Role of the HSP70 Co-Chaperone SIL1 in Health and Disease

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.     

唐钢ER70S-6焊丝钢的生产工艺研究及质量分析

介绍了唐钢ER70S-6焊丝钢的特点，对盘条质量进行了分析。对该钢化学成分、冶炼和轧制工艺进行了优化研究，使其综合性能明显改善。     

我国第一套Φ170mm三辊轧管机组

介绍了我国第一套Φ１７０ｍｍ三辊轧管机组热轧设备和产品规格及技术特点。     

舞钢管线钢的开发与生产

系统介绍了舞钢开发的针状铁素体型X70宽厚板的设计思路、工艺特点和实物水平；采用电炉 炉外精炼 连铸 TMCP工艺生产的X70宽厚板满足西气东输专用技术条件的要求，并批量应用在西气东输主干线工程中。     

首钢“川气出川”工程用17.5mmX70管线钢的试制

介绍了首钢试制17.5mm规格高强度、高韧性X70管线钢的生产工艺（成分设计、炼钢和轧钢工艺）及产品的力学性能。在华油钢管厂完成制管（管径为1016mm）以及相应的检验后，所有性能指标全部达到“川气出川”管道工程的技术规范要求。     

Optimal estimation of parameters of dynamical systems by neural network collocation method

In this paper we propose a new method to estimate parameters of a dynamical system from observation data on the basis of a neural network collocation method. We construct an object function consisting of squared residuals of dynamical model equations at collocation points and squared deviations of the observations from their corresponding computed values. The neural network is then trained by optimizing the object function.The proposed method is demonstrated by performing several numerical experiments for the optimal estimates of parameters for two different nonlinear systems. Firstly, we consider the weakly and highly nonlinear cases of the Lorenz model and apply the method to estimate the optimum values of parameters for the two cases under various conditions. Then we apply it to estimate the parameters of one-dimensional oscillator with nonlinear damping and restoring terms representing the nonlinear ship roll motion under various conditions. Satisfactory results have been obtained for both the problems.     

西气东输工程用X70热轧板卷屈服强度检测值波动大的探讨

对西气东输工程用X70热轧板卷检验过程中出的屈服强度σ0.5波动大的情况进行了分析，认为拉伸试样弯曲是主要原因。这一问题可通过反复压平试样，确保试样的平直度以及适当提高钢的强度的方法加以解决。     

CB-60/CB-70还原态重整催化剂的应用

介绍了福建炼油化工有限公司重整装置CB -60 /CB -70重整催化剂 (还原态 )分段组合装填的应用情况 ,列出了还原态催化剂和氧化态催化剂开工的不同特点。通过开工初期标定的数据与CB -6/CB -7催化剂的对比 ,说明CB -60 /CB -70催化剂有良好的活性和选择性 ,尤其是选择性、产氢率优于CB -6/CB -7,且使开工步骤得到了简化     

西门子自动化实验室的建立及应用

介绍以西门子S7-300/200PLC、WINCC6.0、MM440变频器、6SE70系列变频器等产品为基础建立的莱钢新区炼钢厂西门子自动化实验室。     

Differential Effects of STCH and Stress-Inducible Hsp70 on the Stability and Maturation of NKCC2

Mutations in the Na-K-2Cl co-transporter NKCC2 lead to type I Bartter syndrome, a life-threatening kidney disease. We previously showed that export from the ER constitutes the limiting step in NKCC2 maturation and cell surface expression. Yet, the molecular mechanisms involved in this process remain obscure. Here, we report the identification of chaperone stress 70 protein (STCH) and the stress-inducible heat shock protein 70 (Hsp70), as two novel binding partners of the ER-resident form of NKCC2. STCH knock-down increased total NKCC2 expression whereas Hsp70 knock-down or its inhibition by YM-01 had the opposite effect. Accordingly, overexpressing of STCH and Hsp70 exerted opposite actions on total protein abundance of NKCC2 and its folding mutants. Cycloheximide chase assay showed that in cells over-expressing STCH, NKCC2 stability and maturation are heavily impaired. In contrast to STCH, Hsp70 co-expression increased NKCC2 maturation. Interestingly, treatment by protein degradation inhibitors revealed that in addition to the proteasome, the ER associated degradation (ERAD) of NKCC2 mediated by STCH, involves also the ER-to-lysosome-associated degradation pathway. In summary, our data are consistent with STCH and Hsp70 having differential and antagonistic effects with regard to NKCC2 biogenesis. These findings may have an impact on our understanding and potential treatment of diseases related to aberrant NKCC2 trafficking and expression.