Aliases

• 156DAG
• A3a
• AGRNR
• DAG
• DAG1
• alpha-dystroglycan
• beta-dystroglycan
• dystroglycan 1
• dystroglycan 1 (dystrophin-associated glycoprotein 1)
• dystrophin-associated glycoprotein-1

Description

The dystroglycan complex is involved in a number of processes including laminin and basement membrane assembly, sacrolemmal stability, cell survival, peripheral nerve myelination, nodal structure, cell migration, and epithelial polarization. DAG1 is the laminin binding component of the dystrophin-glycoprotein complex which provides a linkage between the subsarcolemmal cytoskeleton and the extracellular matrix. The DAG1 gene 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. DAG1 is expressed in a variety of fetal and adult tissues.

Attributes

QA State

Under Review

Type

Protein

HGNC Name

DAG1