{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,30]],"date-time":"2026-04-30T00:58:52Z","timestamp":1777510732401,"version":"3.51.4"},"reference-count":33,"publisher":"SAGE Publications","issue":"5","license":[{"start":{"date-parts":[[2022,10,1]],"date-time":"2022-10-01T00:00:00Z","timestamp":1664582400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Cellular Reprogramming"],"published-print":{"date-parts":[[2022,10,1]]},"abstract":"<jats:p>\n                    Mitochondrial diseases are a heterogeneous group of rare genetic disorders caused by mutations in nuclear or mitochondrial DNA (mtDNA). These diseases are frequently multisystemic, although mainly affect tissues that require large amounts of energy such as the brain. Mutations in mitochondrial transfer RNA (mt-tRNA) lead to defects in protein translation that may compromise some or all mtDNA-encoded proteins. Mitochondrial Encephalomyopathy, Lactic Acidosis and Stroke-like episodes (MELAS) syndrome is mainly caused by the m.3243A&gt;G mutation in the mt-tRNA\n                    <jats:sup>Leu(UUR)<\/jats:sup>\n                    (\n                    <jats:italic toggle=\"yes\">MT-TL1<\/jats:italic>\n                    ) gene. Owing to the lack of proper animal models, several cellular models have been developed to study the disease, providing insight in the pathophysiological mechanisms of MELAS. In this study, we show a successful direct conversion of MELAS patient-derived fibroblasts into induced neurons (iNs) for the first time, as well as an electrophysiological characterization of iNs cocultured with astrocytes. In addition, we performed bioenergetics analysis to study the consequences of m.3243A&gt;G mutation in this neuronal model of MELAS syndrome.\n                  <\/jats:p>","DOI":"10.1089\/cell.2022.0055","type":"journal-article","created":{"date-parts":[[2022,7,8]],"date-time":"2022-07-08T12:34:46Z","timestamp":1657283686000},"page":"294-303","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":6,"title":["Modeling Mitochondrial Encephalomyopathy, Lactic Acidosis, and Stroke-Like Episodes Syndrome Using Patient-Derived Induced Neurons Generated by Direct Reprogramming"],"prefix":"10.1177","volume":"24","author":[{"given":"Suleva","family":"Povea-Cabello","sequence":"first","affiliation":[{"name":"Centro Andaluz de Biolog\u00eda del Desarrollo (CABD-CSIC-Universidad Pablo de Olavide), and Centro de Investigaci\u00f3n Biom\u00e9dica en Red: Enfermedades Raras, Instituto de Salud Carlos III, Sevilla, Spain."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4728-201X","authenticated-orcid":false,"given":"Marina","family":"Villanueva-Paz","sequence":"additional","affiliation":[{"name":"Unidad de Gesti\u00f3n Cl\u00ednica de Gastroenterolog\u00eda, Servicio de Farmacolog\u00eda Cl\u00ednica, Instituto de Investigaci\u00f3n Biom\u00e9dica de M\u00e1laga-IBIMA, Hospital Universitario Virgen de la Victoria, Universidad de M\u00e1laga, M\u00e1laga, Spain."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Irene","family":"Villal\u00f3n-Garc\u00eda","sequence":"additional","affiliation":[{"name":"Centro Andaluz de Biolog\u00eda del Desarrollo (CABD-CSIC-Universidad Pablo de Olavide), and Centro de Investigaci\u00f3n Biom\u00e9dica en Red: Enfermedades Raras, Instituto de Salud Carlos III, Sevilla, Spain."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marta","family":"Talaver\u00f3n-Rey","sequence":"additional","affiliation":[{"name":"Centro Andaluz de Biolog\u00eda del Desarrollo (CABD-CSIC-Universidad Pablo de Olavide), and Centro de Investigaci\u00f3n Biom\u00e9dica en Red: Enfermedades Raras, Instituto de Salud Carlos III, Sevilla, Spain."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M\u00f3nica","family":"\u00c1lvarez-Cordoba","sequence":"additional","affiliation":[{"name":"Centro Andaluz de Biolog\u00eda del Desarrollo (CABD-CSIC-Universidad Pablo de Olavide), and Centro de Investigaci\u00f3n Biom\u00e9dica en Red: Enfermedades Raras, Instituto de Salud Carlos III, Sevilla, Spain."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Juan M.","family":"Su\u00e1rez-Rivero","sequence":"additional","affiliation":[{"name":"Centro Andaluz de Biolog\u00eda del Desarrollo (CABD-CSIC-Universidad Pablo de Olavide), and Centro de Investigaci\u00f3n Biom\u00e9dica en Red: Enfermedades Raras, Instituto de Salud Carlos III, Sevilla, Spain."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mar\u00eda \u00c1ngeles","family":"Montes","sequence":"additional","affiliation":[{"name":"Departamento de Fisiolog\u00eda, Anatom\u00eda y Biolog\u00eda Celular, Universidad Pablo de Olavide, Sevilla, Spain."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Antonio","family":"Rodr\u00edguez-Moreno","sequence":"additional","affiliation":[{"name":"Laboratorio de Neurociencia Celular y Plasticidad, Departamento de Fisiolog\u00eda, Anatom\u00eda y Biolog\u00eda Celular, Universidad Pablo de Olavide, Sevilla, Spain."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5295-0169","authenticated-orcid":false,"given":"Yuniesky","family":"Andrade-Talavera","sequence":"additional","affiliation":[{"name":"Laboratorio de Neurociencia Celular y Plasticidad, Departamento 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