In this setting, animals exhibited a baseline ascending aortic expansion that mimics the TAA observed in patients with MFS, which as alluded to previously, is commensurate with the phenotypic similarities between NPR-C−/− mice and MFS.33 In addition, NPR-C−/− mice also exhibited a worsened phenotype, with greater severity in AAA characterized by immune cell infiltrate, elastin strand breaks, fibrosis, calcification, and myofibroblast differentiation. This evidence concerns the gene ELN and triple-A syndrome.