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NEUROLOGY 1988;38:476
© 1988 American Academy of Neurology

Free cytoplasmic Ca++ at rest and after cholinergic stimulus is increased in cultured muscle cells from Duchenne muscular dystrophy patients

T. Mongini, MD, D. Ghigo, MD, C. Doriguzzi, MD, F. Bussolino, MD, G. Pescarmona, MD, B. Polio, MD, D. Schiffer, MD and A. Bosia, MD

From Clinica Neurologica II (Drs. Mongini, Doriguzzi, Pollo, and Schiffer) and Dipartimento di Genetica (Sezione di Chimica Medica) (Drs. Ghigo, Bussolino, Pescarmona, and Bosia), Università degli Studi, Torino, Italy.

We used a fluorescent dye, quin 2, to measure intracellular free calcium ([Ca+ +]i) in cultured skeletal muscle cells and skin fibroblasts from five Duchenne muscular dystrophy (DMD) patients and from five controls. We observed an enhanced [Ca+ +]i level, at rest and after acetylcholine (ACh) stimulation, in DMD muscle cells, but we did not detect any difference between DMD and normal skin fibroblasts. The abnormally higher [Ca+ +]i transient induced by ACh suggests that it plays a critical role in muscle degeneration. The skin fibroblast results suggest that there is no generalized membrane defect.

Address correspondence and reprint requests to Dr. Mongini, Clinica Neurologies II, Via Cherasco 15, 10126, Torino, Italy.

Presented in part at the thirty-ninth annual meeting of the American Academy of Neurology, New York, NY, April 1987.

Received April 24, 1987. Accepted for publication in final form July 28, 1987.




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