Fig. 2 |. Effect of SRP-2001 on cellular proliferation and B-type lamin protein expression in the fibroblasts of a patient with HGPS.

a, Treatment of three independent patients with classic HGPS and sex-matched parental control fibroblast cell lines with SRP-2001 improves proliferative capacity in culture. Cells received either no treatment, saline (vehicle) or 6 μM of SRP-2001, performed in triplicate. *P < 0.05, **P < 0.01, ***P < 0.001 versus vehicle control. b, Western blot analysis of A- and B-type lamins showing partial rescue of B-type lamins, particularly LMNB2, in HGPS fibroblasts treated with SRP-2001. LMNB1 and LMNB2 levels increased 80 (P = 0.11) and 100% (P < 0.05), respectively in HGPS fibroblasts at 12 μM of SRP-2001 versus cells receiving no treatment. Values and error bars represent the mean ± s.d. of triplicate cultures.