|Other Names||Dystroglycan, Dystrophin-associated glycoprotein 1, Alpha-dystroglycan, Alpha-DG, Beta-dystroglycan, Beta-DG, DAG1|
|Target/Specificity||The synthetic peptide sequence used to generate the antibody AP14101b was selected from the C-term region of DAG1. A 10 to 100 fold molar excess to antibody is recommended. Precise conditions should be optimized for a particular assay.|
|Format||Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.|
|Storage||Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C.|
|Precautions||This product is for research use only. Not for use in diagnostic or therapeutic procedures.|
|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.|
|Cellular Location||Alpha-dystroglycan: Secreted, extracellular space|
|Tissue Location||Expressed in a variety of fetal and adult tissues. In epidermal tissue, located to the basement membrane Also expressed in keratinocytes and fibroblasts|
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Dystroglycan is a laminin binding component of thedystrophin-glycoprotein complex which provides a linkage betweenthe subsarcolemmal cytoskeleton and the extracellular matrix.Dystroglycan 1 is a candidate gene for the site of the mutation inautosomal recessive muscular dystrophies. The dramatic reduction ofdystroglycan 1 in Duchenne muscular dystrophy leads to a loss oflinkage between the sarcolemma and extracellular matrix, renderingmuscle fibers more susceptible to necrosis. Dystroglycan alsofunctions as dual receptor for agrin and laminin-2 in the Schwanncell membrane. The muscle and nonmuscle isoforms of dystroglycandiffer by carbohydrate moieties but not protein sequence.Alternative splicing results in multiple transcript variants allencoding the same protein.
Bailey, S.D., et al. Diabetes Care 33(10):2250-2253(2010)Nilsson, J., et al. Glycobiology 20(9):1160-1169(2010)Lara-Chacon, B., et al. J. Cell. Biochem. 110(3):706-717(2010)Sgambato, A., et al. Pathology 42(3):248-254(2010)Masaki, T., et al. J. Biomed. Biotechnol. 2010, 740403 (2010) :
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