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.     

Ceramics composites from iron ore tailings and blast furnace slag

The search for materials and methods capable of reducing human impacts on the environment is of utmost importance nowadays. This study's primary purpose was to analyze the technical feasibility of ceramic composites production utilizing Fundão Dam's Iron Ore Tailings (IOT), Blast Furnace Slag (BFS) from charcoal, and Foundry Sand (FS) as partial substitutes for the traditional raw materials – sand and clay – for application in building industry materials. The composites were molded in rectangular specimens and fired at temperatures of 900, 950, 1000, 1050, and 1200 °C. The developed materials were analyzed and characterized by X-Ray Diffraction (XRD), Scanning Electron Microscopy (SEM), Energy Dispersive Spectroscopy (EDS), Thermogravimetry (TGA), and Differential Thermal Analysis (DTA). The obtained materials had flexural strength modulus of up to 12.19 MPa, water absorption ranging from 2 to 22%, linear shrinkage ranging from 0.02 to 6.50%, and apparent density ranging from 2.03 to 1.63 g/cm³. The study of the internal structure formation process revealed the formation of amorphous structures in the composites. The results demonstrated that these waste materials may be jointly used in construction materials, contributing to the reduction of natural resource extraction, besides enabling their correct disposal, minimizing environmental impacts, and improving the life quality of the surrounding communities.     

燃气热水器在无回水管水路中实现零冷水的方案研究

本文分析了燃气热水器行业针对无回水管水路系统实现零冷水功能的现有技术方案,指出现有技术方案存在的一些缺陷,并结合试验研究,对无回水管水路系统提出了一套全新的实现零冷水功能的技术解决方案。     

浅析火力发电厂汽水系统中内径管与外径管的设计选择

按照管道加工工艺的不同,管道可分为内径控制管和外径控制管。通过热轧工艺生产的外径控制无缝钢管,可满足火力发电厂汽水系统中绝大部分管道的使用要求。对于超(超)临界机组的主蒸汽和高温再热蒸汽管道采用的P91/P92材质的大口径厚壁无缝钢管,由于对材料性能和加工工艺有特殊要求,因此宜采用内径控制管。     

不同管距时玉米渗灌效果的试验研究

在运城市夹马口灌区和红旗灌区进行了1996,1997两个年度的玉米渗灌试验,研究了不同毛管间距条件下渗灌对其产量及水分生产率等的影响。结果表明,玉米渗灌毛管间距采用0.8~1.0 m较为适宜。毛管间距愈大,产量愈低;渗灌较地面灌平均减产7.23%,较旱地平均增产30.8%;玉米生长期内总耗水量地面灌最大,渗灌次之,旱地最小。渗灌可明显提高玉米水分生产率,渗灌处理水分生产率平均为1.65 kg/m3,是地面灌的1.2倍,是旱地的1.33倍;两年度渗灌较地面灌平均节水44.4%。     

聚丙烯装置聚合区设备布置及管廊的配管设计

结合工程特点及工艺流程,阐述了300 kt/a聚丙烯装置的布置原则,管道、管架设计要点和方法,以及需要注意的问题。     

射孔完井出砂预测新模型及其在射孔优化中的应用

分析了射孔完井的油层出砂机理，以线弹性理论为基础，考虑变温应力和流体渗流作用的拖曳力影响，建立了一种新的射孔完井出砂预测模型。应用该模型研究射孔方位、射孔参数对油井出砂的影响，结果表明，深穿透射孔弹比大孔半径射孔弹能更好地避免孔道出砂，采用定向射孔能有效地减少油井出砂问题。其结果对砂岩油藏射孔优化设计具有一定指导意义。     

国内外聚乙烯管材树脂生产现状

介绍国内外聚乙烯管材料的开发和生产情况,以及聚乙烯管材树脂的主要生产厂家。     

气动贯通式潜孔锤反循环连续取心取样钻进新技术在河南钼矿中应用

三道庄钼矿勘探生产中钻遇的地层非常复杂，采用其他方法均无法实现钻进，经过分析比较，决定采用贯通式潜孔锤反循环连续取心（样）钻进新技术来解决该地层钻进难、成孔难、取样难等三大问题。文章从钻进技术特点、工作原理等方面对该技术进行详细分析，得出其具有钻进效率高、岩心（样）采取率高、成孔质量好等突出优点。并介绍了钻进主要设备机具的配套、施工工序、操作要点、遇到问题的解决方法等。在河南钼矿露天采石场应用该项技术，有效避免了塌孔和卡钻事故，工程进展顺利、质量好、岩心（样）采取率在98％以上，取得了良好的效果。针对在生产试验中遇到的某些问题，作者提出了一些改进建议。     

川东气藏直井完井方法的产能预测研究

在分析研究目前国内外油气井完井产能计算经验公式的基础上，针对四川气田东部浅层气藏常用的几种完井方式，建立了该地区完井产能预测的数学模型。通过实例计算，表明了其数学模型的准确可行性，并为出砂井和非出砂井的完井方式提供了选择依据：对于出砂井，砾石裸眼充填完井方式是最理想的完井方式；对于非出砂井，实际裸眼完井的产能最大。得出的结论对该地区直井完井方式的选择，完井参数优化设计以及产能预测具有一定的指导作用。