dc.contributor.author | Wipff, Julien | - |
dc.contributor.author | Allanore, Yannick | - |
dc.contributor.author | Boileau, Catherine | - |
dc.date.accessioned | 2015-04-29T07:32:42Z | |
dc.date.available | 2015-04-29T07:32:42Z | |
dc.date.issued | 2009 | fr_FR |
dc.identifier.citation | Wipff, Julien ; Allanore, Yannick ; Boileau, Catherine ; Interactions entre la Fibrilline-1 et le TGF-β, Med Sci (Paris), 2009, Vol. 25, N° 2; p. 161-167 ; DOI : 10.1051/medsci/2009252161 | fr_FR |
dc.identifier.issn | 1958-5381 | fr_FR |
dc.identifier.uri | http://hdl.handle.net/10608/6579 | |
dc.description.abstract | La fibrilline-1 (FBN-1) est le principal composant des microfibrilles de la matrice extracellulaire (MEC), soutien architectural des tissus qui participe également à la régulation de différentes cytokines et facteurs de croissance. De nombreux arguments plaident en faveur d’une relation étroite entre la FBN-1 et le TGF-β (transforming growth factor β), prototype des cytokines profibrosantes. Cette interaction pourrait participer à la physiopathologie du syndrome de Marfan et de la sclérodermie systémique. Mieux comprendre les relations entre la FBN-1 et le TGF-β est ainsi un enjeu primordial dans les pathologies matricielles. Cela pourrait conduire à repenser le cadre nosologique de celles-ci en les intégrant dans les « signalopathies » TGF-β. Cette nouvelle approche pourrrait ouvrir des perspectives thérapeutiques innovantes. | fr |
dc.description.abstract | Fibrillin-1 (FBN-1) is the main component of the 10-12 nm microfibrils found in the extracellular matrix (ECM). ECM dysplays a structural role in the tissue-specific organization and takes part in the regulation of various cytokines and growth factors. A growing body of evidences supports a narrow relationship between FBN-1 and TGF-β. Homology between FBN-1 and latent TGF-β (LTGF) allows microfibrills to be a reservoir for this cytokine. The Marfan syndrome (MFS), a prototypic fibrillinopathy, highlights these relationships as it relates to 2 major genes that are FBN1 and TGF-β type II receptor (TGFBR2) genes. In a mouse model of MFS, an up-regulation of the TGF-β pathway is partly responsible for the phenotype. This FBN-1/TGF-β relationship may play also a role in systemic sclerosis (SSc), a multigenic disease characterized by excessive generalised ECM deposit. Indeed, two related animal models results from both gene mutations: the Tight Skin 1 mouse is due to a partial in-frame duplication of the Fbn1 gene and another model conditionally overexpresses TGF-β type I receptor. A better understanding of FBN-1/TGF-β relationship appears of great importance in fibrillinopathies: it may allow reconsidering the nosologic framework of these diseases including the TGF-β signalopathies and could lead to innovative therapeutic strategies. | en |
dc.language.iso | fr | fr_FR |
dc.publisher | EDK | fr_FR |
dc.relation.ispartof | M/S revues : Synthèse | fr_FR |
dc.rights | Article en libre accès | fr |
dc.rights | Médecine/Sciences - Inserm - SRMS | fr |
dc.source | M/S. Médecine sciences [ISSN papier : 0767-0974 ; ISSN numérique : 1958-5381], 2009, Vol. 25, N° 2; p. 161-167 | fr_FR |
dc.subject.mesh | Matrice extracellulaire | fr |
dc.subject.mesh | Homéostasie | fr |
dc.subject.mesh | Humains | fr |
dc.subject.mesh | Syndrome de Marfan | fr |
dc.subject.mesh | Protéines des microfilaments | fr |
dc.subject.mesh | Protein-Serine-Threonine Kinases | fr |
dc.subject.mesh | Récepteurs TGF-bêta | fr |
dc.subject.mesh | Sclérodermie systémique | fr |
dc.subject.mesh | Facteur de croissance transformant bêta | fr |
dc.title | Interactions entre la Fibrilline-1 et le TGF-β : Conséquences en pathologie humaine | fr |
dc.title.alternative | FBN-1/TGF-β relationships and fibrillopathies | en |
dc.type | Article | fr_FR |
dc.contributor.affiliation | Inserm U781, Université Paris Descartes, Hôpital Necker, 149, rue de Sèvres, 75015 Paris, France. | fr_FR |
dc.contributor.affiliation | Service de Rhumatologie A, Université Paris Descartes, Hôpital Cochin, AP-HP, 27, rue du Faubourg Saint Jacques, 75014 Paris, France | fr_FR |
dc.contributor.affiliation | Laboratoire de Biochimie, d’Hormonologie et de Génétique Moléculaire, UVSQ, Hôpital Ambroise Paré, 92100 Boulogne-Billancourt, France | fr_FR |
dc.identifier.doi | 10.1051/medsci/2009252161 | fr_FR |
dc.identifier.pmid | 19239848 | fr_FR |