{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,3]],"date-time":"2026-10-03T12:14:55Z","timestamp":1791029695208,"version":"4.1.0"},"reference-count":69,"publisher":"Wiley","issue":"19","license":[{"start":{"date-parts":[[2011,9,29]],"date-time":"2011-09-29T00:00:00Z","timestamp":1317254400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["The Journal of Physiology"],"published-print":{"date-parts":[[2011,10]]},"abstract":"<jats:p><jats:bold>Non\u2010Technical Summary\u2002<\/jats:bold> Central core disease (CCD) is linked to mutations in the skeletal muscle ryanodine receptor (RyR1) gene that are believed to disrupt excitation\u2013contraction (EC) coupling. EC coupling requires activation of RyR1s to release Ca<jats:sup>2+<\/jats:sup> from the sarcoplasmic reticulum (SR). Subsequently, the SR Ca<jats:sup>2+<\/jats:sup>\u2010ATPase (SERCA) returns cytoplasmic Ca<jats:sup>2+<\/jats:sup> to intracellular stores. Here, we have investigated the effects of two CCD RyR1 mutants (I4897T and Y523S) in C2C12 myotubes. Both mutations significantly altered myogenesis and SERCA gene expression \u2013 inhibition by I4897T and stimulation by Y523S. They are thought to behave differently (\u2018Ca<jats:sup>2+<\/jats:sup>\u2010impermeable\u2019 and \u2018leaky\u2019, respectively), but disrupted EC coupling through the same mechanism (store depletion). It is conceivable that reduced SERCA expression by I4897T could explain this paradox. In both cases, the neuropeptide CGRP restored EC coupling by increasing SR Ca<jats:sup>2+<\/jats:sup> content. We propose that CGRP and its corresponding signalling pathway exert beneficial effects in myotubes expressing CCD mutants.<\/jats:p>","DOI":"10.1113\/jphysiol.2011.210765","type":"journal-article","created":{"date-parts":[[2011,8,9]],"date-time":"2011-08-09T05:46:18Z","timestamp":1312868778000},"page":"4649-4669","source":"Crossref","is-referenced-by-count":16,"title":["Calcitonin gene\u2010related peptide restores disrupted excitation\u2013contraction coupling in myotubes expressing central core disease mutations in RyR1"],"prefix":"10.1113","volume":"589","author":[{"given":"Ana 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