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. 2020 Sep 10;11(1):237–249. doi: 10.1080/19491034.2020.1815410

Figure 7.

Figure 7.

Expression of KASH2 in smooth muscle cells (SMCs) ameliorates aortic disease in a mouse model of HGPS. The HGPS mice (LmnaG609G/G609G mice) were homozygous for the most common point mutation found in children with HGPS. KASH2 expression was from a Cre-activatable transgene (KASH2-EGFP); KASH2 expression was activated by an Sm22-Cre transgene. (a) Representative images of H&E-stained cross sections of the outer curvature of the ascending aorta in wild-type (WT) mice (Lmna+/+KASH2-EGFP+Sm22-Cre+), HGPS mice (LmnaG609G/G609GKASH2-EGFP+), and HGPS mice that expressed KASH2 in aortic SMCs (LmnaG609G/G609GKASH2-EGFP+Sm22-Cre+). Dotted white lines outline the adventitial layer of the aorta. Colored yellow arrow indicates the medial layer of the aorta (m). Scale bars, 50 μm. (b) Bar graphs depicting medial SMCs (nuclei per μm2) and adventitial area as a percentage of total area of the cross section. n = 6/group; **P < 0.001 as defined by a Student’s t test. Reproduced with permission from Kim et al. [47].