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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...
Autores principales: | Robertson, Ian B., Dias, Hans F., Osuch, Isabelle H., Lowe, Edward D., Jensen, Sacha A., Redfield, Christina, Handford, Penny A. |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Cell Press
2017
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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 |
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