As our data point to markedly increased TGF-β2 levels in Fibulin-4 deficient SMCs, analyses on TGF-β receptors on these SMCs might further clarify the process of TGF-β regulation and determine its role in the pathogenesis of Fibulin-4 associated aortic aneurysms. Here, EFEMP2 is linked to aortic aneurysm.