Abstract
A reduction of ATP content has been observed in muscle from patients with Duchenne muscular dystrophy. We tested the hypothesis that there is an accelerated rate of ATP turnover in the cultured muscle cells and fibroblasts from patients with Duchenne muscular dystrophy. ATP and ADP levels were similar in both muscle cells and fibroblasts from normal subjects and Duchenne patients. The rates of ATP and ADP formed from radioactive adenine and the enrichment of these pools was the same. ATP degradation was evaluated during a 10-minute incubation in 5.5 mM 2-deoxyglucose or during a 72-hour period of growth. There was significantly less radioactively-labelled ATP after incubation with deoxyglucose in Duchenne cells than in normal muscle cells, but there was no difference in total ATP concentration or ADP concentration or radioactive labelling. There was no difference in ATP or ADP degradation in fibroblasts. In longterm study of fibroblasts, radioactively labelled ATP decreased to 50% of control values at 24 hours and about 30% of control values at 72 hours, but there was no difference between normal and Duchenne cells. The data suggest that ATP turnover is normal in cells from Duchenne muscular dystrophy under the study conditions.
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Fox, I., Shefner, R., Palmieri, G. et al. DUCHENNE MUSCULAR DYSTROPHY: NORMAL ATP TURNOVER IN CULTURED CELLS: 65. Pediatr Res 19, 754 (1985). https://doi.org/10.1203/00006450-198507000-00085
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DOI: https://doi.org/10.1203/00006450-198507000-00085