> Antigen, Antibodies, ELISA, Western Blot > Primary Antibody > Monoclonal Antibodies > Beta-Dystroglycan AntibodyBrand |
Leading Biology | Catalog Number |
APR11519G |
Product Type |
Monoclonal Antibodies | Field of Research |
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Product Overview |
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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Molecular Weight |
97441 Da
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Cellular Localization |
Antigen Cellular Localization:
Alpha-dystroglycan: Secreted, extracellular space
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Host |
Rabbit
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Species Reactivity |
Human
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Target |
A synthetic peptide corresponding o residues near the C-terminus of human Beta-Dystroglycan was used as an immunogen.
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Clone |
EP2200Y
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GeneID |
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UniProt ID |
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Function |
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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Summary |
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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Form |
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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Storage & Stability |
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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Applications |
WB
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Dilution |
WB~~1:1000
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Synonyms |
Dystroglycan, Dystrophin-associated glycoprotein 1, Alpha-dystroglycan, Alpha-DG, Beta-dystroglycan, Beta-DG, DAG1
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Images |
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Specification |
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Quantity |
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Leading Biology Inc.
2600 Hilltop DR, Building G, B Suite C138
Richmond, CA, 94806
Tel: 1-661-524(LBI)-0262
Email: info@leadingbiology.com
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