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Little is known about CAPN3\u2019s function in muscle, but its loss results in aberrant sarcomere formation. Human muscle structure was analyzed in this study, with observations including integrin \u03b21D isoform (ITG\u03b21D) mislocalization, a lack of Talin-1 (TLN1) in the sarcolemma and the irregular expression of focal adhesion kinase (FAK) in LGMDR1 muscles, suggesting a lack of integrin activation with an altered sarcolemma, extracellular matrix (ECM) assembly and signaling pathway deregulation, which may cause frailty in LGMDR1 muscle fibers. Additionally, altered nuclear morphology, centrosome distribution and microtubule organization have been found in muscle cells derived from LGMDR1 patients.<\/jats:p>","DOI":"10.3390\/cells14060446","type":"journal-article","created":{"date-parts":[[2025,3,17]],"date-time":"2025-03-17T08:57:02Z","timestamp":1742201822000},"page":"446","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["The Role of Integrin \u03b21D Mislocalization in the Pathophysiology of Calpain 3-Related Limb\u2013Girdle Muscular Dystrophy"],"prefix":"10.3390","volume":"14","author":[{"given":"Andrea","family":"Valls","sequence":"first","affiliation":[{"name":"Neuromuscular Diseases Group, Neurosciences Area, Biogipuzkoa Health Research Institute, 20014 San Sebastian, Spain"},{"name":"Center for Biomedical Network Research on Neurodegenerative Diseases (CIBERNED), Spanish Ministry of Science & Innovation, Carlos III Health Institute, 28029 Madrid, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0009-0000-0900-1534","authenticated-orcid":false,"given":"Cristina","family":"Ruiz-Rold\u00e1n","sequence":"additional","affiliation":[{"name":"Neuromuscular Diseases Group, Neurosciences Area, Biogipuzkoa Health Research Institute, 20014 San Sebastian, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7236-1855","authenticated-orcid":false,"given":"Jenita","family":"Immanuel","sequence":"additional","affiliation":[{"name":"Neuromuscular Diseases Group, Neurosciences Area, Biogipuzkoa Health Research Institute, 20014 San Sebastian, Spain"},{"name":"Center for Biomedical Network Research on Neurodegenerative Diseases (CIBERNED), Spanish Ministry of Science & Innovation, Carlos III Health Institute, 28029 Madrid, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3254-0365","authenticated-orcid":false,"given":"Sonia","family":"Alonso-Mart\u00edn","sequence":"additional","affiliation":[{"name":"Center for Biomedical Network Research on Neurodegenerative Diseases (CIBERNED), Spanish Ministry of Science & Innovation, Carlos III Health Institute, 28029 Madrid, Spain"},{"name":"Stem Cells and Aging Group, Bioengineering Area, Biogipuzkoa Health Research Institute, 20014 San Sebastian, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Eduard","family":"Gallardo","sequence":"additional","affiliation":[{"name":"Neuromuscular Diseases Unit, Department of Neurology, Hospital de la Santa Creu i Sant Pau, 08041 Barcelona, Spain"},{"name":"Institut de Recerca Sant Pau, IR-SantPau, 08041 Barcelona, Spain"},{"name":"Center for Biomedical Network Research on Rare Diseases (CIBERER), Spanish Ministry of Science & Innovation, Carlos III 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Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5151-988X","authenticated-orcid":false,"given":"Cristina","family":"Dom\u00ednguez-Gonz\u00e1lez","sequence":"additional","affiliation":[{"name":"Center for Biomedical Network Research on Rare Diseases (CIBERER), Spanish Ministry of Science & Innovation, Carlos III Health Institute, 28029 Madrid, Spain"},{"name":"Instituto de Investigaci\u00f3n Sanitaria Hospital 12 de Octubre (imas12), 28041 Madrid, Spain"},{"name":"Neuromuscular Unit, Department of Neurology, Hospital 12 de Octubre, 28041 Madrid, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0532-2872","authenticated-orcid":false,"given":"Juan Jes\u00fas","family":"V\u00edlchez","sequence":"additional","affiliation":[{"name":"Center for Biomedical Network Research on Rare Diseases (CIBERER), Spanish Ministry of Science & Innovation, Carlos III Health Institute, 28029 Madrid, Spain"},{"name":"Neuromuscular and Ataxias Research Group, Instituto de Investigaci\u00f3n Sanitaria La Fe, 46026 Valencia, Spain"},{"name":"Neuromuscular Diseases Unit, Neurology Department, Hospital Universitari I Polit\u00e8cnic La Fe, 46026 Valencia, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0331-6222","authenticated-orcid":false,"given":"Pablo","family":"Iruzubieta","sequence":"additional","affiliation":[{"name":"Center for Biomedical Network Research on Neurodegenerative Diseases (CIBERNED), Spanish Ministry of Science & Innovation, Carlos III Health Institute, 28029 Madrid, Spain"},{"name":"Neurogenetics, RNA Biology and Therapies Group, Neurosciences Area, Biogipuzkoa Health Research Institute, 20014 San Sebastian, Spain"},{"name":"Department of Neurology and Neurosurgery, Montreal Neurological Hospital and Institute, McGill University, Montreal, QC H3A 2B4, 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