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81.
82.
Dystrophin is a plasma membrane-associated cytoskeletal protein of the spectrin superfamily. The dystrophin cytoskeleton has been first characterized in muscle. Muscular 427 kDa dystrophin binds to subplasmalemmal actin filaments via its amino-terminal domain. The carboxy-terminus of dystrophin binds to a plasma membrane anchor, beta-dystroglycan, which is associated on the external side with the extracellular matrix receptor, alpha-dystroglycan, that binds to the basal lamina proteins laminin-1, laminin-2, and agrin. In the muscle, the dystroglycan complex is associated with the sarcoglycan complex that consists of several glycosylated, integral membrane proteins. The absence or functional deficiency of the dystrophin cytoskeleton is the cause of several types of muscular dystrophies including the lethal Duchenne muscular dystrophy (DMD), one of the most severe and most common genetic disorders of man. The dystrophin complex is believed to stabilize the plasma membrane during cycles of contraction and relaxation. Muscular dystrophin and several types of dystrophin variants are also present in extramuscular tissues, e.g. in distinct regions of the central nervous systems including the retina. Absence of dystrophin from these sites is believed to be responsible for some extramuscular symptoms of DMD, e.g. mental retardation and disturbances in retinal electrophysiology (reduced b-wave in electroretinograms). The reduced b-wave in electroretinograms indicated a disturbance of neurotransmission between photoreceptors and ON-bipolar cells. At least two different dystrophin variants are present in photoreceptor synaptic complexes. One of these dystrophins (Dp260) is virtually exclusively expressed in the retina. In the neuroretina, dystrophin is found in significant amounts in the invaginated photoreceptor synaptic complexes. At this location dystrophin colocalizes with dystroglycan. Agrin, an extracellular ligand of alpha-dystroglycan, is also present at this location whereas the proteins of the sarcoglycan complex appear to be absent in photoreceptor synaptic complexes. Dystrophin and dystroglycan are located distal from the ribbon-containing active synaptic zones where both proteins are restricted to the photoreceptor plasma membrane bordering on the lateral sides of the synaptic invagination. In addition, some neuronal profiles of the postsynaptic complex also contain dystrophin and beta-dystroglycan. These profiles appear to belong at least in part to projections of the photoreceptor terminals into the postsynaptic dendritic complex. In view of the abnormal neurotransmission between photoreceptors and ON-bipolar cells in DMD patients the dystrophin/beta-dystroglycan-containing projections of photoreceptor presynaptic terminals into the postsynaptic dendritic plexus might somehow modify the ON-bipolar pathway. Another retinal site associated with dystrophin/beta-dystropglycan is the plasma membrane of Müller cells where dystrophin/beta-dystroglycan appear to be present at particular high concentrations. At this location the dystrophin/dystroglycan complex may play a role in the attachment of the retina to the vitreous, and, under pathological conditions, in traction-induced retinal detachment. 相似文献
83.
步进式加热炉炉温优化设定模型及软件开发 总被引:1,自引:0,他引:1
针对线材厂步进式加热炉实际工况,提出了基于经验规则的炉温优化设定模型和在线自学习获取知识的思想,并以炉温优化设定模型为核心,开发出加热炉计算机控制系统的优化运行软件。 相似文献
84.
Low back pain (LBP), a common illness that may progress to chronic disability, costs many billions for care, lost work, and compensation. Conventional medicine does not effectively restore chronic LBP patients to work; multidisciplinary rehabilitation does, but limited or delayed access risks unnecessary costs, additional morbidity, and permanent disability. The authors examine costs of delayed treatment for 23 disabled LBP patients in a rehabilitation program. Compensation costs average $26,159 per patient, a sum covering treatment for 3 patients. Additional medical and societal costs are estimated. Factors causing delay, such as economic incentives and ignorance about pain, and policies to remediate these problems, are discussed. 相似文献
85.
化学结构式的拓扑描述 总被引:1,自引:0,他引:1
化学结构式的拓扑描述,是化学信息计算机化的手段之一。这里系统地建立了一整套的连接表(三类:紧缩连接表、冗余连接表、和连接方阵),用来进行结构式中原子之间连接关系的拓扑描述,对分子结构进行数值化。同时,阐述了连接表唯一化的方法,并介绍了不同类型连接表之间的转换关系和还原为平面结构式图形的绘图程序。 相似文献
86.
Microorganisms capable of degrading di-n-butyl phthalate (DBP) were isolated. The characteristics of DBP biodegradation by immobilized and free cells were investigated. The experimental results showed that the rate of DBP degradation of immobilized cells was higher than that of free cells. 相似文献
87.
88.
SynthesisandCrystalStructureofRE[CH2(CH2)4CONC4H9]3(NO3)3(RE=Dy,La)WangHanzhang(王汉章),XuQingfeng(徐庆锋),QianPu(钱朴)SunJianping(孙建... 相似文献
89.
Embedded deterministic test for low-cost manufacturing 总被引:1,自引:0,他引:1
Rajski J. Kassab M. Mukherjee N. Tamarapalli N. Tyszer J. Jun Qian 《Design & Test of Computers, IEEE》2003,20(5):58-66
You have probably heard that BIST takes too long and its fault coverage is low, and that deterministic test requires too many patterns. This article shows how on-chip compression and decompression techniques provide high fault coverage with low test times. 相似文献
90.