Responses to extracellular ATP of lymphoblastoid cell lines from Duchenne muscular dystrophy patients

Administration of extracellular ATP to lymphoblastoid cell lines in normal subjects and Duchenne muscular dystrophy patients to analyze their response to ATP reveals that normal and dystrophic cell lines respond differently to extracellular ATP and that dystrophy patients are more sensitive to extracellular ATP. Extracellular ATP induces a rise in plasma membrane depolarization and cytoplasmic Ca2+ levels and causes swelling, rounding and lysing of the cells in dystrophy patients, indicating the presence of an ATP-gated membrane channel which is permeable to Ca2+ and Na+.