This webpage contains legacy information. The product is either no longer available from the supplier or has been delisted at Labome.
产品简要
公司名称 :
默克密理博中国
其他品牌 :
Oncogene Research Products, Calbiochem, Novagen, Merck, Upstate Biotechnology, Chemicon, LINCO, Novabiochem, Guava
产品类型 :
抗体
产品名称 :
α-Dystroglycan Antibody, clone VIA4-1
目录 :
05-298
规格 :
200微升
克隆性 :
单克隆
宿主 :
小鼠
共轭标签 :
未共轭
反应物种 :
人类, 小鼠
应用 :
免疫印迹, 免疫组化, 流式细胞仪, 免疫组化-石蜡切片, 免疫组化-冰冻切片
文章摘录数: 27
出版应用/物种/样本/稀释 | 参考文献 |
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Bangratz M, Sarrazin N, Devaux J, Zambroni D, Echaniz Laguna A, Rene F, et al. A mouse model of Schwartz-Jampel syndrome reveals myelinating Schwann cell dysfunction with persistent axonal depolarization in vitro and distal peripheral nerve hyperexcitability when perlecan is lacking. Am J Pathol. 2012;180:2040-55 pubmed 出版商
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Lathia J, Patton B, Eckley D, Magnus T, Mughal M, Sasaki T, et al. Patterns of laminins and integrins in the embryonic ventricular zone of the CNS. J Comp Neurol. 2007;505:630-43 pubmed
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Yang D, Bierman J, Tarumi Y, Zhong Y, Rangwala R, Proctor T, et al. Coordinate control of axon defasciculation and myelination by laminin-2 and -8. J Cell Biol. 2005;168:655-66 pubmed
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Sugita S, Saito F, Tang J, Satz J, Campbell K, Sudhof T. A stoichiometric complex of neurexins and dystroglycan in brain. J Cell Biol. 2001;154:435-45 pubmed
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产品信息
目录代码 :
05-298
亚类别 :
Cell Structure
产品名称 :
Anti-α-Dystroglycan Antibody, clone VIA4-1
产品类型 :
抗体
克隆性 :
单克隆抗体
基因编号 :
Q14118
宿主名称 :
小鼠
抗原 :
α-Dystroglycan
克隆 :
VIA4-1
共轭标签 :
Culture Supernatant
抗体亚型 :
IgG1
产品描述 :
Anti-α-Dystroglycan Antibody, clone VIA4-1
交叉活性 :
狗;豚鼠;人类;小鼠;大鼠;兔
背景 :
Dystroglycan is one of the dystrophin-associated glycoproteins, which is encoded by a 5.5 kb transcript in Homo sapiens by chromosome 3. There are two exons that are separated by a large intron. The spliced exons codes for a protein product is finally cleaved into two non-covalently associated subunits, α (N-terminal) and β (C-terminal). In skeletal muscle the dystroglycan complex works as a transmembrane linkage between the extracellular matrix and the cytoskeleton. α-dystroglycan is extracellular and binds to merosin (α-2 laminin) in the basement membrane, while β-dystroglycan is a transmembrane protein and binds to dystrophin, which is a large rod-like cytoskeletal protein, absent in Duchenne muscular dystrophy patients. Dystrophin binds to intracellular actin cables. In this way, the dystroglycan complex, which links the extracellular matrix to the intracellular actin cables, is thought to provide structural integrity in muscle tissues. The dystroglycan complex is also known to serve as an agrin receptor in muscle, where it may regulate agrin-induced acetylcholine receptor clustering at the neuromuscular junction. There is also evidence that suggests the function of dystroglycan as a part of the signal transduction pathway because it is shown that Grb2, a mediator of the Ras-related signal pathway, can interact with the cytoplasmic domain of dystroglycan. In general, aberrant expression of dystrophin-associated protein complex underlies the pathogenesis of Duchenne muscular dystrophy, Becker muscular dystrophy and severe childhood autosomal recessive muscular dystrophy. Interestingly, no genetic disease has been described for either α- or β-dystroglycan.
Dystroglycan is widely distributed in non-muscle tissues as well as in muscle tissues. During epithelial morphogenesis of kidney, the dystroglycan complex is shown to act as a receptor for the basement membrane. Dystroglycan expression in Mus musculus brain and neural retina has also been reported. However, the physiological role of dystroglycan in non-muscle tissues has remained unclear.
免疫原 :
Rabbit skeletal muscle membrane preparation
特异性 :
Recognizes α-dystroglycan/LARGE-glycan.
包装尺寸 :
200微升
用途 :
免疫组化;免疫印迹
储存 :
Maintain for 2 years at -20°C from date of shipment. Aliquot to avoid repeated freezing and thawing. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
公司信息
默克密理博中国
上海市浦东张江高科技园区晨晖路88号2号楼2楼
asiatechserv@millipore.com
http://www.millipore.com/china/wwcn/cnhome400-889-1988
公司总部: 美国
EMD Millipore is the Life Science division of Merck KGaA of Darmstadt, Germany
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