Fibulin-4 is an extracellular matrix protein that is essential for proper assembly of arterial
elastic fibers. Mutations in fibulin-4 cause cutis laxa with thoracic aortic aneurysms (TAAs) …

Rationale: Loss of fibulin-4 during embryogenesis results in perinatal lethality because of
aneurysm rupture, and defective elastic fiber assembly has been proposed as an underlying …

The Fibulins are a 6-member protein family hypothesized to function as intermolecular
bridges that stabilize the organization of extracellular matrix structures. Here, we show that …

Aims Destructive remodelling of extracellular matrix (ECM) and inflammation lead to dilation
and ultimately abdominal aortic aneurysm (AAA). Fibulin-5 (FBLN5) mediates cell–ECM …

Medial degeneration is a key feature of aneurysm disease and aortic dissection. In a murine
aneurysm model we investigated the structural and functional characteristics of aortic wall …

Background Homeostasis of the vessel wall is cooperatively maintained by endothelial cells
(ECs), smooth muscle cells, and adventitial fibroblasts. The genetic deletion of fibulin‐4 …

Fibulin-4 is an extracellular matrix (ECM) protein essential for elastogenesis and mutations
in this protein lead to aneurysm formation. In this study, we isolated vascular smooth muscle …

Homozygous or compound heterozygous mutations in fibulin-4 (FBLN4) lead to autosomal
recessive cutis laxa type 1B (ARCL1B), a multisystem disorder characterized by significant …

Aortic aneurysms are life-threatening and often associated with defects in connective tissues
and mutations in smooth muscle cell (SMC) contractile proteins. Despite recent advances in …

Mice with a smooth muscle cell (SMC)-specific deletion of Fibulin-4 (SMKO) show
decreased expression of SMC contractile genes, decreased circumferential compliance, and …