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抵抗运动训练拯救了患有1型肌肉性缩症患者骨肌肉中的线粒体功能障碍

Valeria Di Leo^1,2, Conor Lawless¹, Marie-Pier Roussel³

¹Wellcome Centre for Mitochondrial Research, Translational and Clinical Research Institute, Faculty of Medical Sciences, Newcastle University, UK.

Journal of neuromuscular diseases

|August 28, 2023

概括

抵抗运动在某种程度上可以在1型肌肉性缩症 (DM1) 患者中缓解线粒体功能障碍. 12周的训练增加了线粒体质量和改善了呼吸链蛋白水平,这表明线粒体在运动反应中发挥作用.

科学领域:

神经肌肉疾病神经肌肉疾病
线粒体生物学线粒体生物学
运动生理学运动生理学

背景情况:

肌性缩症1型 (DM1) 是一种遗传性神经肌肉疾病,没有治愈方法.
DM1是由DMPK基因中CTG三重重复扩张引起的.
线粒体功能障碍与DM1病变发生有关.

研究的目的:

调查12周阻力运动训练对DM1患者骨肌肉线粒体氧化酸化的影响.
将DM1患者的线粒体功能与健康对照进行比较.
评估运动后呼吸链复合体和线粒体质量的变化.

主要方法:

从DM1患者和对照人群中获得了骨肌肉活检.
免疫光评估了呼吸链复合体I (CI) 和IV (CIV) 的蛋白质水平,以及线粒体质量.
纤维根据运动前和运动后的CI和CIV水平进行分类.
转换试验分析了患者内部的变化.

主要成果:

在DM1患者表现出 mitochondrial 质量减少和 CI/CIV 缺乏在基线.
抵抗运动训练在大多数患者中显著增加了线粒体质量.
所有患者在训练后显示CI和/或CIV蛋白水平显著增加.
线粒体质量的改善与力量的增加相关.

结论:

12周的抵抗运动训练可以在DM1患者中部分恢复线粒体功能.
运动诱导的改善表明线粒体适应在DM1中的作用.
抵抗运动可能是DM1的有益治疗策略.

关键词:

肌性缩症 1 型 1 型线粒体功能障碍线粒体功能障碍肌性缩症1型治疗疗法氧化酸化缺陷氧化酸化缺陷阻力运动训练阻力运动训练这是骨肌肉的骨架肌肉.力量训练力量训练力量训练力量训练

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