dystroglycan
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Dystroglycan was first identified in skeletal muscle and is a component of the dystrophin-glycoprotrein complex. It is composed of two nonconvalently linked alpha- and beta-subunits that are posttranslationnaly derived from a single gene (DAG1).
Dystroglycan is an adhesion molecule composed of two subunits, alpha and beta, that are produced by the post-translational cleavage of a single precursor molecule.
Dystroglycan is a pivotal component of the dystrophin-glycoprotein complex (DGC), which connects the extracellular matrix to the cytoskeleton in skeletal muscle and many other tissues.
Some muscular dystrophies are caused by mutations of DGC components, such as dystrophin, sarcoglycan or laminin-2, or also of DGC-associated molecules, such as caveolin-3.
Dystroglycan null mice
DG-null mice died during early embriogenesis and no neuromuscular diseases directly associated to genetic abnormalities of DG were identified so far.
Pathology
DGC-related neuromuscular disorders