Structural Abnormalities of the Optic Nerve and Retina in Huntington’s Disease Pre-Clinical and Clinical Settings

Huntington’s disease (HD) is a fatal neurodegenerative disorder caused by a polyglutamine expansion in the huntingtin protein. HD-related pathological remodelling has been reported in HD mouse models and HD carriers. In this study, we studied structural abnormalities in the optic nerve by employing Spectral Domain Optical Coherence Tomography (SD-OCT) in pre-symptomatic HD carriers of Caucasian origin. Transmission Electron Microscopy (TEM) was used to investigate ultrastructural changes in the optic nerve of the well-established R6/2 mouse model at the symptomatic stage of the disease. We found that pre-symptomatic HD carriers displayed a significant reduction in the retinal nerve fibre layer (RNFL) thickness, including specific quadrants: superior, inferior and temporal, but not nasal. There were no other significant irregularities in the GCC layer, at the macula level and in the optic disc morphology. The ultrastructural analysis of the optic nerve in R6/2 mice revealed a significant thinning of the myelin sheaths, with a lamellar separation of the myelin, and a presence of myelonoid bodies. We also found a significant reduction in the thickness of myelin sheaths in peripheral nerves within the choroids area. Those ultrastructural abnormalities were also observed in HD photoreceptor cells that contained severely damaged membrane disks, with evident vacuolisation and swelling. Moreover, the outer segment of retinal layers showed a progressive disintegration. Our study explored structural changes of the optic nerve in pre- and clinical settings and opens new avenues for the potential development of biomarkers that would be of great interest in HD gene therapies.     

一种新型的机液集成变速器

提出了一种新型的机液集成变速器,介绍了其结构、工作原理和结构特点,总参数选择及系统设计方法,可指导新产品的研究开发,满足众多领域对低转速、大扭矩、高效率、低能耗变速器的需要.     

机车牵引通风机传动方式选型的可行性探讨

为适应铁路机车现代化的发展,提高机车运用率,简要介绍了机车牵引通风机传动方式选型的可行性。为以后装车运用提供了可靠依据。     

传动带成型机张力控制系统的优化设计

通过对传动带成型机张力控制系统的控制方案进行比较与分析,优化设计了以单片机为核心、以磁粉制动器为执行元件的恒张力控制系统。该张力控制系统采用智能调节,能进行参数修改、调节变频器加减速曲线及时间常数,可防止卷绕物拉断,具有在线监视、通讯功能,系统操作方便、直观,排线间距控制精度较高,能大幅度提高传动带成品率,具有推广应用价值。     

Development and Use of a Kinetical and Real-Time Monitoring System to Analyze the Replication of Hepatitis C Virus

In microbiological research, it is important to understand the time course of each step in a pathogen’s lifecycle and changes in the host cell environment induced by infection. This study is the first to develop a real-time monitoring system that kinetically detects luminescence reporter activity over time without sampling cells or culture supernatants for analyzing the virus replication. Subgenomic replicon experiments with hepatitis C virus (HCV) showed that transient translation and genome replication can be detected separately, with the first peak of translation observed at 3–4 h and replication beginning around 20 h after viral RNA introduction into cells. From the bioluminescence data set measured every 30 min (48 measurements per day), the initial rates of translation and replication were calculated, and their capacity levels were expressed as the sums of the measured signals in each process, which correspond to the areas on the kinetics graphs. The comparison of various HuH-7-derived cell lines showed that the bioluminescence profile differs among cell lines, suggesting that both translation and replication capacities potentially influence differences in HCV susceptibility. The effects of RNA mutations within the 5′ UTR of the replicon on viral translation and replication were further analyzed in the system developed, confirming that mutations to the miR-122 binding sites primarily reduce replication activity rather than translation. The newly developed real-time monitoring system should be applied to the studies of various viruses and contribute to the analysis of transitions and progression of each process of their life cycle.     

Mitigation of Damages to Electric Utilities Due to High Winds

Extreme winds, hurricanes, and tornadoes produce significant damage to electrical transmission and distribution line structures. The damage produces loss of power to the affected communities for a variable length of time, depending on severity of damage. Most institutions whose operations would be life threatening in the event of power failure have emergency power. However, loss of power to both residences and businesses can have economic impact on the community in the form of temporary housing shortages and loss of business. Utility companies seem to cope with wind damage fairly well, following risk management procedures in designing for wind and in providing alternate routing for distribution lines. However, continuing research and developments are needed to keep abreast of the problem.     

抛物线型有损传输线频域灵敏度分析的伴随方程法

提出了一种求解含抛物线型有损非均匀线网络频域灵敏度的方法。该方法首先将抛物线型有损非均匀线用科罗达(Kuroda)等效方法变换成有损均匀线及若干集中元件组成的等效电路,然后借助伴随网络方程法求出抛物线型有损非均匀传输线等参数的频域灵敏度。给出了计算实例,并通过扰动法进行了验证。     

互联网远程视频监控系统传输与控制分析

简述了远程监控系统的发展和技术要求,并基于目前流行的H.264编码格式和RTP协议探讨了远程监控的网络管理模块的视频传输与控制设计思路和方案.     

A virtual plant modeller (VPMOD) for batch-chemical processes

This paper presents a virtual plant modeller (VPMOD), which formally characterizes and integrates chemical product designs, batch-chemical equipment (plants), the real-time scheduling of chemical batches, and the control of chemical transport through the plant. These models provide a framework for agile batch-chemical manufacturing that has the ability to reroute and control chemical product flow automatically in a flexible plant subject to unexpected events, such as changes in demand patterns and equipment failure. A formal logic model is generated to control the actual system events, which are non-deterministic. A simulation environment in VPMOD is used to validate schedules and control logic based on plant models supplied by industry. The formal models have been implemented in an object-oriented language.     

e-Automation,an architecture for distributed industrial automation systems

Due to the complexity of modern industrial systems, a conventional automation system is not capable of providing sufficient information management and high-level intelligent approaches, as achieving these functionalities requires the support of comprehensive data management and coordination between system devices and heterogenous information. This paper proposes the concept of e-Automation, in which computer networking and distributed intelligence agent technologies are applied to industrial automation systems, and presents a hardware and software architecture that implements this concept. An open infrastructure based on multi-agent systems is employed in the proposed architecture of e-Automation, which aims to allow the implementation of diverse tasks and to permit greater configurability than can be obtained from a traditional system. To evaluate our proposed e-Automation concept, this paper presents a case study of substation information management which adopts the proposed e-Automation architecture in power system domain.