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.     

Trajectories of knowledge economy in SSA and MENA countries

In the first critical assessment of knowledge economy dynamic paths in Africa and the Middle East, but for a few exceptions, we find overwhelming support for diminishing cross-country disparities in knowledge-based economy dimensions. The paper employs all the four components of the World Bank's Knowledge Economy Index (KEI): economic incentives, innovation, education, and information infrastructure. The main finding suggests that sub-Saharan African (SSA) and the Middle East and North African (MENA) countries with low levels of KE dynamics and catching-up their counterparts of higher KE levels. We provide the speeds of integration and time necessary to achieve full (100%) integration. Policy implications are also discussed.     

Alloying behavior and novel properties of CoCrFeNiMn high-entropy alloy fabricated by mechanical alloying and spark plasma sintering

An equiatomic CoCrFeNiMn high-entropy alloy was synthesized by mechanical alloying (MA) and spark plasma sintering (SPS). During MA, a solid solution with refined microstructure of 10 nm which consists of a FCC phase and a BCC phase was formed. After SPS consolidation, only one FCC phase can be detected in the HEA bulks. The as-sintered bulks exhibit high compressive strength of 1987 MPa. An interesting magnetic transition associated with the structure coarsening and phase transformation was observed during SPS process.     

Performance evaluation of noise reduction method during on-line monitoring of MV switchgear for PD measurements by non-intrusive sensors

Partial Discharge (PD) measurement is a globally accepted method for insulation diagnosis of electrical assets. The consequences of insulation breakdown are well known. The trend is to move from conventional offline testing to online monitoring for insulation life prediction, which results in the inclusion of high frequency noise in the captured signals. Therefore de-noising is of paramount importance in online monitoring to obtain useful information from the signal.In this research, a 20 kV switchgear panel has been subjected to PD faults in the laboratory and measurements have been carried out by using different non-intrusive sensors including a novel sensor, the D-dot sensor and recorded by a high frequency oscilloscope. The measured results show the effective applicability of sensors for switchgear. The Discrete Wavelet Transform (DWT) has been used to de-noise PD signals in this paper. Time domain and frequency domain comparison of original and de-noised PD signals reveals the significance of this technique for online monitoring of Medium Voltage (MV) switchgear. Finally, an adaptive online de-noising concept, based on automatic de-noising is also proposed in this paper.     

Biotransformation of l‐menthol by twelve isolates of soil‐borne plant pathogenic fungi (Rhizoctonia solani) and classification of fungi

The microbial transformation of l‐menthol ( 1 ) was investigated by using 12 isolates of soil‐borne plant pathogenic fungi, Rhizoctonia solani (AG‐1‐IA Rs24, Joichi‐2, RRG97‐1; AG‐1‐IB TR22, R147, 110.4; AG‐1‐IC F‐1, F‐4, P‐1; AG‐1‐ID RCP‐1, RCP‐3, and RCP‐7) as a biocatalyst. Rhizoctonia solani F‐1, F‐4 and P‐1 showed 89.7–99.9% yields of converted product from 1 , RCP‐1, RCP‐3, and RCP‐7 26.0–26.9% and the other isolates 0.1–12.0%. In the cases of F‐1, F‐4 and P‐1, substrate 1 was converted to (?)‐(1S,3R,4S,6S)‐6‐hydroxymenthol ( 2 ), (?)‐(1S,3R,4S)‐1‐hydroxymenthol ( 3 ) and (+)‐(1S,3R,4R,6S)‐6,8‐dihydroxymenthol ( 4 ), which was a new compound. Substrate 1 was converted to 2 and/or 3 by RRG97‐1, 110.4, RCP‐1, RCP‐3 and RCP‐7. The structures of the metabolic products were elucidated on the basis of their spectral data. In addition, metabolic pathways of the biotransformation of 1 by Rhizoctonia solani are discussed. Finally, from the main component analysis and the differences in the yields of converted product from 1 , the 12 isolates of Rhizoctonia solani were divided into three groups based on an analysis of the metabolites. Copyright © 2003 Society of Chemical Industry     

Fracture and deformation properties of Ni-Fe superalloy in cryogenic high magnetic field environments

The present paper includes experimental and analytical data on the fracture properties of a nickel-iron superalloy, a ferromagnetic austenite, at 4 K in magnetic fields of 0 and 6 T. The tensile, notch tensile and small punch tests are employed. A finite element analysis is also performed to convert the experimentally measured load-displacement data into useful engineering information. To interpret the results we review the available theory of the influence of magnetic field on the stress intensity factor for a crack in ferromagnetic materials.     

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.     

微机械可调谐滤波器的研制

利用 Ga As/Al Ga As分布反馈 Bragg反射镜在 Ga As衬底上制作了一个微机械的调谐滤波器 .该器件在 7V调谐电压下调谐范围达28nm     

小型月球探测器高精度姿态指向控制

针对小型月球探测器三轴姿态高指向精度和高稳定度的要求 ,采用欧拉动力学和四元数方法描述 ,建立了小型月球探测器环月阶段动力学模型 ,分析了由 3个轻型反作用轮正交安装组成的姿态稳定控制系统 ,给出了一种拟PD姿态指向控制规律。对所设计的指向控制律进行了数学仿真 ,结果表明可达到 0 .0 1°/s的稳定度和 0 .3°的指向精度 ,验证了该控制规律的可行性。     

故县大坝坝基泥化性F_5断层的化学灌浆防渗加固处理

泥化软化性F_5断层是故县大坝坝基范围内最大的一条断层,位于左岸,对左坝肩的稳定极为不利,采用水泥灌浆处理无效,故改用化学灌浆处理。选用EAA化学浆材,采用“优选组合配方综合施灌”的创新施工工艺,效果良好,为今后低渗透性复杂的泥化软化性断层的防渗加固处理,提供了新的依据和途径。