The role of the dystrophin glycoprotein complex on the neuromuscular system

被引：45

作者：

Belhasan, Dina C. ^{[1
]}

Akaaboune, Mohammed ^{[1
,2
]}

机构：

[1] Dept Mol Cellular & Dev Biol, Ann Arbor, MI 48109 USA

[2] Univ Michigan, Program Neurosci, Ann Arbor, MI 48109 USA

来源：

NEUROSCIENCE LETTERS | 2020年 / 722卷

关键词：

Dystrophin glycoprotein complex; Neuromuscular junction; AChR dynamics; ACETYLCHOLINE-RECEPTOR AGGREGATION; MICE LACKING UTROPHIN; NITRIC-OXIDE SYNTHASE; MUSCULAR-DYSTROPHY; ALPHA-DYSTROGLYCAN; EXTRACELLULAR-MATRIX; JUNCTION DEVELOPMENT; BETA-DYSTROGLYCAN; SKELETAL-MUSCLE; GENE-EXPRESSION;

D O I：

10.1016/j.neulet.2020.134833

中图分类号：

Q189 [神经科学];

学科分类号：

071006 [神经生物学];

摘要：

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.

引用

页数：5

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