期刊论文详细信息
NEUROSCIENCE LETTERS 卷:737
Alterations of neuromuscular junctions in Duchenne muscular dystrophy
Review
Lovering, Richard M.1,2,3  Iyer, Shama R.1,2  Edwards, Benjamin4  Davies, Kay E.4 
[1] Univ Maryland, Sch Med, Dept Orthopaed, 100 Penn St,AHB,Room 540, Baltimore, MD 21201 USA
[2] Univ Maryland, Sch Med, Dept Physiol, Baltimore, MD 21201 USA
[3] Univ Maryland, Sch Med, Baltimore, MD 21201 USA
[4] Univ Oxford, MDUK Oxford Neuromuscular Ctr, Dept Physiol Anat & Genet, Oxford, England
关键词: mdx;    NMJ;    Duchenne muscular dystrophy;    Eccentric injury;   
DOI  :  10.1016/j.neulet.2020.135304
来源: Elsevier
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【 摘 要 】

The focus of this review is on Duchenne muscular dystrophy (DMD), which is caused by the absence of the protein dystrophin and is characterized as a neuromuscular disease in which muscle weakness, increased susceptibility to muscle injury, and inadequate repair appear to underlie the pathology. Considerable attention has been dedicated to studying muscle fiber damage, but data show that both human patients and animal models for DMD present with fragmented neuromuscular junction (NMJ) morphology. In addition to preand post-synaptic abnormalities, studies indicate increased susceptibility of the NMJ to contraction-induced injury, with corresponding functional changes in neuromuscular transmission and nerve-evoked electromyographic activity. Such findings suggest that alterations in the NMJ of dystrophic muscle may play a role in muscle weakness via impairment of neuromuscular transmission. Further work is needed to fully understand the role of the NMJ in the weakness, susceptibility to injury, and progressive wasting associated with DMD.

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