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61.
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   
62.
We report three children with hemidystonia in whom anti-cardiolipin (aCL) antibodies were demonstrated. Systemic lupus erythematosus was excluded on the basis of both clinical and serological criteria, and the diagnosis of primary antiphospholipid syndrome (PAPS) was made. In two cases, aCL antibodies could be causally related to a presumed immune-mediated thrombotic event involving the basal ganglia as shown by magnetic resonance imaging (MRI). In the remaining patient the finding of white matter alteration on NMR might be due to cross-reactivity of anti-phospholipid (aPL) antibodies with cerebral phospholipids, resulting in demyelination. We suggest that PAPS must always be considered when isolated or recurrent focal cerebral ischaemia, and particularly hemidystonia, occur in childhood.  相似文献   
63.
介绍了可行性研究财务评价中利息计算的内容和方法。  相似文献   
64.
激光技术在医学应用的若干进展   总被引:2,自引:0,他引:2       下载免费PDF全文
钱焕文  单清 《激光技术》1994,18(5):317-320
本文评介激光医学机理、应用和进展,以及发展方向。  相似文献   
65.
罗松  毛谦 《光通信研究》1998,(6):12-15,20
本文根据ITU-T最新的Q.2971协议,以及笔者所从事的实际课题,提出一整套B-ISDN第三层UNI信令的软件实现策略,其特点是能同时满足用户对点到点以及点到多点广播这两种通信方式的要求,而且可以动态配置。  相似文献   
66.
An elastic half plane with an oblique edge crack is considered in this paper. A pair of concentrated forces or point dislocations is assumed to act at an arbitrary point in the half plane. The half plane with an edge crack is first mapped into a unit circle by a rational mapping function so that the following analysis can be carried out on the mapped plane analytically. Then the complex stress functions are derived by separating the whole problem into two parts; one is the principal part corresponding to the infinite plane acted on by concentrated forces or dislocations, the other is the holomorphic part, which can be determined by making use of the property of regularity of complex stress functions. The stress intensity factors of the crack can be calculated with different inclined angles of the crack, and the displacement and stress components at an arbitrary position in the half plane can be expressed explicitly.  相似文献   
67.
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   
68.
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   
69.
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   
70.
由电压型运算放大器组成的精密整流电路要严格地匹配电阻才能获得精密全波整流波形.本文给出一种具有CCⅡ电路特性由运算放大器AD844所构成的电流模精密整流电路.无需匹配电阻就可输出良好的精密全波整流波形.且具有输入电压范围大,频带宽等特点.  相似文献   
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