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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Bibliographic Details
Main Authors: Robertson, IB, Dias, HF, Osuch, IH, Lowe, ED, Jensen, SA, Redfield, C, Handford, PA
Format: Journal article
Language:English
Published: Cell Press 2017