[HTML][HTML] p38 MAPK is an early determinant of promiscuous Smad2/3 signaling in the aortas of fibrillin-1 (Fbn1)-null mice
L Carta, S Smaldone, L Zilberberg, D Loch… - Journal of Biological …, 2009 - ASBMB
Excessive transforming growth factor-β (TGF-β) signaling characterizes the progression of
aortic aneurysm in mouse models of Marfan syndrome, a systemic disorder of the connective
tissue that is caused by mutations in the gene encoding the extracellular matrix protein
fibrillin-1. Fibrillin-1 mutations are believed to promote abnormal Smad2/3 signaling by
impairing the sequestration of latent TGF-β complexes into the extracellular matrix. Here we
report that promiscuous Smad2/3 signaling is the cell-autonomous phenotype of primary …
aortic aneurysm in mouse models of Marfan syndrome, a systemic disorder of the connective
tissue that is caused by mutations in the gene encoding the extracellular matrix protein
fibrillin-1. Fibrillin-1 mutations are believed to promote abnormal Smad2/3 signaling by
impairing the sequestration of latent TGF-β complexes into the extracellular matrix. Here we
report that promiscuous Smad2/3 signaling is the cell-autonomous phenotype of primary …
