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The molecular cross talk of the dystrophin–glycoprotein complex
Author(s) -
Gawor Marta,
Prószyński Tomasz J.
Publication year - 2018
Publication title -
annals of the new york academy of sciences
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.712
H-Index - 248
eISSN - 1749-6632
pISSN - 0077-8923
DOI - 10.1111/nyas.13500
Subject(s) - microbiology and biotechnology , duchenne muscular dystrophy , dystrophin , neuroscience , postsynaptic potential , neuromuscular junction , sarcomere , acetylcholine receptor , skeletal muscle , biology , neurotransmission , chemistry , myocyte , receptor , anatomy , biochemistry , genetics
Abstract The proper function of skeletal muscles relies on their ability to process signals derived from motor neurons, transmit stimuli along the muscle fibers, contract, and regenerate efficiently after injury. The dystrophin–glycoprotein complex (DGC; also called the dystrophin‐associated protein complex) plays a central role in all of these processes. It acts as a transmembrane platform that anchors the extracellular matrix (ECM) to the intracellular cytoskeleton and makes muscle fibers more resistant to injury. The DGC also contributes to the transmission of contraction‐evoked force from the sarcomere to the ECM. The dysfunction of DGC‐associated proteins can lead to myopathies, including Duchenne's muscular dystrophy, manifested by progressive muscle damage and impairments in regeneration. The DGC also plays a pivotal role in the organization of neuromuscular junctions (NMJs), where it stabilizes postsynaptic machinery, including receptors for the neurotransmitter acetylcholine (AChRs). Here, we focus on the role of the DGC complex in NMJ and skeletal muscle physiology and discuss the novel components that are associated with the complex.