Imbalanced synthesis of cyclooxygenase‐derived thromboxane A2 and prostacyclin compromises vasomotor function of the thoracic aorta in Marfan syndrome
AWY Chung, HHC Yang… - British journal of …, 2007 - Wiley Online Library
Background and purpose: Thoracic aortic dissection is a life‐threatening complication of
Marfan syndrome, a connective tissue disorder caused by mutations in the gene encoding …
Marfan syndrome, a connective tissue disorder caused by mutations in the gene encoding …
Relationship between fibrillin-1 genotype and severity of cardiovascular involvement in Marfan syndrome
R Franken, G Teixido-Tura, M Brion, A Forteza… - Heart, 2017 - heart.bmj.com
Background The effect of FBN1 mutation type on the severity of cardiovascular
manifestations in patients with Marfan syndrome (MFS) has been reported with disparity …
manifestations in patients with Marfan syndrome (MFS) has been reported with disparity …
[HTML][HTML] The importance of genotype-phenotype correlation in the clinical management of Marfan syndrome
VM Becerra-Muñoz, JJ Gómez-Doblas… - Orphanet journal of rare …, 2018 - Springer
Background Marfan syndrome (MFS) is a disorder of autosomal dominant inheritance, in
which aortic root dilation is the main cause of morbidity and mortality. Fibrillin-1 (FBN-1) …
which aortic root dilation is the main cause of morbidity and mortality. Fibrillin-1 (FBN-1) …
Pharmacotherapeutics for prevention of aortic root enlargement in Marfan Syndrome–a network meta-analysis of randomized controlled trials
AH Malik, S Yandrapalli, G Pemmasani… - European Journal of …, 2020 - academic.oup.com
Marfan syndrome (MFS) is a life-threatening autosomal dominant condition with a
prevalence ranging from one every 3000 to one every 5000 persons. 1 Aortic root dissection …
prevalence ranging from one every 3000 to one every 5000 persons. 1 Aortic root dissection …
Genotype FBN1/phenotype relationship in a cohort of patients with Marfan syndrome
A Hernándiz, A Zúñiga, F Valera, D Domingo… - Clinical …, 2021 - Wiley Online Library
Marfan syndrome (MFS) is a systemic connective tissue disorder caused by mutations in the
fibrillin‐1 (FBN1) gene, and cardiovascular involvement is the leading cause of mortality. We …
fibrillin‐1 (FBN1) gene, and cardiovascular involvement is the leading cause of mortality. We …
Impact of Notch3 activation on aortic aneurysm development in marfan syndrome
K Jespersen, C Li, R Batra… - Journal of …, 2022 - Wiley Online Library
Background. The leading cause of mortality in patients with Marfan syndrome (MFS) is
thoracic aortic aneurysm and dissection. Notch signaling is essential for vessel …
thoracic aortic aneurysm and dissection. Notch signaling is essential for vessel …
Novel FBN1 mutations are responsible for cardiovascular manifestations of Marfan syndrome
J Wang, Y Yan, J Chen, L Gong, YU Zhang… - Molecular biology …, 2016 - Springer
Abstract The fibrillin-1 (FBN1) gene mutations result in Marfan syndrome (MFS) and have a
variety of phenotypic variations. This disease is involved in the skeletal, ocular and …
variety of phenotypic variations. This disease is involved in the skeletal, ocular and …
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 …
expression. The main cardiovascular sequelae of MFS include aortic aneurysm/dissection …
Marfan and Sartans: time to wake up!
J De Backer - European heart journal, 2015 - academic.oup.com
Marfan syndrome (MFS) is a pleiotropic inheritable connective tissue disorder with its main
manifestations in the ocular, skeletal, and cardiovascular organ systems. 1 Life expectancy …
manifestations in the ocular, skeletal, and cardiovascular organ systems. 1 Life expectancy …
MMP-2 regulates Erk1/2 phosphorylation and aortic dilatation in Marfan syndrome
W Xiong, T Meisinger, R Knispel, JM Worth… - Circulation …, 2012 - Am Heart Assoc
Rationale: Aneurysm and dissection of the ascending thoracic aorta are the main
cardiovascular complications of Marfan syndrome (MFS) resulting in premature death …
cardiovascular complications of Marfan syndrome (MFS) resulting in premature death …