Anti -DAG1 (Dystroglycan, Dystrophin-associated Glycoprotein 1, Alpha-dystroglycan,
alpha-DG)
Pricing
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| Clone |
Host |
Grade |
Applications |
| Monoclonal |
Mouse |
Affinity Purified |
E B IH |
|
| Alpha-dystroglycan (alpha-DG), also known as dystrophin-associated glycoprotein, is a laminin-binding protein of ~156kD (including glyco-groups). Alpha-DG is a component of the dystroglycan complex, which is involved in early development, morphogenesis and in the pathogenesis of muscular dystrophies. Alpha- and beta-DG are encoded by a single gene and are derived from a precursor polypeptide by posttranslational cleavage. Beta-DG is an integral membrane protein, whereas alpha-DG is membrane- associated through its noncovalent interaction with the extracellular domain of beta-DG. The alpha- and beta-DGs provide important physical linkages between components of basement membranes and cytoplasmic proteins that bind to the actin cytoskeleton. Alpha-DG is a heavily glycosylated, mucin-like protein anchored on the extracellular surface of the myotube, where it may provide linkage between the sarcolemma and extracellular matrix (ECM). Alpha-DG is expressed in a variety of fetal and adult tissues. Tissue-specific glycosylation modifies the laminin specificity of alpha-DG. The muscle and nonmuscle isoforms of dystroglycan differ by carbohydrate moieties but not protein sequence. Alpha-DG has been shown to colocalize with laminin in skeletal and cardiac muscle and a number of other cells including peripheral nerve, astrocytes, Purkinje neurons and kidney epithelium. Laminin-10/11 was shown to bind preferentially to brain alpha-DG. In Duchenne muscular dystrophy, the expression of alpha-DG is dramatically reduced leading to a loss of linkage between the sarcolemma and extracellular matrix, rendering muscle fibers more susceptible to necrosis. In the central nervous system, dystroglycan functions as a dual receptor for agrin and laminin-2 for instance in the Schwann cell membrane. Furthermore, defects in dystroglycan are central to the pathogenesis of structural and functional brain abnormalities seen in congenital muscular dystrophies (CMD). | | | Catalog # | D9905-04D | | Applications | Suitable for use in Western Blot, ELISA and Immunohistochemistry. Other applications not tested. | | Recommended Dilution | Western Blot: 1:50 | | Immunohistochemistry: Paraffin | | Optimal dilutions to be determined by the researcher. | | Storage and Stability | May be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Aliquots are stable for at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. | | CAS Number | n/a | | Clone Type | Monoclonal | | Isotype | IgG2b | | Clone No | 13A44 | | Host | Mouse | | Source | Bovine | | Concentration | ~0.1mg/ml | | Form | Supplied as a liquid in PBS, 0.1% BSA, 0.02% sodium azide. | | Purity | Purified by Protein G affinity chromatography. | | Immunogen | Dystrophin-associated glycoprotein 1 | | Specificity | Recognizes a glycoepitope unique to bovine brain alpha-dystroglycan. Species Crossreactivity: human, mouse, rabbit and rat. | | | Important Note | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications without the expressed written authorization of United States Biological. |
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