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. Author manuscript; available in PMC: 2019 Oct 1.
Published in final edited form as: Matrix Biol. 2017 Aug 4;71-72:82–89. doi: 10.1016/j.matbio.2017.07.004

Figure 1.

Figure 1

(A) Original disease model of a linear relationship between AT1r activation and TGFβ signaling derived from the characterization of TAA in Fbn1C1039G/+ mice (modified from Habashi et al, 2006 and Holm et al, 2011). (B) Revised model of TAA progression derived from studies of Fbn1mgR/mgR mice (modified from Cook et al, 2015). (C) Model of medial degeneration during TAA progression derived from the analysis of MFS-iPSCs differentiated into vascular SMCs (modified from Granata et al, 2017).