Fig. 2

Remodelin ameliorates cardiac and other pathologies of HGPS mice. Pathological staining in panels a–d was carried out on the materials from end-point mice (presented in Fig.  1f) of indicated genotypes (n = 6 per genotype) treated with vehicle or Remodelin 100 mg per kg per day (for detailed ages of the mice, see Supplementary Table 4). All images are representative (scale bar 50 µm) and the correspondent bar graph quantifications are presented (mean ± s.e.m.; individual data points represented; n.s. not significant; *p < 0.05, **p < 0.01, ***p < 0.001, ****p < 0.0001 two-tailed Student’s t-test). In WT mice, Remodelin treatment has no significant effect, as compared to vehicle treatment; and for simplicity these animals have been pooled in one group; the individual comparison is presented in Supplementary Fig. 2b. a Hematoxilin and eosin (H&E) staining of skin, indicating fat layer thickness (vertical bars indicate the fat layer) and showing amelioration of the fat layer thickness upon Remodelin treatment in HGPS mice. b H&E staining of heart aorta, indicating increased adventitial width in the HGPS mice, as compared to WT controls, which is rescued by Remodelin treatment (arrowheads demarcate the aorta and vertical bars the adventitia). c DAPI staining of heart aorta, showing a decreased number of nuclei in the HGPS mice, rescued by Remodelin treatment (dotted white lines delineate the aorta edges). d Smooth muscle actin (SMA) staining (green) of heart aorta sections, showing loss of integrity of the artery wall in HGPS mice, improved by Remodelin treatment (dotted white lines delineate the aorta). e, f Representative western blotting analysis of the representative heart (e) and lung (f) tissues from end-point mice, showing that Remodelin decreased γH2AX levels in Lmna^G609G/G609G tissues (see quantification below each Western blot, relative to total H2AX levels). Western blots were performed more than once on n ≥ 4/group