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We used muscle fibers isolated from 3-mo-old DMDmdx rats to further investigate the case. Most DMDmdx fibers exhibited no sign of trophic or morphology distinction as compared with WT fibers and mitochondria and t-tubule membrane networks also showed no stringent discrepancy. Under voltage clamp, values for holding current were similar in the two groups, whereas values for capacitance were larger in DMDmdx fibers, suggestive of enhanced amount of t-tubule membrane. The Ca2+ current density across the channel carried by the EC coupling voltage sensor (CaV1.1) was unchanged. The maximum rate of voltage-activated sarcoplasmic reticulum (SR) Ca2+ release was reduced by 25% in the DMDmdx fibers, with no change in voltage dependency. Imaging resting Ca2+ revealed rare spontaneous local SR Ca2+ release events with no sign of elevated activity in DMDmdx fibers. Under current clamp, DMDmdx fibers generated similar trains of action potentials as WT fibers. Results suggest that reduced peak amplitude of SR Ca2+ release is an inherent feature of this DMD model, likely contributing to muscle weakness. This occurs despite a preserved amount of releasable Ca2+ and with no change in excitability, CaV1.1 channel activity, and SR Ca2+ release at rest. Although we cannot exclude that fibers from the 3-mo-old animals do not yet display a fully developed disease phenotype, results provide limited support for pathomechanistic concepts frequently associated with DMD such as membrane fragility, excessive Ca2+ entry, or enhanced SR Ca2+ leak.<\/jats:p>","DOI":"10.1085\/jgp.202413588","type":"journal-article","created":{"date-parts":[[2024,12,7]],"date-time":"2024-12-07T02:20:21Z","timestamp":1733538021000},"update-policy":"https:\/\/doi.org\/10.1085\/jgp.crossmarkpolicy","source":"Crossref","is-referenced-by-count":2,"title":["Reduced voltage-activated Ca2+ release flux in muscle fibers from a rat model of Duchenne dystrophy"],"prefix":"10.1085","volume":"157","author":[{"ORCID":"https:\/\/orcid.org\/0009-0004-3221-2590","authenticated-orcid":true,"given":"Jonathan","family":"Schreiber","sequence":"first","affiliation":[{"id":[{"id":"https:\/\/ror.org\/029brtt94","id-type":"ROR","asserted-by":"publisher"}],"name":"University Lyon, Universit\u00e9 Claude Bernard Lyon 1, CNRS UMR-5261, INSERM U-1315, Institut NeuroMyoG\u00e8ne - Pathophysiology and Genetics of Neuron and Muscle 1 , Lyon,","place":["France"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0008-0150-1604","authenticated-orcid":true,"given":"Ludivine","family":"Rotard","sequence":"additional","affiliation":[{"id":[{"id":"https:\/\/ror.org\/029brtt94","id-type":"ROR","asserted-by":"publisher"}],"name":"University Lyon, Universit\u00e9 Claude Bernard Lyon 1, CNRS UMR-5261, INSERM U-1315, Institut NeuroMyoG\u00e8ne - Pathophysiology and Genetics of Neuron and Muscle 1 , Lyon,","place":["France"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1506-1901","authenticated-orcid":true,"given":"Yves","family":"Tourneur","sequence":"additional","affiliation":[{"name":"Cidade Universit\u00e1ria 2 UFPE Department Nutri\u00e7\u00e3o, , Recife,","place":["Brazil"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1140-0336","authenticated-orcid":true,"given":"Aude","family":"Lafoux","sequence":"additional","affiliation":[{"id":[{"id":"https:\/\/ror.org\/03gnr7b55","id-type":"ROR","asserted-by":"publisher"}],"name":"Therassay Platform, CAPACITES, Nantes Universit\u00e9 3 , Nantes,","place":["France"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9608-643X","authenticated-orcid":true,"given":"Christine","family":"Berthier","sequence":"additional","affiliation":[{"id":[{"id":"https:\/\/ror.org\/029brtt94","id-type":"ROR","asserted-by":"publisher"}],"name":"University Lyon, Universit\u00e9 Claude Bernard Lyon 1, CNRS UMR-5261, INSERM U-1315, Institut NeuroMyoG\u00e8ne - Pathophysiology and Genetics of Neuron and Muscle 1 , Lyon,","place":["France"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7131-7828","authenticated-orcid":true,"given":"Bruno","family":"Allard","sequence":"additional","affiliation":[{"id":[{"id":"https:\/\/ror.org\/029brtt94","id-type":"ROR","asserted-by":"publisher"}],"name":"University Lyon, Universit\u00e9 Claude Bernard Lyon 1, CNRS UMR-5261, INSERM U-1315, Institut NeuroMyoG\u00e8ne - Pathophysiology and Genetics of Neuron and Muscle 1 , Lyon,","place":["France"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0003-2725-5206","authenticated-orcid":true,"given":"Corinne","family":"Huchet","sequence":"additional","affiliation":[{"id":[{"id":"https:\/\/ror.org\/03gnr7b55","id-type":"ROR","asserted-by":"publisher"}],"name":"Therassay Platform, CAPACITES, Nantes Universit\u00e9 3 , Nantes,","place":["France"]},{"id":[{"id":"https:\/\/ror.org\/03gnr7b55","id-type":"ROR","asserted-by":"publisher"}],"name":"Nantes Universit\u00e9, INSERM UMR TARGET 1089 4 Nantes Gene Therapy Laboratory, , Nantes,","place":["France"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4944-270X","authenticated-orcid":true,"given":"Vincent","family":"Jacquemond","sequence":"additional","affiliation":[{"id":[{"id":"https:\/\/ror.org\/029brtt94","id-type":"ROR","asserted-by":"publisher"}],"name":"University Lyon, Universit\u00e9 Claude Bernard Lyon 1, CNRS UMR-5261, INSERM U-1315, Institut NeuroMyoG\u00e8ne - Pathophysiology and Genetics of Neuron and Muscle 1 , Lyon,","place":["France"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"291","published-online":{"date-parts":[[2024,12,24]]},"reference":[{"key":"2024122414190784600_bib1","doi-asserted-by":"publisher","first-page":"18763","DOI":"10.1073\/pnas.0900705106","article-title":"T-tubule disorganization and defective excitation-contraction coupling in muscle fibers lacking myotubularin lipid phosphatase","volume":"106","author":"Al-Qusairi","year":"2009","journal-title":"Proc. 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