全部产品分类
品牌 |
Leading Biology | 货号 |
APR11519G |
产品分类 |
Monoclonal Antibodies | 研究领域 |
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产品概述 |
We constantly strive to ensure we provide our customers with the best antibodies. As a result of this work we offer this antibody in purified format.
We are in the process of updating our datasheets. If you have any questions regarding this update, please feel free to contact our technical support team.
This product is a high quality Beta-Dystroglycan antibody.
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分子量 |
97441 Da
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细胞定位 |
Antigen Cellular Localization:
Alpha-dystroglycan: Secreted, extracellular space
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宿主 |
Rabbit
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种属反应性 |
Human
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靶点 |
A synthetic peptide corresponding o residues near the C-terminus of human Beta-Dystroglycan was used as an immunogen.
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克隆号 |
EP2200Y
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基因ID |
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UniProt ID |
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功能 |
The dystroglycan complex is involved in a number of processes including laminin and basement membrane assembly, sarcolemmal stability, cell survival, peripheral nerve myelination, nodal structure, cell migration, and epithelial polarization. Beta-dystroglycan is a transmembrane protein that plays important roles in connecting the extracellular matrix to the cytoskeleton. Acts as a cell adhesion receptor in both muscle and non-muscle tissues. Receptor for both DMD and UTRN and, through these interactions, scaffolds axin to the cytoskeleton. Also functions in cell adhesion-mediated signaling and implicated in cell polarity.
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总结 |
The dystroglycan complex is a membrane-spanning complex composed of two subunits, alpha- and beta-dystroglycan. Alpha-dystroglycan is a cell surface peripheral membrane protein which binds to the extracellular matrix (ECM), whereas beta-dystroglycan is an integral membrane protein which anchors alpha-dystroglycan to the cell membrane. The dystroglycan complex provides a tight link between the ECM and cell membrane. Dysfunction of the dystroglycan complex has commonly been implicated in the molecular pathogenesis of severe forms of hereditary neuromuscular diseases, including Duchenne muscular dystrophy, Fukuyama-type congenital muscular dystrophy and sarcoglycanopathy (1). Human dystroglycan is expressed in a variety of fetal and adult tissues. Data suggest that muscle and non-muscle isoforms of dystroglycan differ by carbohydrate moieties but not protein sequence. Therefore variable glycosylation of the conserved protein core might modulate laminin binding (2). beta-dystroglycan has been localized to microvilli structures in a number of cell types where it associates with the cytoskeletal adaptor ezrin, through which it is able to modulate the actin cytoskeleton and induce peripheral filopodia and microvilli (3).
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形式 |
50 mM Tris-Glycine (pH 7.4), 0.15 M NaCl, 40% Glycerol, 0.01% sodium azide and 0.05% BSA.
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储存条件 |
Store at +4°C short term. For long-term storage, aliquot and store at -20°C or below. Stable for 12 months at -20°C. Avoid repeated freeze-thaw cycles.
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应用 |
WB
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稀释方法 |
WB~~1:1000
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别名 |
Dystroglycan, Dystrophin-associated glycoprotein 1, Alpha-dystroglycan, Alpha-DG, Beta-dystroglycan, Beta-DG, DAG1
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图像 |
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说明书 |
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数量 |
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