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.     

新一代深度张力面板

新一代深度张力面板不仅具有基本的深度、张力采集、处理、显示功能,同时还具有多种报警及通讯功能.其最大的特点是采用了液晶显示屏和触摸屏,使得信息的显示更加丰富、直观和醒目.同时,触摸屏的采用,省去了传统的按钮、开关及键盘操作模式,使操作更加方便简洁、自然.该面板可以单独工作,也可以配备于测井、录井地面系统中使用.     

Fe_(78)Si_9B_(13)非晶合金的高温变形性能

通过高温拉伸及胀形实验,研究了Fe78Si9B13非晶合金的塑性变形性能。高温拉伸的温度范围为430℃~530℃,初始应变速率为1.67×10-4s-1~1.67×10-3s-1。利用X射线衍射(XRD)和扫描电镜(SEM)对高温变形后的微观组织进行了分析。高温拉伸的延伸率随温度的升高先增大后减小,450℃时达到最大;在450℃,初始应变速率为8.33×10-4s-1时延伸率为40%。在450℃胀形得到半径为5mm、高4mm的近半球试件,显示了Fe78Si9B13非晶合金具有良好的高温变形性能。高温塑性变形过程中伴随着非晶的晶化,使塑性流动应力增大,影响了Fe78Si9B13非晶合金的高温变形性能。     

制备条件对非晶态Fe78B13Si9合金的热膨胀各向异性的影响

本文研究了不同条件下形成的非易态Fe_(78)B_(13)Si_9合金在长轴和短轴方向上的热膨胀。结果表明,在所有条件下均存在热膨胀的各向异性。热膨胀系数随着冷却速度的增加而增加,但其各向异性减小;随着熔体喷铸温度的提高两者均减小。在热循环的作用下,短轴方向的热膨胀系数及其各向异性均减小;而在长轴方向的热膨胀系数却增大。我们认为热膨胀各向异性来源于原子交互作用力的方向性、原子振动频率和短程有序结构或缺陷的方向性。     

燃气表无线抄表系统的设计与实现

针对现在抄表系统复杂度高或成本高等问题,本文设计了一套燃气表无线抄表系统,从基本架构、主要功能、软硬件的实现加以说明,具有实时、可靠、经济、低功耗等优点.该系统采用M430F413和EM78P153S为控制单元,CC1100为无线通信单元.该系统稳定可靠,具有良好的应用和推广价值.     

Sharing electronic structure and crystallographic data with ETSF_IO

We present a library of routines whose main goal is to read and write exchangeable files (NetCDF file format) storing electronic structure and crystallographic information. It is based on the specification agreed inside the European Theoretical Spectroscopy Facility (ETSF). Accordingly, this library is nicknamed ETSF_IO. The purpose of this article is to give both an overview of the ETSF_IO library and a closer look at its usage. ETSF_IO is designed to be robust and easy to use, close to Fortran read and write routines. To facilitate its adoption, a complete documentation of the input and output arguments of the routines is available in the package, as well as six tutorials explaining in detail various possible uses of the library routines.

Program summary

Program title: ETSF_IOCatalogue identifier: AEBG_v1_0Program summary URL:http://cpc.cs.qub.ac.uk/summaries/AEBG_v1_0.htmlProgram obtainable from: CPC Program Library, Queen's University, Belfast, N. IrelandLicensing provisions: Gnu Lesser General Public LicenseNo. of lines in distributed program, including test data, etc.: 63 156No. of bytes in distributed program, including test data, etc.: 363 390Distribution format: tar.gzProgramming language: Fortran 95Computer: All systems with a Fortran95 compilerOperating system: All systems with a Fortran95 compilerClassification: 7.3, 8External routines: NetCDF, http://www.unidata.ucar.edu/software/netcdfNature of problem: Store and exchange electronic structure data and crystallographic data independently of the computational platform, language and generating softwareSolution method: Implement a library based both on NetCDF file format and an open specification (http://etsf.eu/index.php?page=standardization)     

Design of experiments for bivariate binary responses modelled by Copula functions

Optimal design for generalized linear models has primarily focused on univariate data. Often experiments are performed that have multiple dependent responses described by regression type models, and it is of interest and of value to design the experiment for all these responses. This requires a multivariate distribution underlying a pre-chosen model for the data. Here, we consider the design of experiments for bivariate binary data which are dependent. We explore Copula functions which provide a rich and flexible class of structures to derive joint distributions for bivariate binary data. We present methods for deriving optimal experimental designs for dependent bivariate binary data using Copulas, and demonstrate that, by including the dependence between responses in the design process, more efficient parameter estimates are obtained than by the usual practice of simply designing for a single variable only. Further, we investigate the robustness of designs with respect to initial parameter estimates and Copula function, and also show the performance of compound criteria within this bivariate binary setting.     

On the existence and convergence of the solution of PML equations

In this article we study the mesh termination method in computational scattering theory known as the method of Perfectly Matched Layer (PML). This method is based on the idea of surrounding the scatterer and its immediate vicinity with a fictitious absorbing non-reflecting layer to damp the echoes coming from the mesh termination surface. The method can be formulated equivalently as a complex stretching of the exterior domain. The article is devoted to the existence and convergence questions of the solutions of the resulting equations. We show that with a special choice of the fictitious absorbing coefficient, the PML equations are solvable for all wave numbers, and as the PML layer is made thicker, the PML solution converge exponentially towards the actual scattering solution. The proofs are based on boundary integral methods and a new type of near-field version of the radiation condition, called here the double surface radiation condition. Partly supported by the Finnish Academy, project 37692.     

Measurement of 76Se and 78Se (γ, n) Cross Sections

The (γ, n) cross sections of Se isotopes (⁷⁶Se,⁷⁸Se) were measured to supply fundamental data for estimating the inverse reaction cross section, i.e., the ⁷⁹Se(n, γ)⁸⁰Se cross section. The enriched samples and a reference ¹⁹⁷Au sample were irradiated with laser-Compton scattering (LCS) γ-rays. The excitation function of each (γ, n) cross section was determined for the energy range from each near neutron separation energy to the threshold energy of (γ, 2n) reaction. The energy point corresponding to each cross section was deduced using the accurately determined energy distribution of LCS γ-rays. Systematic (γ, n) cross sections for Se isotopes including ⁸⁰Se were compared with those calculated by using a statistical model calculation code TALYS.     

一种基于车载MP3播放机的倒车雷达设计

提出了一种基于车载MP3播放机-SH252的倒车雷达设计方案,并介绍了系统的软硬件设计思路。系统采用EM78P153单片机作为倒车雷达数据采集部分,采用超声波探测方式测距。在倒车时,倒车雷达会自动启动,并在EM78P153的控制下,由装置于车尾保险杠上的探头发送超声波信号,当遇到障碍物时,产生回波信号,探头接收到回波信号后经控制器进行数据处理并计算出车体与障碍物之间的实际距离,然后将数据发送到车载MP3播放机上进行距离显示和语音同步提示,为驾驶员提供报警提示,提高驾驶的安全性。