A similar mechanism described for fibulin-4 results from mutations in COL3A1, the causative gene of vEDS, with a main function to protect against the catastrophic disruption of the aortic wall that may result from an impaired deposition and maturation of collagen [51], mostly in syndromic forms of TAA [20]. This evidence concerns the gene COL3A1 and Ehlers-Danlos syndrome, vascular type.