Bicuspid aortic valve aortopathy is characterized by embryonic epithelial to mesenchymal transition and endothelial instability

Figures

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  Fig. 1. Movat pentachrome staining of dilated ascending aortas (magnification × 20) from patients with BAV (n = 11) and TAV (n = 8), respectively. BAV, bicuspid aortic valve; TAV, tricuspid aortic valve

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  Fig. 2. ORIS migration assay using ascending aortic vascular smooth muscle cells isolated from patients with BAV and TAV, respectively, visualized by IncuCyte live cell imaging. a Area covered by cells at 0–24 h of growth and b fluorescent dye intensity at 0–10 h of growth. Red dotted lines represent the regression line for BAV (n = 5) and TAV cells (n = 3), respectively. c proliferation at 24 h, P = 0.036, Mann Whitney U-test. BAV, bicuspid aortic valve; TAV, tricuspid aortic valve

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  Fig. 3. a Network of BAV-specific (red), TAV-specific (green), and common (yellow) Hallmark EMT genes and their predicted miRNAs (identified using TargetScan). Gray squares and arrows mark predicted miRNAs and their targets. b Zoomed network of BAV-specific Hallmark EMT genes and their predicted miRNAs. Red arrows show negative correlation between gene and miRNA expression with corresponding P and correlation values presented in c. BAV, bicuspid aortic valve; TAV, tricuspid aortic valve

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  Fig. 4. a Laminin γ1 (red) expression (magnification × 10) and b quantification, non-dilated ascending aortas of TAV (n = 11) and BAV (n = 8) patients. c LAMC1/TAGLN intima-media protein expression (global protein expression measured previously using HiRIEF LC–MS/MS, described previously in Maleki et al. [13]) in relation ascending aortic diameter in patients with BAV (n = 11), P = 0.004, Pearson correlation = − 0.818. d CD31 (green) and vWF (red) expression (magnification × 10) and e quantification, non-dilated ascending aortas of BAV (n = 7) and TAV (n = 6) patients. BAV, bicuspid aortic valve; TAV, tricuspid aortic valve; D, dilated; ND, non-dilated

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  Fig. 5. a Endothelial and medial albumin expression in non-dilated and dilated ascending aortas of BAV (n = 9 non-dilated, n = 5 dilated) and TAV (n = 9 non-dilated, n = 7 dilated) patients (magnification × 10). b vWF expression, non-dilated and dilated ascending aortas, BAV (n = 9 non-dilated, n = 5 dilated), and TAV (n = 9 non-dilated, n = 7 dilated) patients (magnification × 10). c, d Quantification of albumin staining in intima and media layers, respectively. BAV, bicuspid aortic valve; TAV, tricuspid aortic valve; vWF, von Willebrand factor