Fig. 1.

Treatment groups, aortic enlargement, and gene expression analysis. a Treatment groups. Group 1 was comprised of five adult IDUA^−/− mice and four age-matched WT mice of 7 and 8 months of age that were analyzed by echocardiography immediately prior to elective sacrifice. Group 2 was n = 3 each male/female WT and 4 each IDUA^−/− (age 6–8 months) whose aortic root/arch tissue was harvested for gene quantitative expression analysis. Group 3 was n = 4 each IDUA^−/− male animals that were left untreated or were treated with losartan. Group 4 were juvenile IDUA^−/− or WT male mice whose numbers were split into six each in the no treatment or ARB group, respectively. X = untreated group, q weekly refers to the weekly replacement of losartan in the animals water. Ascending aorta echo analysis. Seven-month-old male mice were analyzed by high-resolution echo and the diameter of their aorta measured. A representative image is shown for b WT and c IDUA^−/− mice and mean measurements (determined at orange line) were: WT = 1.38 mm ± 0.87 mm and IDUA^−/− = 2.87 mm ± 0.88 mm and, p = 0.012). d Targeted gene expression analysis in MPS male and female mice. Aortic root/arch tissue was analyzed by qRT-PCR and was internally normalized to the GAPDH housekeeping gene with further standardization to reference sex and age matched C57/Bl6 mice. Relative quantitation using the 2-ddCT methodology is shown with standard deviation. * = p < 0.01 by Student’s t-test. ACE = angiotensin converting enzyme or ACE2 variant. AT = angiotensin receptor 1 or 2, CTS = cathepsin D or S, MMP = matrix metalloproteinase, TGF = transforming growth factor, OPN = osteopontin, ELN = elastin. WT mice (n = 3), IDUA^−/− each for age matched male or female mice (n = 4)