Fig 1: ITGAV, COL1A1, and MMP2 expression are increased in MFS iPSC SMCs. Western blot results for (A) COL1A1 and (B) MMP2 expression in iPSC SMCs. Expression is increased in MFS LM samples compared to control. (C) MMP2 gelatin zymography representative gel image and quantitation. (D) ITGAV western blot in iPSC SMC system shows pronounced ITGAV increase in MFS LM compared to control. (E) ITGAV western blot in human aortic tissue lysates from MFS patients (n = 3) and healthy control showing increased expression in the aneurysmal aortic root segment in MFS patients. (F) Aortic tissue protein lysate ELISA results comparing MFS patients (n = 6) to healthy controls (n = 4). Western blots and MMP assay from iPSC SMC lines represent at least three technical replicates from the indicated sample Groups tested by ELISA were compared by non-parametric analysis, Mann–Whitney rank sum test. Significance is assigned based on the P-value (P < 0.05, **P < 0.01, ***P < 0.001).