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The N-Terminal Region of Fibrillin-1 Mediates a Bipartite Interaction with LTBP1

Fibrillin-1 (FBN1) mutations associated with Marfan syndrome lead to an increase in transforming growth factor β (TGF-β) activation in connective tissues resulting in pathogenic changes including aortic dilatation and dissection. Since FBN1 binds latent TGF-β binding proteins (LTBPs), the major rese...

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Detalles Bibliográficos
Autores principales: Robertson, Ian B., Dias, Hans F., Osuch, Isabelle H., Lowe, Edward D., Jensen, Sacha A., Redfield, Christina, Handford, Penny A.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cell Press 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5548924/
https://www.ncbi.nlm.nih.gov/pubmed/28669633
http://dx.doi.org/10.1016/j.str.2017.06.003