|Other Names||Alpha-sarcoglycan, Alpha-SG, 50 kDa dystrophin-associated glycoprotein, 50DAG, Adhalin, Dystroglycan-2, SGCA, ADL, DAG2|
|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||Component of the sarcoglycan complex, a subcomplex of the dystrophin-glycoprotein complex which forms a link between the F-actin cytoskeleton and the extracellular matrix.|
|Cellular Location||Cell membrane, sarcolemma; Single-pass type I membrane protein. Cytoplasm, cytoskeleton|
|Tissue Location||Most strongly expressed in skeletal muscle. Also expressed in cardiac muscle and, at much lower levels, in lung. In the fetus, most abundant in cardiac muscle and, at lower levels, in lung. Also detected in liver and kidney. Not expressed in brain|
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This gene encodes a component of thedystrophin-glycoprotein complex (DGC), which is critical to thestability of muscle fiber membranes and to the linking of the actincytoskeleton to the extracellular matrix. Its expression is thoughtto be restricted to striated muscle. Mutations in this gene resultin type 2D autosomal recessive limb-girdle muscular dystrophy.Multiple transcript variants encoding different isoforms have beenfound for this gene.
Mendell, J.R., et al. Ann. Neurol. 68(5):629-638(2010)Fernandez, K., et al. Am. J. Pathol. 176(1):416-434(2010)Klinge, L., et al. Neuromuscul. Disord. 18(12):934-941(2008)Delgado-Olguin, P., et al. Biochim. Biophys. Acta 1779(1):74-80(2008)Rafii, M.S., et al. J. Cell. Physiol. 209(2):439-447(2006)
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