Literature DB >> 32057921

The role of the dystrophin glycoprotein complex on the neuromuscular system.

Dina C Belhasan1, Mohammed Akaaboune2.   

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.
Copyright © 2020 Elsevier B.V. All rights reserved.

Entities:  

Keywords:  AChR dynamics; Dystrophin glycoprotein complex; Neuromuscular junction

Mesh:

Substances:

Year:  2020        PMID: 32057921      PMCID: PMC8792866          DOI: 10.1016/j.neulet.2020.134833

Source DB:  PubMed          Journal:  Neurosci Lett        ISSN: 0304-3940            Impact factor:   3.046


  94 in total

1.  The absence of dystrophin rather than muscle degeneration causes acetylcholine receptor cluster defects in dystrophic muscle.

Authors:  Jie Kong; Liqing Yang; Qiuling Li; Jiqing Cao; Juan Yang; Fei Chen; Yanyun Wang; Cheng Zhang
Journal:  Neuroreport       Date:  2012-01-25       Impact factor: 1.837

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Journal:  Nat Genet       Date:  1999-11       Impact factor: 38.330

3.  Disruption of the beta-sarcoglycan gene reveals pathogenetic complexity of limb-girdle muscular dystrophy type 2E.

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Journal:  Mol Cell       Date:  2000-01       Impact factor: 17.970

4.  Interactions of the rapsyn RING-H2 domain with dystroglycan.

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Journal:  J Biol Chem       Date:  2001-05-07       Impact factor: 5.157

5.  The molecular basis of muscular dystrophy in the mdx mouse: a point mutation.

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Review 6.  The dystrophin glycoprotein complex: signaling strength and integrity for the sarcolemma.

Authors:  Karen A Lapidos; Rahul Kakkar; Elizabeth M McNally
Journal:  Circ Res       Date:  2004-04-30       Impact factor: 17.367

7.  Agrin mediates cell contact-induced acetylcholine receptor clustering.

Authors:  J T Campanelli; W Hoch; F Rupp; T Kreiner; R H Scheller
Journal:  Cell       Date:  1991-11-29       Impact factor: 41.582

8.  Muscle regeneration after imposed injury is better in younger than older mdx dystrophic mice.

Authors:  J M Zacharias; J E Anderson
Journal:  J Neurol Sci       Date:  1991-08       Impact factor: 3.181

9.  Identification of the Syrian hamster cardiomyopathy gene.

Authors:  V Nigro; Y Okazaki; A Belsito; G Piluso; Y Matsuda; L Politano; G Nigro; C Ventura; C Abbondanza; A M Molinari; D Acampora; M Nishimura; Y Hayashizaki; G A Puca
Journal:  Hum Mol Genet       Date:  1997-04       Impact factor: 6.150

10.  The sparing of extraocular muscle in dystrophinopathy is lost in mice lacking utrophin and dystrophin.

Authors:  J D Porter; J A Rafael; R J Ragusa; J K Brueckner; J I Trickett; K E Davies
Journal:  J Cell Sci       Date:  1998-07       Impact factor: 5.285

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3.  Distinct roles of the dystrophin-glycoprotein complex: α-dystrobrevin and α-syntrophin in the maintenance of the postsynaptic apparatus of the neuromuscular synapse.

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4.  Phosphorylation of α-dystrobrevin is essential for αkap accumulation and acetylcholine receptor stability.

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Review 7.  Recent insights into neuromuscular junction biology in Duchenne muscular dystrophy: Impacts, challenges, and opportunities.

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Review 8.  Secreted Signaling Molecules at the Neuromuscular Junction in Physiology and Pathology.

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Journal:  Int J Mol Sci       Date:  2021-02-28       Impact factor: 5.923

Review 9.  Multiple MuSK signaling pathways and the aging neuromuscular junction.

Authors:  Lauren A Fish; Justin R Fallon
Journal:  Neurosci Lett       Date:  2020-04-28       Impact factor: 3.046

10.  Myasthenia Gravis: From the Viewpoint of Pathogenicity Focusing on Acetylcholine Receptor Clustering, Trans-Synaptic Homeostasis and Synaptic Stability.

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