{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,19]],"date-time":"2026-08-19T20:16:25Z","timestamp":1787170585402,"version":"build-2736575974"},"reference-count":70,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2022,4,13]],"date-time":"2022-04-13T00:00:00Z","timestamp":1649808000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100004923","name":"French Muscular Dystrophy Association","doi-asserted-by":"publisher","award":["Grant number 20002"],"award-info":[{"award-number":["Grant number 20002"]}],"id":[{"id":"10.13039\/501100004923","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100010663","name":"H2020 European Research Council","doi-asserted-by":"publisher","award":["RegeneratioNfix 280611"],"award-info":[{"award-number":["RegeneratioNfix 280611"]}],"id":[{"id":"10.13039\/100010663","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["pathsocjournals.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["The Journal of Pathology"],"published-print":{"date-parts":[[2022,7]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    Muscular dystrophies are genetic diseases characterized by chronic inflammation and fibrosis. Macrophages are immune cells that sustain muscle regeneration upon acute injury but seem deleterious in the context of chronic muscle injury such as in muscular dystrophies. Here, we observed that the number of macrophages expressing the transcription factor Nfix increases in two distinct mouse models of muscular dystrophies. We showed that the deletion of Nfix in macrophages in dystrophic mice delays the establishment of fibrosis and muscle wasting, and increases grasp force. Macrophages lacking Nfix expressed more TNF\u03b1 and less TGF\u03b21, thus promoting apoptosis of fibro\u2010adipogenic progenitors. Moreover, pharmacological treatment of dystrophic mice with a ROCK inhibitor accelerated fibrosis through the increase of Nfix expression by macrophages. Thus, we have identified Nfix as a macrophage profibrotic factor in muscular dystrophies, whose inhibition could be a therapeutic route to reduce severity of the dystrophic disease. \u00a9 2022 The Authors.\n                    <jats:italic>The Journal of Pathology<\/jats:italic>\n                    published by John Wiley &amp; Sons Ltd on behalf of The Pathological Society of Great Britain and Ireland.\n                  <\/jats:p>","DOI":"10.1002\/path.5895","type":"journal-article","created":{"date-parts":[[2022,3,17]],"date-time":"2022-03-17T08:16:26Z","timestamp":1647504986000},"page":"352-366","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":14,"title":["Selective ablation of\n                    <scp>Nfix<\/scp>\n                    in macrophages attenuates muscular dystrophy by inhibiting fibro\u2010adipogenic progenitor\u2010dependent fibrosis"],"prefix":"10.1002","volume":"257","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5194-682X","authenticated-orcid":false,"given":"Marielle","family":"Saclier","sequence":"first","affiliation":[{"name":"Department of Biosciences University of Milan  Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giuseppe","family":"Angelini","sequence":"additional","affiliation":[{"name":"Department of Biosciences University of Milan  Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chiara","family":"Bonfanti","sequence":"additional","affiliation":[{"name":"Department of Biosciences University of Milan  Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giada","family":"Mura","sequence":"additional","affiliation":[{"name":"Department of Biosciences University of Milan  Milan 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