关键词: developmental biology dystroglycanopathies neuromuscular development skeletal muscle zebrafish

Mesh : Animals Electric Stimulation Humans Muscle, Skeletal / physiology Muscular Dystrophy, Duchenne / genetics therapy Neuromuscular Junction / physiology Zebrafish

来  源:   DOI:10.7554/eLife.62760

Abstract:
Neuromuscular electrical stimulation (NMES) allows activation of muscle fibers in the absence of voluntary force generation. NMES could have the potential to promote muscle homeostasis in the context of muscle disease, but the impacts of NMES on diseased muscle are not well understood. We used the zebrafish Duchenne muscular dystrophy (dmd) mutant and a longitudinal design to elucidate the consequences of NMES on muscle health. We designed four neuromuscular stimulation paradigms loosely based on weightlifting regimens. Each paradigm differentially affected neuromuscular structure, function, and survival. Only endurance neuromuscular stimulation (eNMES) improved all outcome measures. We found that eNMES improves muscle and neuromuscular junction morphology, swimming, and survival. Heme oxygenase and integrin alpha7 are required for eNMES-mediated improvement. Our data indicate that neuromuscular stimulation can be beneficial, suggesting that the right type of activity may benefit patients with muscle disease.
摘要:
神经肌肉电刺激(NMES)允许在没有自愿产生力的情况下激活肌肉纤维。在肌肉疾病的背景下,NMES可能有促进肌肉稳态的潜力,但是NMES对患病肌肉的影响尚不清楚。我们使用斑马鱼Duchenne肌营养不良(dmd)突变体和纵向设计来阐明NMES对肌肉健康的影响。我们根据举重方案宽松地设计了四种神经肌肉刺激范例。每个范式都不同地影响神经肌肉结构,函数,和生存。只有耐力神经肌肉刺激(eNMES)改善了所有结果指标。我们发现eNMES改善了肌肉和神经肌肉接头的形态,游泳,和生存。血红素加氧酶和整联蛋白α7是eNMES介导的改善所必需的。我们的数据表明神经肌肉刺激可能是有益的,表明正确的活动类型可能会使肌肉疾病患者受益。
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