McGarrell Nicole T, Green Max E, McCully Kevin K
Department of Kinesiology, University of Georgia, Athens, GA 30602, USA.
Northside Hospital, Atlanta, GA 30342, USA.
Muscles. 2025 Jan 9;4(1):1. doi: 10.3390/muscles4010001.
Friedreich's ataxia (FRDA) results from a faulty mitochondrial protein known as Frataxin. The purpose of this case report was to test whether skeletal muscle in FRDA can adapt to an endurance-based training program using neuromuscular electrical stimulation (NMES). A 36-year-old female with FRDA completed twelve training sessions, each lasting 30 min over 30 days, focused on the forearm muscles using NMES. Pre- and post-training session measurements of contractions, muscle-specific endurance, and muscle mitochondrial capacity were taken per training session. Training contractions increased from 4200 to 9420. Muscle-specific endurance increased by 14% at 2 Hz and 17% at 4 Hz. Muscle endurance at 6 Hz increased from 0% to 51%. The rate constant of mitochondrial capacity was 0.95 min pre- and 0.99 min post-training session. In conclusion, one month of NMES increased training volume and muscle-specific endurance but did not change mitochondrial capacity. Muscle adaptations to endurance training were seen in FRDA, but increased training might be needed to test if mitochondrial capacity can improve.
弗里德赖希共济失调(FRDA)是由一种名为铁调素的线粒体蛋白缺陷引起的。本病例报告的目的是测试FRDA患者的骨骼肌是否能通过神经肌肉电刺激(NMES)适应基于耐力的训练计划。一名36岁的FRDA女性患者完成了12次训练课程,每次持续30分钟,共30天,训练重点是使用NMES锻炼前臂肌肉。每次训练前和训练后都测量了收缩、肌肉特异性耐力和肌肉线粒体容量。训练收缩次数从4200次增加到9420次。肌肉特异性耐力在2赫兹时增加了14%,在4赫兹时增加了17%。6赫兹时的肌肉耐力从0%增加到51%。线粒体容量的速率常数在训练前为0.95分钟,训练后为0.99分钟。总之, 一个月的NMES增加了训练量和肌肉特异性耐力,但没有改变线粒体容量。在FRDA患者中观察到了肌肉对耐力训练的适应性,但可能需要增加训练量来测试线粒体容量是否可以改善。