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Science 2 November 1990:
Vol. 250. no. 4981, pp. 673 - 676
DOI: 10.1126/science.2173137
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Articles

Science, Vol 250, Issue 4981, 673-676
Copyright © 1990 by American Association for the Advancement of Science

articles

Increased activity of calcium leak channels in myotubes of Duchenne human and mdx mouse origin

PY Fong, PR Turner, WF Denetclaw, and RA Steinhardt

Department of Molecular and Cell Biology, University of California, Berkeley 94720.

Elevated free Ca2+ concentrations found in adult dystrophic muscle fibers result in enhanced protein degradation. Since the difference in concentrations may reflect differences in entry, Ca2+ leak channels in cultures of normal and Duchenne human myotubes, and normal and mdx murine myotubes, have been identified and characterized. The open probability of leak channels is markedly increased in dystrophic myotubes. Other channel properties, such as mean open times, single channel conductance, ion selectivity, and behavior in the presence of pharmacological agents, were similar among myotube types. Compared to the Ca2+ concentrations in normal human and normal mouse myotubes, intracellular resting free Ca2+ concentrations ([Ca2+]i) in myotubes of Duchenne and mdx origin were significantly higher at a time when dystrophin is first expressed in normal tissue. Taken together, these findings suggest that the increased open probability of Ca2+ leak channels contributes to the elevated free intracellular Ca2+ concentration in Duchenne human and mdx mouse myotubes.


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