Exercise biology of neuromuscular disorders

被引:14
作者
Ng, Sean Y. [1 ]
Manta, Alexander [1 ]
Ljubicic, Vladimir [1 ]
机构
[1] McMaster Univ, Dept Kinesiol, Hamilton, ON L8S 4K1, Canada
基金
加拿大健康研究院; 加拿大自然科学与工程研究理事会;
关键词
Duchenne muscular dystrophy; spinal muscular atrophy; myotonic dystrophy type 1; AMPK; PGC-1; alpha; SPINAL MUSCULAR-ATROPHY; MYOTONIC-DYSTROPHY TYPE-1; SKELETAL-MUSCLE; THERAPEUTIC STRATEGIES; MODEL MOUSE; ACTIVATION; UTROPHIN; EXPRESSION; STRENGTH; SURVIVAL;
D O I
10.1139/apnm-2018-0229
中图分类号
R15 [营养卫生、食品卫生]; TS201 [基础科学];
学科分类号
100403 ;
摘要
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.
引用
收藏
页码:1194 / 1206
页数:13
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