{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,11]],"date-time":"2026-04-11T11:53:54Z","timestamp":1775908434412,"version":"3.50.1"},"reference-count":43,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2019,3,12]],"date-time":"2019-03-12T00:00:00Z","timestamp":1552348800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100003196","name":"Ministero della Salute","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100003196","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["faseb.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["The FASEB Journal"],"published-print":{"date-parts":[[2019,6]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>\n                    Oculopharyngeal muscular dystrophy (OPMD) is a late\u2010onset muscle disease caused by an abnormal (GCN) triplet expansion within the polyadenylate\u2010binding protein nuclear 1 gene and consequent mRNA processing impairment and myogenic defects. Because a reduced cell proliferation potential and the consequent regeneration failure of aging muscle have been shown to be governed by lethal\u20107 (\n                    <jats:italic>let\u20107<\/jats:italic>\n                    ) microRNA\u2010mediated mechanisms, in the present study, we evaluated the role of\n                    <jats:italic>let\u20107<\/jats:italic>\n                    in the pathogenesis of OPMD. By a multidisciplinary approach, including conf ocal microscopy, Western blot, and quantitative PCR analyses on muscle biopsies from patients and unaffected individuals, we found a significant increase in\n                    <jats:italic>let\u20107<\/jats:italic>\n                    expression in OPMD muscles associated with an unusual high percentage of paired box 7\u2013positive satellite cells. Furthermore, IL\u20106, a cytokine involved in the regulation of satellite cell proliferation and differentiation and a potential target of\n                    <jats:italic>let\u20107<\/jats:italic>\n                    , was found strongly down\u2010regulated in OPMD compared with control muscles. The decrease in IL\u20106 transcript levels and protein content was also confirmed\n                    <jats:italic>in vitro<\/jats:italic>\n                    during differentiation of patients' and controls' muscle cells. Overall, our data suggest a key role of\n                    <jats:italic>let\u20107<\/jats:italic>\n                    in the regeneration and degeneration process in OPMD muscle and pointed to IL\u20106 as a potential target molecule for new therapeutic approaches for this disorder.\u2014Cappelletti, C., Galbardi, B., Bruttini, M., Salerno, F., Canioni, E., Pasanisi, M. B., Rodolico, C., Brizzi, T., Mora, M., Renieri, A., Maggi, L., Bernasconi, P., Mantegazza, R. Aging\u2010associated genes and\n                    <jats:italic>let\u20107<\/jats:italic>\n                    microRNAs: a contribution to myogenic program dysregulation in oculopharyngeal muscular dystrophy. FASEB J. 33, 7155\u20137167 (2019).\n                    <jats:ext-link xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" xlink:href=\"http:\/\/www.fasebj.org\">www.fasebj.org<\/jats:ext-link>\n                  <\/jats:p>","DOI":"10.1096\/fj.201801577rr","type":"journal-article","created":{"date-parts":[[2019,3,12]],"date-time":"2019-03-12T13:46:49Z","timestamp":1552398409000},"page":"7155-7167","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":11,"title":["Aging\u2010associated genes and\n                    <i>let\u20107<\/i>\n                    microRNAs: a contribution to myogenic program dysregulation in oculopharyngeal muscular dystrophy"],"prefix":"10.1096","volume":"33","author":[{"given":"Cristina","family":"Cappelletti","sequence":"first","affiliation":[{"name":"Neurology IV\u2013Neuroimmunology and Neuromuscular Diseases Unit Fondazione Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS) Istituto Neurologico 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