Dystroglycan is a laminin binding component of thendystrophin-glycoprotein complex which provides a linkage betweennthe subsarcolemmal cytoskeleton and the extracellular matrix.nDystroglycan 1 is a candidate gene for the site of the mutation innautosomal recessive muscular dystrophies. The dramatic reduction ofndystroglycan 1 in Duchenne muscular dystrophy leads to a loss ofnlinkage between the sarcolemma and extracellular matrix, renderingnmuscle fibers more susceptible to necrosis. Dystroglycan alsonfunctions as dual receptor for agrin and laminin-2 in the Schwannncell membrane. The muscle and nonmuscle isoforms of dystroglycanndiffer by carbohydrate moieties but not protein sequence.nAlternative splicing results in multiple transcript variants allnencoding the same protein.
应用类型
WB
免疫原
This DAG1 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 718-747 amino acids from the C-terminal region of human DAG1.
