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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
24.
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
25.
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
26.
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
27.
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
28.
创业家与企业家的相关内涵思辨 总被引:2,自引:0,他引:2
学术界和实践界对entrepreneur和entrepreneurship的内涵界定不清晰,缺少概念基础,从而限制了《创业学》的发展。分析了创业与创新的本质;区别了活跃在企业创立和成长阶段、致力于创造生产函数的创业家和扮演“权威”、致力于改变函数自变量的企业家;界定了在不同语境中创业家与企业家的含义。最后,强调了需要根据上下文来判断它们的内涵。 相似文献
29.
介绍了一种在强γ辐射场中探测快中子的探测系统,利用实验室n-γ放射源,调试了系统n-γ甄别性能,确定了其最佳工艺状态。运用Monte-Carlo数值模拟,从理论上计算了模拟探测器的探测效率,并与实验测量结果进行了对比分析。 相似文献
30.
Sijin Qian 《Nuclear instruments & methods in physics research. Section A, Accelerators, spectrometers, detectors and associated equipment》1994,350(3):618-620
The fast vertex fitting method [P. Billoir and S. Qian, Nucl. Instr. and Meth. A 311 (1992) 139] has been enhanced by a steering procedure which performs the track selection to insure the vertex fitting quality. Meanwhile, a sign error in a formula in the above publication has been corrected. 相似文献