Fibrillin-rich microfibrils are extracellular assemblies that impart structural properties to the
connective tissue. To elucidate the contribution of fibrillin-rich microfibrils to organogenesis,
we have examined the vascular phenotype of a newly created strain of mice that completely
lacks fibrillin-1 and the consequences of combined deficiency of fibrillins 1 and 2 on tissue
formation. The results demonstrated that fibrillins 1 and 2 perform partially overlapping
functions during aortic development. Fbn1-/-mice died soon after birth from ruptured aortic …