{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,23]],"date-time":"2026-10-23T20:26:30Z","timestamp":1792787190342,"version":"4.5.0"},"reference-count":56,"publisher":"American Association for the Advancement of Science (AAAS)","issue":"42","funder":[{"name":"French National Research Agency","award":["ANR-19-CE13-0010 and ANR-19-CE14-0008"],"award-info":[{"award-number":["ANR-19-CE13-0010 and ANR-19-CE14-0008"]}],"id":[{"id":"https:\/\/ror.org\/00rbzpz17","id-type":"ROR","asserted-by":"crossref"}]},{"name":"Association Fran\u00e7aise contre les Myopathies","award":["PROJECT22946"],"award-info":[{"award-number":["PROJECT22946"]}],"id":[{"id":"https:\/\/ror.org\/0162y2387","id-type":"ROR","asserted-by":"crossref"}]},{"name":"AFM-Telethon","award":["28851"],"award-info":[{"award-number":["28851"]}]},{"name":"Genetic Aortic Disorders Association Canada"},{"name":"LABEX REVIVE","award":["ANR-10-LABX-73"],"award-info":[{"award-number":["ANR-10-LABX-73"]}]},{"name":"NWO"}],"content-domain":{"domain":["www.science.org"],"crossmark-restriction":true},"short-container-title":["Sci. Adv."],"published-print":{"date-parts":[[2026,10,16]]},"abstract":"<jats:p>\n                    Diverse biological mechanisms regulate muscle stem cell quiescence, including inhibition of differentiation, adhesion, and translational control. Here, we identify the matrix glycoprotein Fibrillin-1 (FBN1) as a Notch-regulated, cell-autonomous effector, essential for maintaining quiescence. Known for its causal role in Marfan syndrome (MFS), our work positions FBN1 as a critical niche component that protects muscle stem cells from aberrant growth factor signaling. Targeted deletion of\n                    <jats:italic toggle=\"yes\">Fbn1<\/jats:italic>\n                    in muscle stem cells leads to dose-dependent quiescence defects, characterized by loss of cellular projections, depletion of the stem cell pool, and progressive decline in muscle function. Consistently, human MFS muscle biopsies show abnormally activated satellite cells, implicating stem cell imbalance in the development of MFS-associated myopathy. Mechanistically, the loss of FBN1 up-regulates transforming growth factor\u2013\u03b2 (TGF\u03b2) signaling, and pharmacological blockade of TGF\u03b2 signaling rescues the cellular and physiological defects of FBN1-deficient muscle. These findings reveal a new quiescence-preserving mechanism through extracellular matrix\u2013mediated shielding from mitogenic signals and position stem cell dysfunction as a driver of MFS myopathy.\n                  <\/jats:p>","DOI":"10.1126\/sciadv.aeb8190","type":"journal-article","created":{"date-parts":[[2026,10,16]],"date-time":"2026-10-16T17:58:53Z","timestamp":1792173533000},"update-policy":"https:\/\/doi.org\/10.34133\/aaas_crossmark","source":"Crossref","is-referenced-by-count":0,"title":["Muscle stem cells produce a protective Fibrillin-1 matrix to prevent precocious activation"],"prefix":"10.1126","volume":"12","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2455-6321","authenticated-orcid":true,"given":"Eleni","family":"Chrysostomou","sequence":"first","affiliation":[{"name":"Universit\u00e9 Paris Cit\u00e9, INSERM U1151, CNRS UMR8253, Institut Necker Enfants Malades (INEM), 75015 Paris, France."},{"name":"Univ Paris Est Cr\u00e9teil, INSERM, IMRB, EnvA, APHP, EFS, 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