{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,28]],"date-time":"2026-09-28T21:31:47Z","timestamp":1790631107059,"version":"4.1.0"},"reference-count":35,"publisher":"MDPI AG","issue":"6","license":[{"start":{"date-parts":[[2020,6,23]],"date-time":"2020-06-23T00:00:00Z","timestamp":1592870400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Italian Ministry of Health","award":["AIProSaB grants 02\/2013 and 03\/2016"],"award-info":[{"award-number":["AIProSaB grants 02\/2013 and 03\/2016"]}]},{"name":"Associazione Alessandra Proietti onlus IGM","award":["AIDMED onlus grant 17\/2015"],"award-info":[{"award-number":["AIDMED onlus grant 17\/2015"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cells"],"abstract":"<jats:p>Laminopathies are a wide and heterogeneous group of rare human diseases caused by mutations of the LMNA gene or related nuclear envelope genes. The variety of clinical phenotypes and the wide spectrum of histopathological changes among patients carrying an identical mutation in the LMNA gene make the prognostic process rather difficult, and classical genetic screens appear to have limited predictive value for disease development. The aim of this study was to evaluate whether a comprehensive profile of circulating cytokines may be a useful tool to differentiate and stratify disease subgroups, support clinical follow-ups and contribute to new therapeutic approaches. Serum levels of 51 pro- and anti-inflammatory molecules, including cytokines, chemokines and growth factors, were quantified by a Luminex multiple immune-assay in 53 patients with muscular laminopathy (Musc-LMNA), 10 with non-muscular laminopathy, 22 with other muscular disorders and in 35 healthy controls. Interleukin-17 (IL-17), granulocyte colony-stimulating factor (G-CSF) and transforming growth factor beta (TGF-\u03b22) levels significantly discriminated Musc-LMNA from controls; interleukin-1\u03b2 (IL-1\u03b2), interleukin-4 (IL-4) and interleukin-8 (IL-8) were differentially expressed in Musc-LMNA patients compared to those with non-muscular laminopathies, whereas IL-17 was significantly higher in Musc-LMNA patients with muscular and cardiac involvement. These findings support the hypothesis of a key role of the immune system in Musc-LMNA and emphasize the potential use of cytokines as biomarkers for these disorders.<\/jats:p>","DOI":"10.3390\/cells9061532","type":"journal-article","created":{"date-parts":[[2020,6,24]],"date-time":"2020-06-24T07:14:19Z","timestamp":1592982859000},"page":"1532","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":12,"title":["Cytokine Profile in Striated Muscle Laminopathies: New Promising Biomarkers for Disease Prediction"],"prefix":"10.3390","volume":"9","author":[{"given":"Cristina","family":"Cappelletti","sequence":"first","affiliation":[{"name":"Neurology IV-Neuroimmunology and Neuromuscular Diseases Unit, Fondazione IRCCS Istituto Neurologico Carlo Besta, 20133 Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Irene","family":"Tramacere","sequence":"additional","affiliation":[{"name":"Department of Research and Clinical Development, Fondazione IRCCS Istituto Neurologico \u201cCarlo Besta\u201d, 20133 Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paola","family":"Cavalcante","sequence":"additional","affiliation":[{"name":"Neurology IV-Neuroimmunology and Neuromuscular Diseases Unit, Fondazione IRCCS Istituto Neurologico Carlo Besta, 20133 Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elisa","family":"Schena","sequence":"additional","affiliation":[{"name":"CNR Institute of Molecular Genetics, Unit of Bologna, 40136 Bologna, Italy"},{"name":"Endocrinology Unit, Department of Medical &amp; Surgical Sciences, Alma Mater Studiorum University of Bologna, S Orsola-Malpighi Hospital, 40138 Bologna, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0925-7158","authenticated-orcid":false,"given":"Luisa","family":"Politano","sequence":"additional","affiliation":[{"name":"Cardiomyology and Medical Genetics, Department of Experimental Medicine, University of Campania, Luigi Vanvitelli, 80138 Naples, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3245-9169","authenticated-orcid":false,"given":"Nicola","family":"Carboni","sequence":"additional","affiliation":[{"name":"Neurology Department, Hospital San Francesco of Nuoro, 08100 Nuoro, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alessandra","family":"Gambineri","sequence":"additional","affiliation":[{"name":"Endocrinology Unit, Department of Clinical and Medical Science, S. Orsola-Malpighi Hospital, University of Bologna, 40138 Bologna, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Adele","family":"D\u2019Amico","sequence":"additional","affiliation":[{"name":"Unit of Neuromuscular and Neurodegenerative Disorders, Bambino Ges\u00f9 Children\u2019s Hospital, 00165 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7756-9567","authenticated-orcid":false,"given":"Lucia","family":"Ruggiero","sequence":"additional","affiliation":[{"name":"Department of Neurosciences and Reproductive and Odontostomatologic Sciences, University Federico II, 80137 Naples, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giulia","family":"Ricci","sequence":"additional","affiliation":[{"name":"Department of Clinical and Experimental Medicine, Neurological Clinic, 56126 Pisa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gabriele","family":"Siciliano","sequence":"additional","affiliation":[{"name":"Department of Clinical and Experimental Medicine, Neurological Clinic, 56126 Pisa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9820-4815","authenticated-orcid":false,"given":"Giuseppe","family":"Boriani","sequence":"additional","affiliation":[{"name":"Cardiology Division, Department of Biomedical, Metabolic and Neural Sciences, University of Modena and Reggio Emilia, Policlinico di Modena, 41121 Modena, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tiziana Enrica","family":"Mongini","sequence":"additional","affiliation":[{"name":"Department of Neurosciences \u201cRita Levi Montalcini\u201d, University of Turin, 10124 Turin, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8352-1087","authenticated-orcid":false,"given":"Liliana","family":"Vercelli","sequence":"additional","affiliation":[{"name":"Department of Neurosciences \u201cRita Levi Montalcini\u201d, University of Turin, 10124 Turin, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0295-0440","authenticated-orcid":false,"given":"Elena","family":"Biagini","sequence":"additional","affiliation":[{"name":"Azienda Ospedaliero Universitaria - Policlinico di St. Orsola, Cardiology Unit, Cardio-Thoracic-Vascular Department, 40138 Bologna, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matteo","family":"Ziacchi","sequence":"additional","affiliation":[{"name":"Azienda Ospedaliero Universitaria - Policlinico di St. Orsola, Cardiology Unit, Cardio-Thoracic-Vascular Department, 40138 Bologna, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria Rosaria","family":"D\u2019Apice","sequence":"additional","affiliation":[{"name":"Medical Genetics Unit, Policlinico Tor Vergata University Hospital, 00133 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7103-8722","authenticated-orcid":false,"given":"Giovanna","family":"Lattanzi","sequence":"additional","affiliation":[{"name":"CNR Institute of Molecular Genetics, Unit of Bologna, 40136 Bologna, Italy"},{"name":"IRCCS Istituto Ortopedico Rizzoli, 40136 Bologna, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Renato","family":"Mantegazza","sequence":"additional","affiliation":[{"name":"Neurology IV-Neuroimmunology and Neuromuscular Diseases Unit, Fondazione IRCCS Istituto Neurologico Carlo Besta, 20133 Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0932-5173","authenticated-orcid":false,"given":"Lorenzo","family":"Maggi","sequence":"additional","affiliation":[{"name":"Neurology IV-Neuroimmunology and Neuromuscular Diseases Unit, Fondazione IRCCS Istituto Neurologico Carlo Besta, 20133 Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0869-2104","authenticated-orcid":false,"given":"Pia","family":"Bernasconi","sequence":"additional","affiliation":[{"name":"Neurology IV-Neuroimmunology and Neuromuscular Diseases Unit, Fondazione IRCCS Istituto Neurologico Carlo Besta, 20133 Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2020,6,23]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"427","DOI":"10.4161\/nucl.36289","article-title":"Diverse lamin-dependent mechanisms interact to control chromatin dynamics. 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