{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,3]],"date-time":"2026-10-03T16:52:10Z","timestamp":1791046330934,"version":"4.1.0"},"reference-count":63,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2004,5,4]],"date-time":"2004-05-04T00:00:00Z","timestamp":1083628800000},"content-version":"vor","delay-in-days":3,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["The Journal of Physiology"],"published-print":{"date-parts":[[2004,5]]},"abstract":"<jats:p>The <jats:italic>mdx<\/jats:italic> mouse, a model of the human disease Duchenne muscular dystrophy, has skeletal muscle fibres which display incompletely understood impaired contractile function. We explored the possibility that action potential\u2010evoked Ca<jats:sup>2+<\/jats:sup> release is altered in <jats:italic>mdx<\/jats:italic> fibres. Action potential\u2010evoked Ca<jats:sup>2+<\/jats:sup>\u2010dependent fluorescence transients were recorded, using both low and high affinity Ca<jats:sup>2+<\/jats:sup> indicators, from enzymatically isolated fibres obtained from extensor digitorum longus (EDL) and flexor digitorum brevis (FDB) muscles of normal and <jats:italic>mdx<\/jats:italic> mice. Fibres were immobilized using either intracellular EGTA or <jats:italic>N<\/jats:italic>\u2010benzyl\u2010<jats:italic>p<\/jats:italic>\u2010toluene sulphonamide, an inhibitor of the myosin II ATPase. We found that the amplitude of the action potential\u2010evoked Ca<jats:sup>2+<\/jats:sup> transients was significantly decreased in <jats:italic>mdx<\/jats:italic> mice with no measured difference in that of the surface action potential. In addition, Ca<jats:sup>2+<\/jats:sup> transients recorded from <jats:italic>mdx<\/jats:italic> fibres in the absence of EGTA also displayed a marked prolongation of the slow decay phase. Model simulations of the action potential\u2010evoked transients in the presence of high EGTA concentrations suggest that the reduction in the evoked sarcoplasmic reticulum Ca<jats:sup>2+<\/jats:sup> release flux is responsible for the decrease in the peak of the Ca<jats:sup>2+<\/jats:sup> transient in <jats:italic>mdx<\/jats:italic> fibres. Since the myoplasmic Ca<jats:sup>2+<\/jats:sup> concentration is a critical regulator of muscle contraction, these results may help to explain the weakness observed in skeletal muscle fibres from <jats:italic>mdx<\/jats:italic> mice and, possibly, Duchenne muscular dystrophy patients.<\/jats:p>","DOI":"10.1113\/jphysiol.2004.061291","type":"journal-article","created":{"date-parts":[[2004,3,9]],"date-time":"2004-03-09T01:43:51Z","timestamp":1078796631000},"page":"59-75","source":"Crossref","is-referenced-by-count":108,"title":["The action potential\u2010evoked sarcoplasmic reticulum calcium release is impaired in <i>mdx<\/i> mouse muscle fibres"],"prefix":"10.1113","volume":"557","author":[{"given":"Christopher 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