Dystrophin-glycoprotein complex (DGC) (DGC)

The Dystrophin-glycoprotein complex (DGC) is a vital multi-protein assembly that spans the sarcolemma of skeletal and cardiac muscle cells (PMID: 25857461). It serves as a mechanical bridge connecting the internal actin cytoskeleton to the laminin in the extracellular matrix, thereby protecting the muscle cell membrane from the physical stress of contraction (PMID: 30635514). The complex is composed of several subunits, including dystrophin, dystroglycans, sarcoglycans, sarcospan, syntrophins, and dystrobrevins. Deficiencies or mutations in any of these components, particularly dystrophin, disrupt the entire complex, leading to membrane fragility, chronic inflammation, and progressive muscle wasting, as seen in Duchenne and Becker muscular dystrophies (UniProt: P11532). Modern therapeutic interventions aim to restore the functional assembly of the DGC by promoting the production of truncated but functional dystrophin or by delivering synthetic versions of the gene (FDA: Elevidys). These treatments are designed to stabilize the muscle fiber membrane and slow the clinical decline in patients with these debilitating neuromuscular disorders.

Other names

Dystrophin-associated glycoprotein complexDAGCDystrophin-associated protein complexDAPCDystrophin-glycoprotein complex components

Restoration of dystrophin protein expression through exon skipping, nonsense mutation suppression, or gene replacement to stabilize the sarcolemma and maintain the structural integrity of the dystrophin-glycoprotein complex.