Tgfbr2 f/f mice to test the hypothesis that aortic disease attributable to mutant fibrillin-1 is prevented by inhibition of TGF-β signaling in smooth muscle (17 , 20).
There were more severe pathological changes of the aorta in compound mutant mice than in those with mutant fibrillin-1 alone (Figure 6, A and B, and Supplemental Figure 13).