Hemi- and Homozygous Loss-of-Function Mutations in DSG2 (Desmoglein-2) Cause Recessive Arrhythmogenic Cardiomyopathy with an Early Onset

About 50% of patients with arrhythmogenic cardiomyopathy (ACM) carry a pathogenic or likely pathogenic mutation in the desmosomal genes. However, there is a significant number of patients without positive familial anamnesis. Therefore, the molecular reasons for ACM in these patients are frequently unknown and a genetic contribution might be underestimated. Here, we used a next-generation sequencing (NGS) approach and in addition single nucleotide polymor-phism (SNP) arrays for the genetic analysis of two independent index patients without familial medical history. Of note, this genetic strategy revealed a homozygous splice site mutation (DSG2–c.378+1G>T) in the first patient and a nonsense mutation (DSG2–p.L772X) in combination with a large deletion in DSG2 in the second one. In conclusion, a recessive inheritance pattern is likely for both cases, which might contribute to the hidden medical history in both families. This is the first report about these novel loss-of-function mutations in DSG2 that have not been previously identi-fied. Therefore, we suggest performing deep genetic analyses using NGS in combination with SNP arrays also for ACM index patients without obvious familial medical history. In the future, this finding might has relevance for the genetic counseling of similar cases.     

处理高炉渣的先进方法--干式成粒法

介绍了先进的高炉渣处理技术——干式成粒法，结合湘潭钢铁公司炼铁工序的实际情况，分析采用干式成粒法所带来的显著的经济效益。     

Availability analysis for the design of distributed processing networks

This paper presents an analytical model for evaluating the availability of distributed processing systems. The model, which is based on a well-known probability formula, has been implemented in FORTRAN and used for the design of a real-time distributed processing system that requires high availability. It is applied to a sample distributed processing system to illustrate its usefulness and to provide good insight into the design of highly available distributed processing systems. Effects of homogeneous/heterogeneous clustering and standby spare are illustrated. The concept of availability threshold where the distributed system may gain or lose availability over the individual processor availability is introduced. It has proven to be a valuable tool for the tradeoffs in the allocation of system requirements. The concluding remarks summarize the lessons learned and suggest cost-effective ways to achieve higher availability for the design of distributed processing systems.     

循环模式DSG槽式太阳能集热器出口蒸汽温度控制策略研究

循环模式直接蒸汽发电(direct steam generation,DSG)强度变化的影响很大,并具有大滞后、大惯性、参数时变等特点。针对该控制对象,提出采用多模型切换受限增量广义预测控制策略,建立典型工况下的子模型并设计相应的控制器。按照跟踪实际工况的运行结果,根据模糊推理的隶属度概念评判子模型的匹配程度,选择适配的子模型和控制器整定参数,并采用输入增量加权控制律和等待周期法实现平滑切换和无扰切换。仿真分析表明,提出的控制策略与常规PI控制方法相比,具有响应速度快、无稳态偏差、系统输出波动小、降低了模型失配影响等优点。仿真结果验证了该控制模型和策略的可行性和有效性。     

DSG变速器换挡系统的结构与工作原理分析

对DSG变速器换挡控制与液压操纵系统的结构进行了研究,并通过绘制出的DSG双离合变速器电子控制与操纵系统组成框图,以及挡位选择器换挡装置、换挡拨叉位置精确度控制装置及高速开关电磁阀的结构图,介绍了DSG变速器换挡控制方法,分析了DSG变速器的电控系统和液压操纵系统的工作原理.     

DSG RealSync复制软件在青海电力营销业务系统中的应用

文章运用比较法从多角度对DSG RealSync、Quest Shareplex、Oracle DataCuard 3种复制软件进行了分析,从原理上对DSG RealSync复制软件进行了阐述,同时介绍了DSG RealSync在青海电力营销业务系统中的应用.     

电励磁双凸极发电机分裂电容全桥整流拓扑分析

以12/8极电励磁双凸极发电机(DSEG)为研究对象,针对DSG2发电方式,提出分裂电容全桥整流方式,即发电机三相绕组中点接分裂电容式整流.借助有限元计算分析电容的不同接法对发电机输出特性的影响.研究结果表明,合理的选择容值,分裂电容全桥整流方式能提高换相后相绕组电流的上升率和有效值,使得一周期内的磁场储能得到提高,从而提高发电机的输出功率;傅里叶分析结果表明,分裂电容全桥整流方式对相电压和相电流的相位关系有一定的改善作用.原理样机的实验结果验证了此方式的有效性.     

Receiver behavior in direct steam generation with parabolic troughs

The receiver behavior of parabolic troughs in direct steam generation (DSG) has been studied under different experimental conditions. When cool water was fed into steel receiver pipes (Ø = 2.54 cm), the pipes deflected. This bend advanced like a wave from the inlet to the outlet end, having a deflection of about 6.5 cm at the center of each section (2.90 m long) in the parabolic trough module (14.5 m long and 2.5 m aperture). This phenomenon was due to a temperature difference of about 50°C in the circumferential direction. When the steel receiver pipe was changed to a copper receiver, the circumferential temperature difference was practically eliminated and no appreciable bending of the pipe was observed, hence DSG was obtained without these problems.     

注入模式下DSG系统反馈线性化串级控制器设计

本文是注入模式下DSG槽式太阳能热发电系统蒸汽温度控制器的研究,在已有太阳能集热器动态方程基础上,先建立太阳能阵列动态方程,然后介绍反馈线性化控制算法,并以PI控制为辅设计反馈线性化串级控制器,再在和利时公司DCS软硬件平台上编程实现并仿真运行,最后对仿真曲线分析得出结论:反馈线性化串级控制器可控制注入模式运行的DSG槽式系统太阳能阵列与太阳能集热器出口蒸汽温度,满足稳定与准确性目标。     

牛奶纤维热学和力学性质研究

利用DTG和DSC技术对牛奶纤维的热学性质进行了测试和分析。测试结果显示,牛奶纤维吸湿性不高,热裂解从262.7℃开始,到317.6℃裂解达到高峰;拉伸实验表明,牛奶纤维的湿拉伸断裂强力和断裂伸长均较干态时为低。