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

Title

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

Creator

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

Identifier

Publisher

Muscle and Nerve

Date

2017

Subject

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

Description

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.

Rights

Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).

Citation

Bossie H M; Willingham T B; Schoick R A V; O'Connor P J; McCully K K, “Mitochondrial capacity, muscle endurance, and low energy in friedreich ataxia,” Pediatric Palliative Care Library, accessed October 18, 2021, https://pedpalascnetlibrary.omeka.net/items/show/16594.

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