Dystrophin Associated Glycoprotein 1 (DAG1) ELISA Kit for Pigeon

Dystroglycan is a laminin binding component of the dystrophin-glycoprotein complex which provides a linkage between the subsarcolemmal cytoskeleton and the extracellular matrix. Dystroglycan 1 is a candidate gene for the site of the mutation in autosomal recessive muscular dystrophies. The dramatic reduction of dystroglycan 1 in Duchenne muscular dystrophy leads to a loss of linkage between the sarcolemma and extracellular matrix, rendering muscle fibers more susceptible to necrosis. Dystroglycan also functions as dual receptor for agrin and laminin-2 in the Schwann cell membrane. The muscle and nonmuscle isoforms of dystroglycan differ by carbohydrate moieties but not protein sequence. Alternative splicing results in multiple transcript variants all encoding the same protein.[provided by RefSeq, Apr 2010]

DAG1: a cytoskeletal protein that functions as a laminin receptor. Provides a linkage between the subsarcolemmal cytoskeleton and the extracellular matrix. May be involved in autosomal recessive muscular dystrophies. Its dramatic reduction in Duchenne muscular dystrophy leads to a loss of linkage between the sarcolemma and extracellular matrix, rendering muscle fibers more susceptible to necrosis. Dystroglycan also functions as dual receptor for agrin and laminin-2 in the Schwann cell membrane. Binds to several types of arenaviruses. Is a target for the entry of Mycobacterium leprae into peripheral nerve Schwann cells. The muscle and nonmuscle isoforms of dystroglycan differ by carbohydrate moieties but not protein sequence.

Protein type: Membrane protein, integral; Motility/polarity/chemotaxis; Cytoskeletal

Chromosomal Location of Human Ortholog: 3p21

Cellular Component: dystroglycan complex; extracellular space; focal adhesion; costamere; basolateral plasma membrane; extracellular region; integral to membrane; lipid raft; dystrophin-associated glycoprotein complex; nucleoplasm; postsynaptic membrane; cytoskeleton; cell-cell adherens junction; lamellipodium; cytoplasm; plasma membrane; basement membrane; sarcolemma; filopodium

Molecular Function: viral receptor activity; laminin-1 binding; tubulin binding; protein binding; structural constituent of muscle; protein complex binding; SH2 domain binding; calcium ion binding; alpha-actinin binding; actin binding; vinculin binding

Biological Process: nerve maturation; response to peptide hormone stimulus; extracellular matrix organization and biogenesis; entry of virus into host cell; negative regulation of protein kinase B signaling cascade; negative regulation of MAPKKK cascade; cytoskeletal anchoring; membrane protein ectodomain proteolysis; NLS-bearing substrate import into nucleus; myelination in the peripheral nervous system; virus-host interaction; morphogenesis of an epithelial sheet; calcium-dependent cell-matrix adhesion; negative regulation of cell migration

Disease: Muscular Dystrophy-dystroglycanopathy (limb-girdle), Type C, 9; Muscular Dystrophy-dystroglycanopathy (congenital With Brain And Eye Anomalies), Type A, 9