Induction of macrophage chemotaxis by aortic extracts from patients with Marfan syndrome is related to elastin binding protein

G Guo, P Gehle, S Doelken, JL Martin-Ventura… - PloS one, 2011 - journals.plos.org
Marfan syndrome is an autosomal dominantly inherited disorder of connective tissue with
prominent skeletal, ocular, and cardiovascular manifestations. Aortic aneurysm and …

Induction of macrophage chemotaxis by aortic extracts of the mgR Marfan mouse model and a GxxPG-containing fibrillin-1 fragment

G Guo, P Booms, M Halushka, HC Dietz, A Ney… - Circulation, 2006 - Am Heart Assoc
Background—The primary cause of early death in untreated Marfan syndrome (MFS)
patients is aortic dilatation and dissection. Methods and Results—We investigated whether …

Loss of endothelial barrier in Marfan mice (mgR/mgR) results in severe inflammation after adenoviral gene therapy

PC Seppelt, S Schwill, A Weymann, R Arif, A Weber… - PLoS …, 2016 - journals.plos.org
Objectives Marfan syndrome is an autosomal dominant inherited disorder of connective
tissue. The vascular complications of Marfan syndrome have the biggest impact on life …

Mechanical and pharmacological approaches to investigate the pathogenesis of Marfan syndrome in the abdominal aorta

AWY Chung, HH Yang, K Au Yeung… - Journal of vascular …, 2008 - karger.com
Background: Occurrence of disease complications in the abdominal aorta in Marfan
syndrome, a connective tissue disorder caused by mutations in the gene encoding fibrillin-1 …

Phenotypic alteration of vascular smooth muscle cells precedes elastolysis in a mouse model of Marfan syndrome

TE Bunton, NJ Biery, L Myers, B Gayraud… - Circulation …, 2001 - Am Heart Assoc
Marfan syndrome is associated with early death due to aortic aneurysm. The condition is
caused by mutations in the gene (FBN1) encoding fibrillin-1, a major constituent of …

Pathophysiology and therapeutics of thoracic aortic aneurysm in Marfan syndrome

K Asano, A Cantalupo, L Sedes, F Ramirez - Biomolecules, 2022 - mdpi.com
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 …

Vascular smooth muscle cell phenotypic changes in patients with Marfan syndrome

E Crosas-Molist, T Meirelles… - … , and vascular biology, 2015 - Am Heart Assoc
Objective—Marfan's syndrome is characterized by the formation of ascending aortic
aneurysms resulting from altered assembly of extracellular matrix microfibrils and chronic …

IL‐6 Regulates Extracellular Matrix Remodeling Associated With Aortic Dilation in a Fibrillin‐1 Hypomorphic mgR/mgR Mouse Model of Severe M arfan Syndrome

X Ju, T Ijaz, H Sun, W LeJeune, G Vargas… - Journal of the …, 2014 - Am Heart Assoc
Background Development of thoracic aortic aneurysms is the most significant clinical
phenotype in patients with Marfan syndrome. An inflammatory response has been described …

Loss of elastic fiber integrity and reduction of vascular smooth muscle contraction resulting from the upregulated activities of matrix metalloproteinase-2 and-9 in the …

AWY Chung, K Au Yeung, GGS Sandor… - Circulation …, 2007 - Am Heart Assoc
Thoracic aortic aneurysm (TAA) is the life-threatening complication of Marfan syndrome
(MFS), a connective tissue disorder caused by mutations in the fibrillin-1 gene. TAA is …

[HTML][HTML] Indomethacin prevents the progression of thoracic aortic aneurysm in Marfan syndrome mice

G Guo, CE Ott, J Grünhagen, B Muñoz-García… - Aorta, 2013 - thieme-connect.com
Background: Marfan syndrome (MFS), an inherited disorder of connective tissue
characterized by abnormalities in the skeletal, ocular, and cardiovascular systems, is caused …