{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T12:30:57Z","timestamp":1791203457833,"version":"4.2.0"},"reference-count":44,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2007,8,20]],"date-time":"2007-08-20T00:00:00Z","timestamp":1187568000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Journal Cellular Physiology"],"published-print":{"date-parts":[[2007,11]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Fibrillin\u2010rich microfibrils are specialized extracellular matrix assemblies that endow connective tissues with mechanical stability and elastic properties, and that participate in the regulation of organ formation, growth and homeostasis. Their physiological importance is underscored by the complex spectrum of clinical manifestations associated with mutations of fibrillin\u20101 and fibrillin\u20102 in Marfan syndrome (MFS) and congenital contractural arachnodactyly, respectively. Early evidence suggested that fibrillin\u20101 mutations in MFS lead to loss of tissue integrity by perturbing microfibril assembly and function. Recent studies in genetically targeted mice have however revealed that fibrillin\u20101 and fibrillin\u20102 mutations perturb signaling events mediated by TGF\u2010\u03b2 superfamily members. As such, these studies have established a new biological paradigm whereby fibrillin\u2010rich microfibrils are structural networks that specify the local concentration and timely release of signaling molecules during morphogenesis and tissue remodeling. This review summarizes our current understanding of the role of fibrillin\u2010rich microfibrils in development and disease, as well as exciting new applications in the clinical management of MFS and related connective tissue disorders. J. Cell. Physiol. 213: 326\u2013330, 2007. \u00a9 2007 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/jcp.21189","type":"journal-article","created":{"date-parts":[[2007,8,20]],"date-time":"2007-08-20T22:56:05Z","timestamp":1187650565000},"page":"326-330","source":"Crossref","is-referenced-by-count":94,"title":["Fibrillin\u2010rich microfibrils: Structural determinants of morphogenetic and homeostatic events"],"prefix":"10.1002","volume":"213","author":[{"given":"Francesco","family":"Ramirez","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Harry 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