{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,3]],"date-time":"2026-05-03T01:31:53Z","timestamp":1777771913014,"version":"3.51.4"},"reference-count":76,"publisher":"MDPI AG","issue":"9","license":[{"start":{"date-parts":[[2022,4,22]],"date-time":"2022-04-22T00:00:00Z","timestamp":1650585600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100000049","name":"National Institute on Aging","doi-asserted-by":"publisher","award":["R01-AG056504"],"award-info":[{"award-number":["R01-AG056504"]}],"id":[{"id":"10.13039\/100000049","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cells"],"abstract":"<jats:p>Duchenne muscular dystrophy (DMD) is a common X-linked degenerative muscle disorder that involves mutations in the DMD gene that frequently reduce the expression of the dystrophin protein, compromising the structural integrity of the sarcolemmal membrane and leaving it vulnerable to injury during cycles of muscle contraction and relaxation. This results in an increased frequency of sarcolemma disruptions that can compromise the barrier function of the membrane and lead to death of the myocyte. Sarcolemmal membrane repair processes can potentially compensate for increased membrane disruptions in DMD myocytes. Previous studies demonstrated that TRIM72, a muscle-enriched tripartite motif (TRIM) family protein also known as mitsugumin 53 (MG53), is a component of the cell membrane repair machinery in striated muscle. To test the importance of membrane repair in striated muscle in compensating for the membrane fragility in DMD, we crossed TRIM72\/MG53 knockout mice into the mdx mouse model of DMD. These double knockout (DKO) mice showed compromised sarcolemmal membrane integrity compared to mdx mice, as measured by immunoglobulin G staining and ex vivo muscle laser microscopy wounding assays. We also found a significant decrease in muscle ex vivo contractile function as compared to mdx mice at both 6 weeks and 1.5 years of age. As the DKO mice aged, they developed more extensive fibrosis in skeletal muscles compared to mdx. Our findings indicate that TRIM72\/MG53-mediated membrane repair can partially compensate for the sarcolemmal fragility associated with DMD and that the loss of membrane repair results in increased pathology in the DKO mice.<\/jats:p>","DOI":"10.3390\/cells11091417","type":"journal-article","created":{"date-parts":[[2022,4,22]],"date-time":"2022-04-22T05:07:22Z","timestamp":1650604042000},"page":"1417","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["Reduced Sarcolemmal Membrane Repair Exacerbates Striated Muscle Pathology in a Mouse Model of Duchenne Muscular Dystrophy"],"prefix":"10.3390","volume":"11","author":[{"given":"Brian J.","family":"Paleo","sequence":"first","affiliation":[{"name":"Department of Physiology and Cell Biology, Davis Heart and Lung Research Institute, The Ohio State University, Columbus, OH 43210, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kevin E.","family":"McElhanon","sequence":"additional","affiliation":[{"name":"Department of Physiology and Cell Biology, Davis Heart and Lung Research Institute, The Ohio State University, Columbus, OH 43210, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hannah R.","family":"Bulgart","sequence":"additional","affiliation":[{"name":"Department of Physiology and Cell Biology, Davis Heart and Lung Research Institute, The Ohio State University, Columbus, OH 43210, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kassidy K.","family":"Banford","sequence":"additional","affiliation":[{"name":"Department of Physiology and Cell Biology, Davis Heart and Lung Research Institute, The Ohio State University, Columbus, OH 43210, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Eric X","family":"Beck","sequence":"additional","affiliation":[{"name":"Department of Physiology and Cell Biology, Davis Heart and Lung Research Institute, The Ohio State University, Columbus, OH 43210, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kristina M.","family":"Sattler","sequence":"additional","affiliation":[{"name":"Department of Biology, School of Behavioral & Natural Sciences, Mount St. Joseph University, Cincinnati, OH 45233, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Briana N.","family":"Goines","sequence":"additional","affiliation":[{"name":"Department of Biology, School of Behavioral & Natural Sciences, Mount St. Joseph University, Cincinnati, OH 45233, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Shelby L.","family":"Ratcliff","sequence":"additional","affiliation":[{"name":"Department of Biology, School of Behavioral & Natural Sciences, Mount St. Joseph University, Cincinnati, OH 45233, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kelly E.","family":"Crowe","sequence":"additional","affiliation":[{"name":"Department of Biology, School of Behavioral & Natural Sciences, Mount St. Joseph University, Cincinnati, OH 45233, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2710-3088","authenticated-orcid":false,"given":"Noah","family":"Weisleder","sequence":"additional","affiliation":[{"name":"Department of Physiology and Cell Biology, Davis Heart and Lung Research Institute, The Ohio State University, Columbus, OH 43210, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2022,4,22]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"1747","DOI":"10.1083\/jcb.131.6.1747","article-title":"Calcium-regulated exocytosis is required for cell membrane resealing","volume":"131","author":"Bi","year":"1995","journal-title":"J. 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