leptin and Marfan-Syndrome

Background Marfan syndrome (MFS) is a genetically transmitted connective tissue disorder characterized by aortic root dilatation, dissection, and rupture. Molecularly, MFS pathological features have been shown to be driven by increased angiotensin II in the aortic wall. Using an angiotensin II-driven aneurysm mouse model, we have recently demonstrated that local inhibition of leptin activity restricts aneurysm formation in the ascending and abdominal aorta. As we observed de novo leptin synthesis in the ascending aortic aneurysm wall of patients with MFS, we hypothesized that local counteracting of leptin activity in MFS may also prevent aortic cardiovascular complications in this context. Methods and Results

Topics: Animals; Aorta; Aortic Aneurysm; Dilatation, Pathologic; Disease Models, Animal; Fibrillin-1; Hormone Antagonists; Leptin; Male; Marfan Syndrome; Mice, Mutant Strains; Signal Transduction; Systole; Vascular Remodeling; Ventricular Dysfunction, Left; Ventricular Function, Left