Critical Evaluation of Current Hypotheses for the Pathogenesis of Hypertrophic Cardiomyopathy

Hereditary hypertrophic cardiomyopathy (HCM), due to mutations in sarcomere proteins, occurs in more than 1/500 individuals and is the leading cause of sudden cardiac death in young people. The clinical course exhibits appreciable variability. However, typically, heart morphology and function are normal at birth, with pathological remodeling developing over years to decades, leading to a phenotype characterized by asymmetric ventricular hypertrophy, scattered fibrosis and myofibrillar/cellular disarray with ultimate mechanical heart failure and/or severe arrhythmias. The identity of the primary mutation-induced changes in sarcomere function and how they trigger debilitating remodeling are poorly understood. Support for the importance of mutation-induced hypercontractility, e.g., increased calcium sensitivity and/or increased power output, has been strengthened in recent years. However, other ideas that mutation-induced hypocontractility or non-uniformities with contractile instabilities, instead, constitute primary triggers cannot yet be discarded. Here, we review evidence for and criticism against the mentioned hypotheses. In this process, we find support for previous ideas that inefficient energy usage and a blunted Frank–Starling mechanism have central roles in pathogenesis, although presumably representing effects secondary to the primary mutation-induced changes. While first trying to reconcile apparently diverging evidence for the different hypotheses in one unified model, we also identify key remaining questions and suggest how experimental systems that are built around isolated primarily expressed proteins could be useful.     

非均匀软岩蠕变机理分析

为了对工程围岩的蠕变现象进行解释,探明软岩的蠕变本质,根据岩石压缩过程中变形、弱化本构关系以及变形与承载能力的关系,采用岩石最弱断面并联微元体模型,对非均匀软岩的衰减蠕变和非衰减蠕变机理进行了分析,研究表明,非均匀性岩石内部各点承载能力不同使得变形后岩石内部各点屈服弱化不同步,又由于岩石晶体内部空位或杂质的扩散,二者共同作用导致岩石蠕变．通过对截面积相同,高径比分别为1．5和2．5的页岩岩样进行蠕变试验,验证了岩石的非均匀性是造成岩石蠕变的一个不可忽视的重要因素．     

Using brazing alloys in resistance spot welding of joints in aluminium–magnesium alloys

The results of mathematical modelling of the process of propagation of heat in the parent metal in submerged-arc surfacing with a composite strip electrode are presented. The mathematical model was constructed by solving the differential equation of heat conductivity by the finite element method. The calculations and plotting of the graphs were carried out using the MSC Patran software. The model was used to determine the main parameters of the composite strip electrode influencing the shape and dimensions of the pool and the non-uniformity of penetration. The comparison of the calculated and experimental data shows that they are in good agreement.     

双掺杂LiNbO3晶体中光栅均匀性的优化研究

研究了非挥发全息记录中南于紫外光的强吸收而引起的光栅非均匀性，分析了这种非均匀性对光栅衍射效率的影响。结果显示，非均匀性致使光折变光栅的平均强度减弱，衍射效率降低。提出了采用两束等光强的敏化紫外光由晶体两侧入射的优化方案以改善光栅的均匀性，提高光栅的衍射效率。通过联立两中心带输运物质方程和双光束耦合波方程，进行了相应的理论模拟，并给出实验验证。结果表明双侧紫外光照射能够实现均匀性较好的光栅，是提高衍射效率的有效途径之一。     

基于NIOS II的高速实时非均匀性校正

摘要：实时非均匀性校正是红外应用领域的关键技术之一。针对目前公开发表的论文中，非均匀性校正模块化设计程度不高和利用现场可编程门阵列(FPGA)进行校正，速度设计探讨不够深入等问题，展开了深入的研究，最后巧妙地引入NIOS II软核微处理器解决了模块化设计问题，并对速度设计进行了深入的探讨。经过优化设计，校正模块能以111．51MHz的高速实时地进行校正，具有很大的工程应用价值。     

面向风电消纳的车-储-热协同调度策略

良好的需求侧管理能够有效应对风电出力的不确定性，提升风电消纳能力。然而，传统的需求侧管理机制，受参与元素单一的限制，严重影响了资源的利用效率。因此，文章提出一种面向风电消纳的车-储-热协同调度策略。首先，建立了需求侧用户热负荷非均匀特性模型，并将具有差异性的热负荷视作虚拟储热；其次，基于电动汽车的储能特性，建立了电动汽车充放电模型；随后，综合考虑用户扰动成本和电池折旧成本，建立了以风电消纳量最大为目标的调度策略；最后，在GAMS软件上进行算例分析，并与未计及电动汽车参与的调度策略进行对比。结果表明：通过车-储-热协同调度策略，风电消纳量增大了25.42%，投入产出比减小了10.3%。     

红外焦平面阵列非均匀性校正算法研究

红外焦平面阵列( IRFPA)的非均匀性校正是提升红外系统成像质量的重要环节。非均匀性校正算法主要分为基于参考辐射源的标定类校正算法和基于场景的校正算法。将基于参考辐射源的标定类校正算法按线性模型与非线性模型进行详细分类,基于场景的校正算法按空域和时域进行详细分类。针对不同类特点,分别对上述算法进行了详细的分析及比较,为今后非均匀性校正算法的选择提供理论基础,并根据现有两类算法特点,提出算法未来的研究方向。     

Stereo vision for robotic applications in the presence of non-ideal lighting conditions

Many robotic and machine-vision applications rely on the accurate results of stereo correspondence algorithms. However, difficult environmental conditions, such as differentiations in illumination depending on the viewpoint, heavily affect the stereo algorithms’ performance. This work proposes a new illumination-invariant dissimilarity measure in order to substitute the established intensity-based ones. The proposed measure can be adopted by almost any of the existing stereo algorithms, enhancing it with its robust features. The performance of the dissimilarity measure is validated through experimentation with a new adaptive support weight (ASW) stereo correspondence algorithm. Experimental results for a variety of lighting conditions are gathered and compared to those of intensity-based algorithms. The algorithm using the proposed dissimilarity measure outperforms all the other examined algorithms, exhibiting tolerance to illumination differentiations and robust behavior.     

大幅面太阳全光谱辐照系统的设计

大幅面太阳全光谱辐照系统是太阳模拟器的重要组成部分,辐照不均匀度是其关键技术指标之一,在传统全光谱辐照系统中存在着难以达到国家标准、光源光谱匹配性差等技术难题。结合大幅面太阳模拟器的研究现状,设计了一种全光谱辐照系统。利用光学仿真软件Zemax对所设计的全光谱辐照系统辐照均匀性进行仿真,提出了辐照度衰减模式的新概念,并以此为基础对全光谱辐照系统辐照不均匀度进行了优化。光源采用瑞士Solaronix公司生产的Plasma光源,经过测试光谱达到国际电工委员会标准A级,光源谱段内辐照度用光谱辐射计进行测试。结果表明,在辐照度为280 W/m²时,辐照度衰减模式在未启动和启动时辐照不均匀度分别为8.4%和5.7%,参考国家标准GB/T12637—1990太阳模拟器通用规范,在2 400 mm×6 000 mm有效辐照面内其辐照均匀度满足规范C级标准,接近B级标准。     

自动动态补偿功能改善酸蚀蚀刻不均匀性问题

在酸蚀制程中,由于其蚀铜量较大,蚀刻线生产时蚀刻速度较慢,导致蚀刻不均匀性对线路制作存在很大影响,为改善蚀刻不均匀性的问题,采用奥宝公司专门开发的可制造性设计功能----自动动态补偿(Dynamic Etch Compensation),根据不同的间距设置不同的线宽补偿量,来确保蚀刻后线宽的一致性。