Antagonistic effect of the matricellular signaling protein CCN3 on TGF-β- and Wnt-mediated fibrillinogenesis in systemic sclerosis and Marfan syndrome

Abnormal fibrillinogenesis is associated with connective tissue disorders (CTDs), including Marfan syndrome (MFS), systemic sclerosis (SSc) and Tight-skin (Tsk) mice. We have previously shown that TGF-β and Wnt stimulate fibrillin-1 assembly and that fibrillin-1 and the developmental regulator CCN3...

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Autores principales: Lemaire, Raphael, Farina, Giuseppina, Bayle, Julie, Dimarzio, Michael, Pendergrass, Sarah A., Milano, Ausra, Whitfield, Michael L., Lafyatis, Robert
Formato: Artigo
Lenguaje:Inglês
Publicado: 2010
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Article
Acceso en línea:https://ncbi.nlm.nih.gov/pmc/articles/PMC3071475/
https://ncbi.nlm.nih.gov/pubmed/20182440
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1038/jid.2010.15
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