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Ca2+ release from permeabilized skeletal muscle cells: a potential site for Mg2+-mediated regulation of muscle cell excitability. The myotonic dystrophies (dystrophy type 1, 2, and 3) are autosomal dominantmuscular dystrophies characterized by episodic paralysis and skeletal muscle cell damage. The common link between all three types is an alteration in Mg2+ concentration. In the present study, we haveexamined Mg2+-induced Ca2+ release (CRC) from skeletal muscle cells of patients with the myotonic dystrophies. We have used the thapsigargin-sensitive Ca2+ store-loading mechanism for isolation of theCa2+ store and permeabilization of the plasma membrane. In normal myotubes, the CRC activated by the thapsigargin-sensitive store is mainly due to Ca2+ release from internal stores. In contrast, in
dystrophic myotubes, thapsigargin induces a larger proportion of the total Ca2+ release that can be blocked by 50 microM ruthenium red. The "crowding" effect is observed in long-term incubated myotubes(7-10 days) from young and old subjects with muscular dystrophy type 1, while theDownloadDownload

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