{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,11,2]],"date-time":"2025-11-02T08:54:40Z","timestamp":1762073680905,"version":"build-2065373602"},"reference-count":38,"publisher":"MDPI AG","issue":"11","license":[{"start":{"date-parts":[[2022,11,12]],"date-time":"2022-11-12T00:00:00Z","timestamp":1668211200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Duchenne Parent Project","award":["19.006","RP1201727EA43399","RP12117A5CD9D9E7","23722","PRIN20178L7WRS","PRIN2020Z73J5A","IG-23010","RF-2018-12366215"],"award-info":[{"award-number":["19.006","RP1201727EA43399","RP12117A5CD9D9E7","23722","PRIN20178L7WRS","PRIN2020Z73J5A","IG-23010","RF-2018-12366215"]}]},{"DOI":"10.13039\/501100004271","name":"Sapienza University of Rome","doi-asserted-by":"publisher","award":["19.006","RP1201727EA43399","RP12117A5CD9D9E7","23722","PRIN20178L7WRS","PRIN2020Z73J5A","IG-23010","RF-2018-12366215"],"award-info":[{"award-number":["19.006","RP1201727EA43399","RP12117A5CD9D9E7","23722","PRIN20178L7WRS","PRIN2020Z73J5A","IG-23010","RF-2018-12366215"]}],"id":[{"id":"10.13039\/501100004271","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100007393","name":"AFM-Telethon","doi-asserted-by":"publisher","award":["19.006","RP1201727EA43399","RP12117A5CD9D9E7","23722","PRIN20178L7WRS","PRIN2020Z73J5A","IG-23010","RF-2018-12366215"],"award-info":[{"award-number":["19.006","RP1201727EA43399","RP12117A5CD9D9E7","23722","PRIN20178L7WRS","PRIN2020Z73J5A","IG-23010","RF-2018-12366215"]}],"id":[{"id":"10.13039\/100007393","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100003407","name":"Italian Ministry of University","doi-asserted-by":"publisher","award":["19.006","RP1201727EA43399","RP12117A5CD9D9E7","23722","PRIN20178L7WRS","PRIN2020Z73J5A","IG-23010","RF-2018-12366215"],"award-info":[{"award-number":["19.006","RP1201727EA43399","RP12117A5CD9D9E7","23722","PRIN20178L7WRS","PRIN2020Z73J5A","IG-23010","RF-2018-12366215"]}],"id":[{"id":"10.13039\/501100003407","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100007076","name":"AIRC Foundation for Cancer Research in Italy","doi-asserted-by":"publisher","award":["19.006","RP1201727EA43399","RP12117A5CD9D9E7","23722","PRIN20178L7WRS","PRIN2020Z73J5A","IG-23010","RF-2018-12366215"],"award-info":[{"award-number":["19.006","RP1201727EA43399","RP12117A5CD9D9E7","23722","PRIN20178L7WRS","PRIN2020Z73J5A","IG-23010","RF-2018-12366215"]}],"id":[{"id":"10.13039\/501100007076","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100003196","name":"Italian Ministry of Health","doi-asserted-by":"publisher","award":["19.006","RP1201727EA43399","RP12117A5CD9D9E7","23722","PRIN20178L7WRS","PRIN2020Z73J5A","IG-23010","RF-2018-12366215"],"award-info":[{"award-number":["19.006","RP1201727EA43399","RP12117A5CD9D9E7","23722","PRIN20178L7WRS","PRIN2020Z73J5A","IG-23010","RF-2018-12366215"]}],"id":[{"id":"10.13039\/501100003196","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Life"],"abstract":"<jats:p>Dystrophin is a cytoskeletal protein contributing to the organization of the neuromuscular junction. In Duchenne muscular dystrophy, due to dystrophin absence, the distribution of endplate acetylcholine receptors (AChRs) becomes disorganized. It is still debated whether this is due to the absence of dystrophin or to the repeated damage\/regeneration cycles typical of dystrophic muscle. We addressed this controversy studying the endplate in the first 3 postnatal weeks, when muscle damage in dystrophic (mdx) mice is minimal. By synaptic and extra-synaptic patch-clamp recordings in acutely dissociated mdx and wt muscle fibers, we recorded unitary events due to openings of AChR-channels containing the \u03b3 and \u03b5 subunit. We also examined AChR distribution at the endplate by immunofluorescence assays. No differences between wt and mdx fibers were found in the \u03b3\/\u03b5 switch, nor in the AChR organization at the endplates up to 21 postnatal days. Conversely, we detected a delayed appearance and disappearance of patches with high channel opening frequency in mdx fibers. Our data emphasize that the innervation-dependent \u03b3\/\u03b5 switch and AChR organization in the endplate are not affected by the absence of dystrophin, while extra-synaptic AChR cluster formation and disassembly could be differentially regulated in mdx mice.<\/jats:p>","DOI":"10.3390\/life12111861","type":"journal-article","created":{"date-parts":[[2022,11,14]],"date-time":"2022-11-14T02:33:10Z","timestamp":1668393190000},"page":"1861","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Early Developmental Changes of Muscle Acetylcholine Receptors Are Little Influenced by Dystrophin Absence in mdx Mouse"],"prefix":"10.3390","volume":"12","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1878-0159","authenticated-orcid":false,"given":"Marta","family":"Morotti","sequence":"first","affiliation":[{"name":"Department of Physiology and Pharmacology, Sapienza University of Rome, 00185 Rome, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alessandro","family":"Gaeta","sequence":"additional","affiliation":[{"name":"Department of Physiology and Pharmacology, Sapienza University of Rome, 00185 Rome, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7504-8197","authenticated-orcid":false,"given":"Cristina","family":"Limatola","sequence":"additional","affiliation":[{"name":"Laboratory Affiliated to Istituto Pasteur Italia, Department of Physiology and Pharmacology, Sapienza University of Rome, 00185 Rome, Italy"},{"name":"Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS) Neuromed, 86077 Pozzilli, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4638-9202","authenticated-orcid":false,"given":"Myriam","family":"Catalano","sequence":"additional","affiliation":[{"name":"Department of Physiology and Pharmacology, Sapienza University of Rome, 00185 Rome, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7480-1087","authenticated-orcid":false,"given":"Maria Amalia","family":"Di Castro","sequence":"additional","affiliation":[{"name":"Department of Physiology and Pharmacology, Sapienza University of Rome, 00185 Rome, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0169-8397","authenticated-orcid":false,"given":"Francesca","family":"Grassi","sequence":"additional","affiliation":[{"name":"Department of Physiology and Pharmacology, Sapienza University of Rome, 00185 Rome, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2022,11,12]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"108","DOI":"10.1016\/j.bbadis.2006.05.010","article-title":"Dystrophin, Its Interactions with Other Proteins, and Implications for Muscular Dystrophy","volume":"1772","author":"Ervasti","year":"2007","journal-title":"Biochim. 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