Page last updated: 2024-10-24

dystrophin-associated glycoprotein complex

Definition

Target type: cellularcomponent

A multiprotein complex that forms a strong mechanical link between the cytoskeleton and extracellular matrix; typical of, but not confined to, muscle cells. The complex is composed of transmembrane, cytoplasmic, and extracellular proteins, including dystrophin, sarcoglycans, dystroglycan, dystrobrevins, syntrophins, sarcospan, caveolin-3, and NO synthase. [PMID:15117830, PMID:16710609]

The dystrophin-associated glycoprotein complex (DGC) is a multiprotein complex that links the cytoskeleton of muscle fibers to the extracellular matrix. It plays a crucial role in maintaining muscle cell integrity and function. The cellular component of the DGC is localized to the sarcolemma, the plasma membrane of muscle cells. It forms a complex network of proteins that spans the entire muscle fiber, providing structural support and mediating signaling pathways. Key components of the DGC include dystrophin, a large scaffolding protein that binds to actin filaments in the cytoskeleton, and a series of transmembrane proteins, such as alpha- and beta-dystroglycan, sarcoglycans, and dystrobrevin. These transmembrane proteins connect dystrophin to the extracellular matrix through laminin, a protein that is found in the basement membrane surrounding muscle fibers. The DGC also interacts with other proteins involved in muscle function, such as neuronal nitric oxide synthase (nNOS), which is involved in the regulation of muscle blood flow. The DGC is a complex and dynamic structure that undergoes constant remodeling and reorganization in response to changes in muscle activity and stress. Mutations in genes encoding DGC components can lead to various muscular dystrophies, characterized by progressive muscle weakness and degeneration.'
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Proteins (1)

ProteinDefinitionTaxonomy
UtrophinA utrophin that is encoded in the genome of human. [PRO:DNx, UniProtKB:P46939]Homo sapiens (human)

Compounds (1)

CompoundDefinitionClassesRoles
5-(ethylsulfonyl)-2-(naphthalen-2-yl)benzo(d)oxazoleezutromid: a utrophin upregulator; structure in first source