{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,11,8]],"date-time":"2025-11-08T12:41:30Z","timestamp":1762605690597},"reference-count":50,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2009,2,6]],"date-time":"2009-02-06T00:00:00Z","timestamp":1233878400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J Exp Zool Pt B"],"published-print":{"date-parts":[[2009,7,15]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Elastic system fibers are composed of two distinct elements, elastin, which is an amorphous component crosslinked in the core, and microfibril, localized in the periphery of elastin. As microfibrillar proteins, fibrillins, microfibril\u2010associated glycoproteins (MAGPs), latent TGF\u2010\u03b2\u2010binding proteins (LTBPs), microfibril\u2010associated proteins (MFAPs), and fibulins are known. Fibrillin\u20101 is a major microfibrillar protein and characterized by calcium binding EGF\u2010like (cbEGF) domain. Association between fibrillin\u20101 and TGF\u2010\u03b2 is a recent topic of this field and this interaction is known to inactivate and target TGF\u2010\u03b2 action. <jats:italic>FBN1<\/jats:italic> encoding fibrillin\u20101 is a responsible gene for Marfan syndrome type 1 (MIM \u266f154700), characterized by increased height and long limbs, ectopia lentis, and cardiovascular disorders, such as mitral valve prolapse and aortic dilation and regurgitation. Animal models suggest that the abnormal TGF\u2010\u03b2 signaling is underlying as the pathogenesis of these conditions. Besides skeletal, ocular and cardiovascular conditions, severe periodontitis is frequently seen in affected patients. To clarify the unknown function of elastic system fibers in the periodontal ligament (PDL), PDL\u2010cells were isolated from a Marfan syndrome type 1 patient who was with the severe periodontitis and had a mutation in one of the cbEGF domain of fibrillin\u20101. These results suggested that wild\u2010type fibrillin\u20101 was required for the normal cell alignment and tissue architecture of PDLs. Evidences are now accumulated to suggest that fibrillin\u20101 is one of the molecule involved in the interaction between cell and extracellular matrix. <jats:italic>J. Exp. Zool. (Mol. Dev. Evol.) 312B:503\u2013509, 2009<\/jats:italic>. \u00a9 2009 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/jez.b.21278","type":"journal-article","created":{"date-parts":[[2009,2,7]],"date-time":"2009-02-07T07:53:11Z","timestamp":1233993191000},"page":"503-509","source":"Crossref","is-referenced-by-count":29,"title":["Marfan syndrome and its disorder in periodontal 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