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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
74.
4,4'-二苯醚二甲酸(OBBA)是一种重要的液晶聚合物单体。以此作为改性组分,合成了一系列PET共聚酯,并用1H-NMR、FT-IR、GPC、二甘醇和端羧基测定等方法进行了表征,同时对改性单体的转化率和合成共聚酯的反应速率进行了测定,结果表明,所选择的合成方法可以获得较高的转化率;反应速率则随OBBA的增加而增大。 相似文献
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光催化氧化后四环素的分解率与COD去除率之间关系的探讨 总被引:1,自引:0,他引:1
以粉状无机半导体(ZnO)为催化剂,探讨了水溶液中四环素经光催化氧化分解率和COD去除率之间的关系。实验表明,在本实验中四环素的分解率和COD去除率之间有较好的正相关性。当反庆系统中有足量的活性催化剂和足够的光照时间时,绝大部分四环素将被较彻底地氧化分解成CO2,这不仅有助于光催化反应机理的研究,助于今后在环保中的实际应用。 相似文献
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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
79.
螺丝湾水电站作为云南省电力局( 集团公司) 、迪庆州、省投资公司三方合资建设的电站,是迪庆藏族自治州自建国以来投资最大的一个建设项目,它的建成,开创了滇西北水电资源多方合资开发的先例,有其特殊的经济和政治意义。本文结合螺丝湾水电站建设的实际情况,论述了在市场经济条件下做好螺丝湾电站建设中“三控制”的几项措施 相似文献
80.
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