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However, the underlying cellular and molecular mechanisms remain poorly understood. Here, we investigated muscle regeneration in MFS mouse models by analyzing the influence of the fibrotic niche on satellite cell function.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>In vivo, ex vivo, and in vitro experiments were performed. In addition, we evaluated the effect of the pharmacological inhibition of fibrosis using Ang\u2010(1\u20137) on regenerating skeletal muscles of MFS mice.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The skeletal muscle of MFS mice shows an increased accumulation of collagen fibers (81.2%), number of fibroblasts (157.1%), and Smad2\/3 signaling (110.5%), as well as an aberrant number of fibro\u2010adipogenic progenitor cells in response to injury compared with wild\u2010type mice. There was an increased number of proinflammatory and anti\u2010inflammatory macrophages (3.6\u2010 and 3.1\u2010fold, respectively) in regenerating muscles of wild\u2010type mice, but not in the regenerating muscles of MFS mice. Our data show that proliferation and differentiation of satellite cells are altered (<jats:italic>p<\/jats:italic>\u2009\u2264\u20090.05) in MFS mice. Myoblast transplantation assay revealed that the regenerating muscles from MFS mice have reduced satellite cell self\u2010renewal capacity (74.7%). In addition, we found that treatment with Ang\u2010(1\u20137) reduces fibrosis (71.6%) and ameliorates satellite cell dysfunction (<jats:italic>p<\/jats:italic>\u2009\u2264\u20090.05) and muscle contractile function (<jats:italic>p<\/jats:italic>\u2009\u2264\u20090.05) in MFS mice.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>The fibrotic niche, caused by <jats:italic>Fbn1<\/jats:italic> mutations, reduces the myogenic potential of satellite cells, affecting structural and functional muscle regeneration. In addition, the fibrosis inhibitor Ang\u2010(1\u20137) partially counteracts satellite cell abnormalities and restores myofiber size and contractile force in regenerating muscles.<\/jats:p><\/jats:sec>","DOI":"10.1111\/apha.13889","type":"journal-article","created":{"date-parts":[[2022,9,27]],"date-time":"2022-09-27T08:44:44Z","timestamp":1664268284000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":13,"title":["The fibrotic niche impairs satellite cell function and muscle regeneration in mouse models of Marfan syndrome"],"prefix":"10.1111","volume":"237","author":[{"given":"Meiricris","family":"Tomaz da Silva","sequence":"first","affiliation":[{"name":"Department of Anatomy, Institute of Biomedical Sciences University of S\u00e3o Paulo  S\u00e3o Paulo Brazil"},{"name":"Lillehei Heart Institute and Department of Pediatrics University of Minnesota  Minneapolis Minnesota USA"},{"name":"Department of Pharmacological and 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