Fibulin-4 Deficiency Results in Ascending Aortic Aneurysms

Loss of fibulin-4 during embryogenesis results in perinatal lethality because of aneurysm rupture, and defective elastic fiber assembly has been proposed as an underlying cause for the aneurysm phenotype. However, aneurysms are never seen in mice deficient for elastin, or for fibulin-5, which absence also leads to compromised elastic fibers.We sought to determine the mechanism of aneurysm development in the absence of fibulin-4 and establish the...