{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,4]],"date-time":"2026-10-04T08:54:34Z","timestamp":1791104074190,"version":"4.1.0"},"reference-count":55,"publisher":"Wiley","issue":"11","license":[{"start":{"date-parts":[[2011,8,12]],"date-time":"2011-08-12T00:00:00Z","timestamp":1313107200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100001677","name":"Institut National de la Sant\u00e9 et de la Recherche M\u00e9dicale","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100001677","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100005737","name":"Universit\u00e9 Pierre et Marie Curie","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100005737","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100004794","name":"Centre National de la Recherche Scientifique","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100004794","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100007393","name":"Association Fran\u00e7aise contre les Myopathies","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100007393","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":[[2011,11]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>\n                    Mutations in the lamin A\/C gene\n                    <jats:italic>(LMNA)<\/jats:italic>\n                    cause several disorders referred to as laminopathies, which include premature aging syndromes, lipodystrophy, and striated muscle disorders. There is evidence that lamin A\/C plays a role in gene expression. MicroRNAs (miRNAs) are short noncoding RNAs regulating mRNAs involved in various biological processes, including the pathophysiology of striated muscles. Here, we profiled the expression of the miRNA transcriptome in skeletal muscle from patients with\n                    <jats:italic>LMNA<\/jats:italic>\n                    \u2010related muscular dystrophy. Results show that control and patient groups can be distinguished based on their miRNA expression profile. Sixteen miRNAs are significantly dysregulated in patients compared with controls. Pathway enrichment analysis in the predicted targets of these miRNAs revealed pathways involved in muscle repair, such as MAPK, transforming growth factor\u2010\u03b2, and Wnt signaling. Interestingly, 9 of these miRNAs (hsa\u2010miR\u2010100, \u2212127\u20133p, \u2212148a, \u2212136\n                    <jats:sup>\u2217<\/jats:sup>\n                    , \u2212192, \u2212335, \u2212376c, \u2212489, and \u2212502\u20133p) are highly expressed in fetal muscle, suggesting that the fetal miRNA gene program mediates a regenerative process. Overexpression of these miRNAs in C2C12 mouse myoblasts revealed that 3 of them (miR\u2010100, \u2212192, and \u2212335) participate in muscle proliferation and differentiation. We identified target genes that likely mediate this effect, which include the calcineurin gene PPP3CA. Our findings are the first to demonstrate that miRNA expression is affected in laminopathies.\u2014Sylvius, N., Bonne, G., Straatman, K., Reddy, T., Gant, T. W., Shackleton, S. MicroRNA expression profiling in patients with lamin A\/C\u2010associated muscular dystrophy.\n                    <jats:italic>FASEB J.<\/jats:italic>\n                    25, 3966\u20133978 (2011).\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.11-182915","type":"journal-article","created":{"date-parts":[[2011,8,12]],"date-time":"2011-08-12T21:12:07Z","timestamp":1313183527000},"page":"3966-3978","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":41,"title":["MicroRNA expression profiling in patients with lamin A\/C\u2010associated muscular dystrophy"],"prefix":"10.1096","volume":"25","author":[{"given":"Nicolas","family":"Sylvius","sequence":"first","affiliation":[{"name":"Department of Biochemistry University of Leicester  Leicester UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gise'le","family":"Bonne","sequence":"additional","affiliation":[{"name":"Institut National de la Sant\u00e9 et de la Recherche M\u00e9dicale, U974  Paris France"},{"name":"Universit\u00e9 Pierre et Marie Curie\u2010Paris 6, UM 76 Centre National de la Recherche Scientifique, UMR7215, Institut de Myologie, IFR14  Paris France"},{"name":"Assistance Publique\u2010H\u00f4pitaux de Paris, Groupe Hospitalier Piti\u00e9\u2010Salp\u00eatrie're, U.F. Cardiog\u00e9n\u00e9tique et Myog\u00e9n\u00e9tique Service de Biochimie M\u00e9tabolique  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kees","family":"Straatman","sequence":"additional","affiliation":[{"name":"Centre for Core Biotechnology Services University of Leicester  Leicester UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thimma","family":"Reddy","sequence":"additional","affiliation":[{"name":"Department of Biochemistry University of Leicester  Leicester UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Timothy W.","family":"Gant","sequence":"additional","affiliation":[{"name":"Department of Genetics University of Leicester  Leicester UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sue","family":"Shackleton","sequence":"additional","affiliation":[{"name":"Department of Biochemistry University of Leicester  Leicester UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2011,8,12]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.yexcr.2007.03.028"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(02)07815-7"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1053\/euhj.1998.1145"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199912023412302"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.2004.023283"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1742-4658.2007.05697.x"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1474-9728.2004.00105.x"},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awl023"},{"key":"e_1_2_6_10_1","doi-asserted-by":"publisher","DOI":"10.1172\/JCI29042"},{"key":"e_1_2_6_11_1","doi-asserted-by":"publisher","DOI":"10.1093\/nar\/gkf587"},{"key":"e_1_2_6_12_1","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.200511149"},{"key":"e_1_2_6_13_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0403250101"},{"key":"e_1_2_6_14_1","doi-asserted-by":"publisher","DOI":"10.1021\/bi020704g"},{"key":"e_1_2_6_15_1","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.200204149"},{"key":"e_1_2_6_16_1","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.200112047"},{"key":"e_1_2_6_17_1","doi-asserted-by":"publisher","DOI":"10.4161\/cc.9.3.10511"},{"key":"e_1_2_6_18_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.tig.2006.01.003"},{"key":"e_1_2_6_19_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0710228105"},{"key":"e_1_2_6_20_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ydbio.2007.08.032"},{"key":"e_1_2_6_21_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng1725"},{"key":"e_1_2_6_22_1","doi-asserted-by":"publisher","DOI":"10.1038\/nature03817"},{"key":"e_1_2_6_23_1","doi-asserted-by":"publisher","DOI":"10.1152\/physiolgenomics.00144.2007"},{"key":"e_1_2_6_24_1","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.107.687947"},{"key":"e_1_2_6_25_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.yjmcc.2008.04.014"},{"key":"e_1_2_6_26_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M109.036541"},{"key":"e_1_2_6_27_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0708115104"},{"key":"e_1_2_6_28_1","doi-asserted-by":"publisher","DOI":"10.1096\/fj.08-128579"},{"key":"e_1_2_6_29_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.22338"},{"key":"e_1_2_6_30_1","doi-asserted-by":"publisher","DOI":"10.1038\/nprot.2006.85"},{"key":"e_1_2_6_31_1","doi-asserted-by":"publisher","DOI":"10.1186\/gb-2002-3-7-research0034"},{"key":"e_1_2_6_32_1","doi-asserted-by":"publisher","DOI":"10.1007\/978-1-60761-175-2_20"},{"key":"e_1_2_6_33_1","doi-asserted-by":"publisher","DOI":"10.1093\/bioinformatics\/btp299"},{"key":"e_1_2_6_34_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1097-2765(00)00025-3"},{"key":"e_1_2_6_35_1","doi-asserted-by":"crossref","DOI":"10.1093\/nar\/gkn725","article-title":"High\u2010throughput stem\u2010loop RT\u2010qPCR miRNA expression profiling using minute amounts of input RNA","volume":"36","author":"Mestdagh P.","year":"2008","journal-title":"Nucleic Acids Res."},{"key":"e_1_2_6_36_1","doi-asserted-by":"crossref","DOI":"10.1186\/1471-2164-9-630","article-title":"Meta\u2010analysis of expression signatures of muscle atrophy: gene interaction networks in early and late stages","volume":"9","author":"Calura E.","year":"2008","journal-title":"BMC Genomics"},{"key":"e_1_2_6_37_1","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0003750","article-title":"Gene expression profiling in limb\u2010girdle muscular dystrophy 2A","volume":"3","author":"Saenz A.","year":"2008","journal-title":"PLoS One"},{"key":"e_1_2_6_38_1","doi-asserted-by":"crossref","DOI":"10.1186\/1471-2164-10-77","article-title":"MicroRNA transcriptome profiles during swine skeletal muscle development","volume":"10","author":"McDaneld T. 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