Figure 6. The expression of the KASH2 domain in smooth muscle cells ameliorates aortic disease in Lmna^G609G/G609G mice.

The Cre-conditional KASH2–EGFP transgene was bred into Lmna^G609G/G609G mice harboring the SMC-specific Sm22–Cre transgene. Three groups were examined: WT, Lmna^+/+KASH2–EGFP⁺Sm22-Cre⁺; MUT, Lmna^G609G/G609GKASH2–EGFP⁺Sm22-Cre^–; and MUT + KASH2, Lmna^G609G/G609GKASH2–EGFP⁺Sm22-Cre⁺. Adventitial fibrosis and SMC nuclei were quantified in the three groups. (a–d) Representative photographs of HNE-stained cross sections at the outer (a) and inner curvature (b) of the ascending aorta, and proximal descending aorta (c–d). Scale bars, 50 μm for panels a–c; 20 μm for panel d. (e) Adventitial area (as a percent of total area) and numbers of SMC nuclei (per media area) at the outer ascending thoracic aorta (mean ± SEM; n = 6/group). (f) Adventitial area and numbers of SMC nuclei at the inner ascending thoracic aorta (mean ± SEM; n = 6/group). (g) Adventitial area and numbers of SMC nuclei in the proximal descending thoracic aorta (mean ± SEM; n = 6/group). *p < 0.01; **p < 0.001; ns, nonsignificant defined as p > 0.40.