Induction of macrophage chemotaxis by aortic extracts from patients with Marfan syndrome is related to elastin binding protein
Marfan syndrome is an autosomal dominantly inherited disorder of connective tissue with
prominent skeletal, ocular, and cardiovascular manifestations. Aortic aneurysm and …
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 …
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 …
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 …
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 …
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 …
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 …
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 …
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 …
(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
Background: Marfan syndrome (MFS), an inherited disorder of connective tissue
characterized by abnormalities in the skeletal, ocular, and cardiovascular systems, is caused …
characterized by abnormalities in the skeletal, ocular, and cardiovascular systems, is caused …