Mitochondrial capacity, muscle endurance, and low energy in friedreich ataxia

Mitochondrial capacity, muscle endurance, and low energy in friedreich ataxia

Bossie H M; Willingham T B; Schoick R A V; O'Connor P J; McCully K K

Muscle and Nerve

2017

adolescent; priority journal; scoring system; school child; cross-sectional study; fatigue; human; article; child; female; male; controlled study; adult; clinical article; comparative study; disease severity; Friedreich ataxia; cellular subcellular and molecular biological phenomena and functions; energy; physical energy; mitochondrial capacity; muscle exercise; electrostimulation; Godin Leisure Time Physical Activity Score; mechanomyography; myography; near infrared spectroscopy; oxygen consumption; skeletal muscle; trajectory; characteristics; endurance; muscle endurance; alertness

Introduction: In this study we noninvasively evaluated skeletal muscle mitochondrial capacity, muscle-specific endurance, and energy/fatigue feelings in persons with Friedreich ataxia (FRDA) and able-bodied controls (AB). Methods: Forearm mitochondrial capacity was measured in FRDA (n = 16) and AB (n = 10) study participants using the rate of recovery of oxygen consumption after electrical stimulation with near-infrared spectroscopy. Mechanomyography (MMG) assessed muscle endurance after electrical stimulation for 3 minutes at 2 Hz, 4 Hz, and 6 Hz. Validated scales assessed disease severity and energy/fatigue feelings. Results: Groups did not differ in mitochondrial capacity (FRDA and AB: 1.8 +/- 0.3 L/min). The difference in muscle endurance at 6 Hz was lower by 19.2% in the FRDA group (group effect: P < 0.001). Feelings of physical energy were 34% lower in FRDA group. In FDRA muscle, endurance was positively related to mitochondrial capacity (r = 0.59, P = 0.03), and disease severity was negatively related to mitochondrial capacity (r = -0.55, P = 0.04) and muscle endurance (r = -0.60, P = 0.01). Conclusion: Non-invasive measures of skeletal muscle mitochondrial capacity and muscle-specific endurance are useful in monitoring FRDA. Muscle Nerve 56: 773-779, 2017.

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