Department of Kinesiology, McMaster University, Hamilton, ON L8S 4K1, Canada.
Appl Physiol Nutr Metab. 2018 Nov;43(11):1194-1206. doi: 10.1139/apnm-2018-0229. Epub 2018 Jun 26.
Neuromuscular disorders (NMDs) are chronic conditions that affect the neuromuscular system. Many NMDs currently have no cure; however, as more effective therapies become available for NMD patients, these individuals will exhibit improved health and/or prolonged lifespans. As a result, persons with NMDs will likely desire to engage in a more diverse variety of activities of daily living, including increased physical activity or exercise. Therefore, there is a need to increase our knowledge of the effects of acute exercise and chronic training on the neuromuscular system in NMD contexts. Here, we discuss the disease mechanisms and exercise biology of Duchenne muscular dystrophy (DMD), spinal muscular atrophy (SMA), and myotonic dystrophy type 1 (DM1), which are among the most prevalent NMDs in children and adults. Evidence from clinical and preclinical studies are reviewed, with emphasis on the functional outcomes of exercise, as well as on the putative cellular mechanisms that drive exercise-induced remodelling of the neuromuscular system. Continued investigation of the molecular mechanisms of exercise adaptation in DMD, SMA, and DM1 will assist in enhancing our understanding of the biology of these most prevalent NMDs. This information may also be useful for guiding the development of novel therapeutic targets for future pursuit.
神经肌肉疾病(NMDs)是影响神经肌肉系统的慢性疾病。目前许多 NMD 尚无治愈方法;然而,随着针对 NMD 患者的更有效疗法的出现,这些患者的健康状况将得到改善和/或寿命延长。因此,患有 NMD 的人可能希望从事更多样化的日常生活活动,包括增加身体活动或锻炼。因此,我们需要增加对急性运动和慢性训练对 NMD 环境中神经肌肉系统的影响的了解。在这里,我们讨论了杜氏肌营养不良症(DMD)、脊髓性肌萎缩症(SMA)和 1 型肌强直性营养不良症(DM1)的疾病机制和运动生物学,这些疾病是儿童和成人中最常见的 NMD 之一。回顾了来自临床和临床前研究的证据,重点介绍了运动的功能结果,以及驱动神经肌肉系统运动诱导重塑的潜在细胞机制。对 DMD、SMA 和 DM1 中运动适应的分子机制的持续研究将有助于增强我们对这些最常见 NMD 生物学的理解。这些信息也可能有助于指导未来寻求新的治疗靶点的发展。