Mitochondrial capacity, muscle endurance & low energy in friedreich ataxia

Hannah. M. Bossie M.S., T. Bradley. Willingham M.S., Robbi. A. Van Schoick M.S., Patrick. J. O'Connor Ph.D. andKevin. K. McCully Ph.D., Muscle & Nerve (Accepted Article) DOI: 10.1002/mus.25524
RESULTS: Groups did not differ in mitochondrial capacity (FRDA and AB: 1.8 ± 0.3 1/min). The difference in muscle endurance at 6 Hz was lower by 19.2% in the FRDA (group affect: P < 0.001). Feelings of physical energy were 34% lower in FRDA. 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). DISCUSSION: Non-invasive measures of skeletal muscle mitochondrial capacity and muscle specific endurance are useful in monitoring FRDA.