Whole-Body Imaging of Vascular Pathology in Fibulin-4 Mice Using Gd-Liposomes and Magnetic Resonance Angiography

Piotr Alfred Wielopolski¹, Gerben Koning¹, Paula van Heijningen¹, Eric Kaijzel², Clemens Lowik², Monique Bernsen¹, Jeroen Essers¹

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.