Fig. 6 |. Partial rescue of vascular tissue degradation in mice treated with SRP-2001.

End point analysis of vascular tissues of LMNA^G/G mice treated twice weekly until the end point at an average of 30.5 weeks with saline (vehicle) compared to the end point at an average of 49.5 weeks with 60 mg kg⁻¹ of SRP-2001. a, Representative images from Movat’s pentachrome-stained ascending aorta sections illustrate VSMC loss, elastin degradation, thickened medial layer and proteoglycan accumulation observed in LMNA^G/G mice receiving saline only. The aortas of mice treated with SRP-2001 contained less proteoglycan (blue) and retained VSMCs (red). H&E stain of the ascending aorta showing VSMC nuclei (blue) presence in SRP-2001-treated mice nearer the lumen of the tunica media. For each sample and stain, a total of four sections were imaged for analysis. b, Left: end point VSMC density within the tunica media of the ascending aortas from mice receiving PPMO or vehicle (saline) only. Right: adventitial area of ascending aortas at the end point. c, SRP-2001 reduces expression of progerin in vivo as determined by qPCR analysis of LMNA transgene expression in vascular tissue from mice receiving saline (vehicle) or SRP-2001. Values and error bars represent the mean ± s.d. for n = 12 mice per treatment group (6 males, 6 females); *P < 0.05, **P < 0.01.