Piotr Alfred Wielopolski1, Gerben Koning1, Paula van Heijningen1, Eric Kaijzel2, Clemens Lowik2, Monique Bernsen1, Jeroen Essers1
1Erasmus MC, Rotterdam, Zuid-Holland, Netherlands; 2Leiden University Medical Center, Netherlands
Fibulins are a six-member protein family hypothesized to function as intermolecular bridges that stabilize the organization of extracellular matrix structures as elastic fibers and basement membranes. Previously, we generated a mouse model underexpressing Fibulin-4 and showed that reduced expression of Fibulin-4 leads to aneurism formation, dissection of the aortic wall and cardiac abnormalities. Mice homozygous for the Fibulin-4 reduced expression allele (Fibulin-4R/R) show dilatation of the ascending aorta and a tortuous and stiffened aorta, resulting from disorganized elastic fiber networks. The non-invasive and multi-contrast capabilities of magnetic resonance imaging (MRI) can aid tremendously in understanding this pathology and the effects of therapy.