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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.
摘要:为了研究300M超高强钢在中性盐雾环境中的腐蚀行为及腐蚀机制,采用失重法,宏观、微观腐蚀形貌分析,三维表面轮廓分析及电化学分析的研究方法,来表征腐蚀实验现象并进行分析。结果表明:300M超高强钢在中性盐雾环境中的腐蚀产物为FeOOH、Fe2O3、Fe(OH)3和Fe3O4;腐蚀速率随着腐蚀时间逐渐降低,腐蚀后期(72h)腐蚀速率降低50%;腐蚀初期以点蚀为主,点蚀坑通过横向扩展,逐渐发展为后期的均匀腐蚀,腐蚀表面形貌呈沟壑状;外腐蚀层对基体的保护能力很弱,Cr元素在锈层靠近基体的一侧偏聚使内腐蚀层具有一定的抗腐蚀性。  相似文献   
3.
The aim of this paper is to assess the closeness of agreement between results of ELISA and LC-MS/MS methods for determination of aflatoxin B1 in corn and aflatoxin M1 in milk. Samples of corn (n=100) and milk (n=250) were simultaneously analyzed using ELISA and LC-MS/MS methods, after the severe drought that affected Serbia in summer 2012 resulting in occurrence of aflatoxin B1 in corn and aflatoxin M1 in milk. Regression analysis showed higher level of agreement between aflatoxin B1 samples (R2=0.994), compared to aflatoxin M1 samples (R2=0.920). However, both techniques were satisfactory in meeting the requirements for official control purposes.  相似文献   
4.
线性调频信号检测方法的研究   总被引:1,自引:0,他引:1  
在线性调频信号体制雷达的数字脉冲压缩系统中对数字脉压后的脉冲串进行DFT相参处理时,若采样时刻偏离脉压回波的峰值时刻,不同的采样频率使得DFT相参积累后的信噪比会有不同程度的损失。本文详细分析了采样频率分别为20MHz和10MHz时对信号相参积累的影响,并对幅值检测和M/N检测两种检测方法的检测性能进行了比较,仿真结果表明,M/N检测优于幅值检测。  相似文献   
5.
Abstract. We consider semiparametric estimation in time‐series regression in the presence of long‐range dependence in both the errors and the stochastic regressors. A central limit theorem is established for a class of semiparametric frequency domain‐weighted least squares estimates, which includes both narrow‐band ordinary least squares and narrow‐band generalized least squares as special cases. The estimates are semiparametric in the sense that focus is on the neighbourhood of the origin, and only periodogram ordinates in a degenerating band around the origin are used. This setting differs from earlier studies on time‐series regression with long‐range dependence, where a fully parametric approach has been employed. The generalized least squares estimate is infeasible when the degree of long‐range dependence is unknown and must be estimated in an initial step. In that case, we show that a feasible estimate which has the same asymptotic properties as the infeasible estimate, exists. By Monte Carlo simulation, we evaluate the finite‐sample performance of the generalized least squares estimate and the feasible estimate.  相似文献   
6.
LB11820M是日本SANYO(三洋)公司开发的三相直流无刷电机驱动芯片。它具备开环三相电机控制所需的全部有效功能,并且具有比较完善的保护电路。文中介绍了LB11820M的特点、功能和工作原理,给出了其典型应用电路。  相似文献   
7.
Isotropic conductive adhesives (ICAs) have recently received a lot of focus and attention from the researchers in electronics industry as a potential substitute to lead-bearing solders. Numerous studies have shown that ICAs possess many advantages over conventional soldering such as environmental friendliness, finer pitch printing, lower temperature processing and more flexible and simpler processing. However, complete replacement of soldering by ICAs is yet not possible owing to several limitations of ICAs which are mainly related to reliability aspects like limited impact resistance, unstable contact resistance, low adhesion and conductivity etc. Continued efforts for last 15 years have resulted in development of ICAs with improved properties. This review article is aimed at providing a better understanding of ICAs, their principles, performance and significant research and development work addressing the technological utility of ICAs.  相似文献   
8.
中兴宽带线路测试技术及功能介绍   总被引:1,自引:0,他引:1  
宽带线路测试是中兴通讯ZXDSL综合宽带运维系统的核心功能,在DSL业务提供过程中主要应用在开通前的预测试、线路测试、开通后的例测试以及故障时的在线测试。对其测试技术和功能作了介绍。  相似文献   
9.
Announces the 2007 recipient of the Gold Medal Award for Life Achievement in the Practice of Psychology: Patricia M. Bricklin. A brief biography, highlighting areas of special focus in Bricklin's work, is provided. (PsycINFO Database Record (c) 2010 APA, all rights reserved)  相似文献   
10.
粉末材料中微夹杂缺陷的超声波检验   总被引:1,自引:0,他引:1  
梁菁  史亦韦 《材料工程》1996,(10):26-28
介绍了用超声波方法检测微夹杂缺陷的一些试验结果,比较了不同频率、不同试验方法检测能力的差异,并通过解剖试验对检出缺陷的性质、尺寸作了比较。  相似文献   
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