the expression of myosin heavy chain (MHC) isoforms towards slowly cycling β–MHC. The type of CdO–NP + PbO–NP combined cardiotoxicity depends on the effect of the toxic impact, the extent of this effect, the ratio of toxicant doses, and the degree of stretching of cardiomyocytes and muscle type studied. Some indices of combined Pb–NP and CdO–NP cardiotoxicity and general toxicity (genotoxicity included) became fully or partly normalized if intoxication developed against background administration of a bioprotective complex. 相似文献
9.
利用金属原位分析技术和钻孔化学分析方法对高淬透性轴承钢GCr15SiMn的Φ450 mm连铸圆坯和Φ130 mm圆钢的C、Si、Mn、Cr元素横截面的分布情况进行分析。采用推力片式接触疲劳试验机进行了材料的接触疲劳寿命测试。结果表明:GCr15SiMn连铸圆坯C元素的偏析倾向较大,易产生中心正偏析,而Cr、Si、Mn元素的偏析倾向较小。通过采取稳定低过热度浇铸、三段强电磁搅拌等措施,铸坯的中心碳偏析得以改善。采用(1240±20)℃×5 h高温扩散、初轧首道次变形量≥90 mm大变形轧制的Φ130 mm圆钢的碳偏析可以得到进一步的改善,试验钢在5.3GPa高应力负载下的接触疲劳额定寿命L10达到3.58×106次,接近电渣重熔钢的水平。 相似文献
10.
Emre Akyurek Alexandre Dilly Fabrice Jourdan Zhu Liu Souraneel Chattoraj Itziar Berruezo Juandeaburre Michel Heinrich Leonid Paramonov Peter Turner Stelarc Tatiana Kalganova 《计算机技术与应用:英文》2013,(11):569-574
In the development of robotic limbs, the side of members is of importance to define the shape of artificial limbs and the range of movements. It is mainly significant tbr biomedical applications concerning patients suffering arms or legs injuries, fn this paper, the concept of an ambidextrous design lbr robot hands is introduced. The fingers can curl in one xvay or another, to imitate either a right hand or a left hand. The advantages and inconveniences of different models have been investigated to optimise the range and the maximum force applied by fingers. Besides, a remote control interthce is integrated to the system, allowing both to send comrnands through internet and to display a video streaming of the ambidextrous hand as feedback. Therefore, a robotic prosthesis could be used for the first time in telerehabilitation. The main application areas targeted are physiotherapy alter strokes or management of phantom pains/br amputees by/earning to control the ambidextrous hand. A client application is also accessible on Facehook social network, making the robotic limb easily reachable for the patients. Additionally the ambidextrous hand can be used tbr robotics research as well as artistic performances. 相似文献