Besides inflammatory cell infiltration and depletion of medial smooth-muscle cells, the ongoing remodeling of extracellular matrix (ECM) in the arterial wall is a prominent feature of the pathomechanism, and it is believed that the enzymolysis of medial and adventitial ECM proteins (mostly elastin and collagen) associated with their impaired remodeling plays a critical role in the pathogenesis of AAA [1,2,3,4,5,6,7,8,9]. This evidence concerns the gene ELN and triple-A syndrome.