Mitochondrial capacity, muscle endurance, and low energy in friedreich ataxia.
Muscle Nerve
; 56(4): 773-779, 2017 Oct.
Article
en En
| MEDLINE
| ID: mdl-28000230
ABSTRACT
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.Palabras clave
Texto completo:
1
Banco de datos:
MEDLINE
Asunto principal:
Resistencia Física
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Ataxia de Friedreich
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Músculo Esquelético
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Fatiga Muscular
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Metabolismo Energético
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Mitocondrias Musculares
Tipo de estudio:
Observational_studies
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Prevalence_studies
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Risk_factors_studies
Límite:
Adolescent
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Adult
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Child
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Female
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Humans
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Male
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Middle aged
Idioma:
En
Revista:
Muscle Nerve
Año:
2017
Tipo del documento:
Article
País de afiliación:
Estados Unidos