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91.
92.
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
93.
小秦岭地区金矿床严格受构造控制,各成矿阶段及矿物学特征明显,通过对矿物学特征的研究找出矿物学标志,认为今后找矿要注意各阶段石英的颜色、黄铁矿中金的含量以及矿石中银的含量,这些与金有密切关系. 相似文献
94.
模拟计算表明:采用活性趋于表层的分布,可以较明显地提高国产中温变换催化剂的内表面利用率,有效地节省催化剂用量,而对“热点”温度没有多大影响。 相似文献
95.
结合当前我国建材市场的形势 ,就建材市场“涨价风潮”的起因 ,从经济增长的结果、市场运行的规律等方面进行了论述 ,提出了涨价风潮对近期全国建材市场的影响 相似文献
96.
Preparation of nanosized titania powder via the controlled hydrolysis of titanium alkoxide 总被引:7,自引:0,他引:7
Jinyuan Chen Lian Gao Junghua Huang Dongsheng Yan 《Journal of Materials Science》1996,31(13):3497-3500
By controlling the hydrolysis of titanium butoxide, followed with or without an ethanol washing process, the preparation of nanoscale titania powder was studied in detail. The characteristics of different powders produced by the direct precipitation (without an ethanol wash) and ethanol wash processes were studied by X-ray diffraction, transmission electron microscopy, BET, thermogravimetry and differential thermal analysis techniques. By comparison, it was found that both the direct precipitation and ethanol wash methods can obtain slightly agglomerated nanoscale titania powders of less than 15 nm, but the ethanol wash can further reduce the agglomeration. The particle sizes of titania powders can be modified while still retaining the anatase structure. 相似文献
97.
Jian -Ping Wang He -Lie Luo Nai -Fei Gao Yuan -Yuan Liu 《Journal of Materials Science》1996,31(3):727-730
The Fe-Ag granular metal solid samples with 10% and 30% weight iron have been successfully fabricated using a sol-gel method, which are characterized by X-ray diffraction and transmission electron micrography. The average diameters of iron particles are from about a few nanometres to a few tens of nanometres controlled by the reducing temperature. The evolution of magnetic properties and microstructure during heat treating are described in detail and explained by using the superparamagnetism, single domain and multi domain theories. The magnetic anisotropy of the Fe-Ag granular solid is studied by using the law of approach to saturation. It is found that the magnetic anisotropy constant is in the order of 105 J m–3 which is higher than the value of the bulk iron and increases with the increase of reducing temperature. 相似文献
98.
99.
文章通过对洋浦电厂海水冷却箱涵沟槽预裂(深孔)爆破技术的研究,提出了复杂地质条件下合理的控制爆破参数及微差起爆方式。针对砂层地段难以钻、爆的特点。笔者进行了有益的探讨。 相似文献
100.