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 …

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 …

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 …

Fibulins are extracellular matrix proteins associated with elastic fibres. Homozygous Fibulin-
4 mutations lead to life-threatening abnormalities such as aortic aneurysms. Aortic …

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 …

About 20% of individuals afflicted with thoracic aortic disease have single-gene mutations
that predispose the vessel to aneurysm formation and/or acute aortic dissection often without …

Background: Marfan syndrome, caused by mutations in the gene for fibrillin-1, leads to
thoracic aortic aneurysms (TAAs). Phenotypic modulation of vascular smooth muscle cells …

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) …

Highlights•Study of Marfan mouse models revealed a key role for TGFβ signaling in the
pathogenesis of aortic aneurysm.•Canonical and noncanonical TGFβ signaling play parts in …

Aims: Increasing evidence supports a role for the angiotensin II-AT 1-receptor axis in
aneurysm development. Here, we studied whether counteracting this axis via stimulation of …