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 …

A novel murine model of Marfan syndrome accelerates aortopathy and cardiomyopathy

NB Cavanaugh, L Qian, NM Westergaard… - The Annals of thoracic …, 2017 - Elsevier
Background Marfan syndrome (MFS) represents a genetic disorder with variable phenotypic
expression. The main cardiovascular sequelae of MFS include aortic aneurysm/dissection …

Therapy of Marfan syndrome

DP Judge, HC Dietz - Annu. Rev. Med., 2008 - annualreviews.org
Marfan syndrome is a common inherited disorder of connective tissue caused by deficiency
of the matrix protein fibrillin-1. Effective surgical therapy for the most life-threatening …

[HTML][HTML] Precise therapy for thoracic aortic aneurysm in marfan syndrome: a puzzle nearing its solution

E Rurali, GL Perrucci, CA Pilato, A Pini… - Progress in …, 2018 - Elsevier
Marfan Syndrome (MFS) is a rare connective tissue disorder, resulting from mutations in the
fibrillin-1 gene, characterized by pathologic phenotypes in multiple organs, the most …

Therapy insight: aortic aneurysm and dissection in Marfan's syndrome

F Ramirez, HC Dietz - Nature Clinical Practice Cardiovascular Medicine, 2004 - nature.com
Aortic dissection and aneurysm are common clinical problems with life-threatening
consequences; they are also the hallmark of several genetic diseases, including Marfan's …

Nitro-oleic acid reduces thoracic aortic aneurysm progression in a mouse model of Marfan syndrome

FS Nettersheim, J Lemties, S Braumann… - Cardiovascular …, 2022 - academic.oup.com
Aims Marfan syndrome (MFS) is a connective tissue disorder caused by mutations in the
Fibrillin-1 gene. It is associated with formation of thoracic aortic aneurysms that can …

[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 …

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 …

Altered tissue behavior of a non-aneurysmal descending thoracic aorta in the mouse model of Marfan syndrome

D Haskett, JJ Doyle, C Gard, H Chen, C Ball… - Cell and tissue …, 2012 - Springer
Aortic aneurysm is predominantly found in the ascending aorta in patients with Marfan
syndrome (MFS). However, descending aortic disease has emerged as a problem since …

An overview of investigational and experimental drug treatment strategies for Marfan syndrome

V Deleeuw, A De Clercq, J De Backer… - Journal of experimental …, 2021 - Taylor & Francis
Marfan syndrome (MFS) is a heritable connective tissue disorder caused by pathogenic
variants in the gene coding for the extracellular matrix protein fibrillin-1. While the disease …