{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,1]],"date-time":"2026-07-01T22:35:09Z","timestamp":1782945309809,"version":"3.54.5"},"reference-count":56,"publisher":"MDPI AG","issue":"4","license":[{"start":{"date-parts":[[2025,2,8]],"date-time":"2025-02-08T00:00:00Z","timestamp":1738972800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100004587","name":"Instituto de Salud Carlos III","doi-asserted-by":"publisher","award":["PI19\/00377 (2019) and FIS PI22\/00142  (2022) grants"],"award-info":[{"award-number":["PI19\/00377 (2019) and FIS PI22\/00142  (2022) grants"]}],"id":[{"id":"10.13039\/501100004587","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Junta de Andalucia","award":["CTS-5725, PY18-850 and UPO-FEDER 2018 (UPO-1380614)"],"award-info":[{"award-number":["CTS-5725, PY18-850 and UPO-FEDER 2018 (UPO-1380614)"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>One of the most prevalent types of congenital myopathy is nemaline myopathy (NM), which is recognized by histopathological examination of muscle fibers for the presence of \u201cnemaline bodies\u201d (rods). Mutations in the actin alpha 1 (ACTA1) and nebulin (NEB) genes result in the most prevalent types of NM. Muscle weakness and hypotonia are the main clinical characteristics of this disease. Unfortunately, the pathogenetic mechanisms are still unknown, and there is no cure. In previous work, we showed that actin filament polymerization defects in patient-derived fibroblasts were associated with mitochondrial dysfunction. In this manuscript, we examined the pathophysiological consequences of mitochondrial dysfunction in patient-derived fibroblasts. We analyzed iron and lipofuscin accumulation and lipid peroxidation both at the cellular and mitochondrial level. We found that fibroblasts derived from patients harboring ACTA1 and NEB mutations showed intracellular iron and lipofuscin accumulation, increased lipid peroxidation, and altered expression levels of proteins involved in iron metabolism. Furthermore, we showed that actin polymerization inhibition in control cells recapitulates the main pathological alterations of mutant nemaline cells. Our results indicate that mitochondrial dysfunction is associated with iron metabolism dysregulation, leading to iron\/lipofuscin accumulation and increased lipid peroxidation.<\/jats:p>","DOI":"10.3390\/ijms26041434","type":"journal-article","created":{"date-parts":[[2025,2,10]],"date-time":"2025-02-10T04:44:03Z","timestamp":1739162643000},"page":"1434","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Iron Accumulation and Lipid Peroxidation in Cellular Models of Nemaline Myopathies"],"prefix":"10.3390","volume":"26","author":[{"ORCID":"https:\/\/orcid.org\/0009-0005-5906-1662","authenticated-orcid":false,"given":"Alejandra","family":"L\u00f3pez-Cabrera","sequence":"first","affiliation":[{"name":"Centro Andaluz de Biolog\u00eda del Desarrollo (CABD-CSIC-Universidad Pablo de Olavide), 41013 Sevilla, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roc\u00edo","family":"Pi\u00f1ero-P\u00e9rez","sequence":"additional","affiliation":[{"name":"Centro Andaluz de Biolog\u00eda del Desarrollo (CABD-CSIC-Universidad Pablo de Olavide), 41013 Sevilla, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M\u00f3nica","family":"\u00c1lvarez-C\u00f3rdoba","sequence":"additional","affiliation":[{"name":"Centro Andaluz de Biolog\u00eda del Desarrollo (CABD-CSIC-Universidad Pablo de Olavide), 41013 Sevilla, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5209-370X","authenticated-orcid":false,"given":"Paula","family":"Cilleros-Holgado","sequence":"additional","affiliation":[{"name":"Centro Andaluz de Biolog\u00eda del Desarrollo (CABD-CSIC-Universidad Pablo de Olavide), 41013 Sevilla, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David","family":"G\u00f3mez-Fern\u00e1ndez","sequence":"additional","affiliation":[{"name":"Centro Andaluz de Biolog\u00eda del Desarrollo (CABD-CSIC-Universidad Pablo de Olavide), 41013 Sevilla, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Diana","family":"Reche-L\u00f3pez","sequence":"additional","affiliation":[{"name":"Centro Andaluz de Biolog\u00eda del Desarrollo (CABD-CSIC-Universidad Pablo de Olavide), 41013 Sevilla, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ana","family":"Romero-Gonz\u00e1lez","sequence":"additional","affiliation":[{"name":"Centro Andaluz de Biolog\u00eda del Desarrollo (CABD-CSIC-Universidad Pablo de Olavide), 41013 Sevilla, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jos\u00e9 Manuel","family":"Romero-Dom\u00ednguez","sequence":"additional","affiliation":[{"name":"Centro Andaluz de Biolog\u00eda del Desarrollo (CABD-CSIC-Universidad Pablo de Olavide), 41013 Sevilla, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1640-7887","authenticated-orcid":false,"given":"Mario","family":"de la Mata","sequence":"additional","affiliation":[{"name":"Departamento de Fisiolog\u00eda, Facultad de Ciencias de la Salud, Universidad de Granada, 51001 Ceuta, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roc\u00edo M.","family":"de Pablos","sequence":"additional","affiliation":[{"name":"Departamento de Bioqu\u00edmica y Biolog\u00eda Molecular, Facultad de Farmacia, Universidad de Sevilla, 41012 Sevilla, Spain"},{"name":"Instituto de Biomedicina de Sevilla (IBiS), Hospital Universitario Virgen del Roc\u00edo (HUVR)-CSIC-Universidad de Sevilla, 41013 Sevilla, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1531-550X","authenticated-orcid":false,"given":"Susana","family":"Gonz\u00e1lez-Granero","sequence":"additional","affiliation":[{"name":"Laboratory of Comparative Neurobiology, Cavanilles Institute of Biodiversity and Evolutionary Biology, University of Valencia and CIBERNED-ISCIII, 46980 Valencia, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9872-6499","authenticated-orcid":false,"given":"Jos\u00e9 Manuel","family":"Garc\u00eda-Verdugo","sequence":"additional","affiliation":[{"name":"Laboratory of Comparative Neurobiology, Cavanilles Institute of Biodiversity and Evolutionary Biology, University of Valencia and CIBERNED-ISCIII, 46980 Valencia, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9705-1469","authenticated-orcid":false,"given":"Jos\u00e9 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