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Review
. 2020 Mar 23:722:134833.
doi: 10.1016/j.neulet.2020.134833. Epub 2020 Feb 10.

The role of the dystrophin glycoprotein complex on the neuromuscular system

Dina C Belhasan  1 , Mohammed Akaaboune  2
Affiliations
Review

The role of the dystrophin glycoprotein complex on the neuromuscular system

Dina C Belhasan et al. Neurosci Lett. .

Abstract

The Dystrophin Glycoprotein Complex (DGC) is a large multi-protein complex that links cytoskeleton actin to the extracellular matrix. This complex is critical in maintaining the structural integrity of muscle fibers and the stability of the neuromuscular synapse. The DGC consists of dystrophin and its utrophin homolog, as well as dystroglycans, sarcoglycans, sarcospan, syntrophins, and dystrobrevins. Deficiencies in DGC proteins result in several forms of muscular dystrophy with varying symptoms and degrees of severity in addition to structurally abnormal neuromuscular junctions (NMJs). This mini-review highlights current knowledge regarding the role of the DGC on the molecular dynamics of acetylcholine receptors (AChRs) as it relates to the formation and maintenance of the mammalian NMJ.

Keywords: AChR dynamics; Dystrophin glycoprotein complex; Neuromuscular junction.

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Figures

Figure:
Figure:
A schematic representation of the relevant components of the dystrophin glycoprotein complex and pathways regulating the density of the nicotinic AChR at the cholinergic neuromuscular junction. The dynamics of AChR (removal, recycling, and insertion of new receptors) at synaptic sites are controlled by different events, including synaptic activity and components of the dystrophin glycoproteins complex.
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