Marfan syndrome is a heritable disorder of the connective tissue with an estimated
prevalence of 1 in 5000 individuals and no predilection for either sex. The syndrome is …

Objective—Low-density lipoprotein receptor–related protein 1 (LRP1) is a large endocytic
and signaling receptor that is abundant in vascular smooth muscle cells. Mice in which the …

Background Development of thoracic aortic aneurysms is the most significant clinical
phenotype in patients with Marfan syndrome. An inflammatory response has been described …

Background—Arterial stiffness has been associated with an increased cardiovascular risk.
The aim of this study was to investigate the interaction between arterial stiffness and …

Fibrillins and latent transforming growth factor β binding proteins (LTBPs) are components of
the extracellular matrix of connective tissue. While fibrillins are integral to the 10nm …

Marfan syndrome (MFS) is a genetic disorder that affects multiple organs. Mortality imposed
by aortic aneurysm and dissections represent the most serious clinical manifestation of MFS …

Elastin, one of the longest-lived proteins, confers elasticity to tissues with high mechanical
constraints. During aging or pathophysiological conditions such as cancer progression, this …

Extracellular matrix (ECM) components confer biomechanical properties, maintain cell
phenotype and mediate tissue homeostasis. ECM remodeling is complex and plays a key …

Aim BicC family RNA-binding protein 1 (BICC1) codes an RNA-binding protein that
regulates gene expression and modulates cell proliferation and apoptosis. We aim at …

Elastin is a crucial extracellular matrix protein that provides structural integrity to tissues.
Crosslinked elastin and associated microfibrils, named elastic fiber, contribute to …