AAA81779.1
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UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
97,441 Da
NCBI Official Full Name
dystroglycan
NCBI Official Synonym Full Names
dystroglycan 1 (dystrophin-associated glycoprotein 1)
NCBI Official Synonym Symbols
A3a; DAG; AGRNR; 156DAG; MDDGC7; MDDGC9 [Similar Products]
NCBI Protein Information
dystroglycan
UniProt Protein Name
Dystroglycan
UniProt Synonym Protein Names
Dystrophin-associated glycoprotein 1Cleaved into the following 2 chains:Alpha-dystroglycan; Alpha-DG; Beta-dystroglycan; Beta-DG
UniProt Synonym Gene Names
UniProt Entry Name
DAG1_HUMAN
NCBI Summary for DAG1
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]
UniProt Comments for DAG1
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: Cytoskeletal; Membrane protein, integral; Motility/polarity/chemotaxis
Chromosomal Location of Human Ortholog: 3p21
Cellular Component: dystroglycan complex; extracellular space; focal adhesion; costamere; basolateral plasma membrane; integral to membrane; extracellular region; lipid raft; nucleoplasm; dystrophin-associated glycoprotein complex; postsynaptic membrane; cell-cell adherens junction; cytoskeleton; lamellipodium; cytoplasm; plasma membrane; basement membrane; sarcolemma; filopodium
Molecular Function: laminin-1 binding; tubulin binding; viral receptor activity; protein binding; structural constituent of muscle; protein complex binding; SH2 domain binding; calcium ion binding; alpha-actinin binding; actin binding; vinculin binding
Biological Process: response to peptide hormone stimulus; nerve maturation; entry of virus into host cell; extracellular matrix organization and biogenesis; 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
Research Articles on DAG1
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