Nitric oxide in the Marfan vasculature: Friend or foe?

AY Tehrani, MA Ciufolini, P Bernatchez - Nitric Oxide, 2021 - Elsevier
Marfan syndrome (MFS) is a connective tissue disorder caused by mutations in the FBN1
gene, which encodes fibrillin-1, a protein essential for the formation and stabilization of …

[HTML][HTML] Sildenafil prevents Marfan-associated emphysema and early pulmonary artery dilation in mice

Z White, N Milad, AY Tehrani, J Lamothe… - The American journal of …, 2019 - Elsevier
Marfan syndrome (MFS) is a connective tissue disorder caused by mutations in fibrillin-1
(Fbn1). Although aortic rupture is the major cause of mortality in MFS, patients also …

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 …

Endothelial dysfunction and compromised eNOS/Akt signaling in the thoracic aorta during the progression of Marfan syndrome

AWY Chung, K Au Yeung, SF Cortes… - British journal of …, 2007 - Wiley Online Library
Background and purpose: Aortic complications account for the major mortality in Marfan
syndrome (MFS), a connective tissue disorder caused by mutations in FBN1 encoding …

Molecular pathogenesis of Marfan syndrome

CJA Ramachandra, A Mehta, KWQ Guo, P Wong… - International journal of …, 2015 - Elsevier
Marfan syndrome (MFS) is a genetic disorder that affects multiple organs. Mortality imposed
by aortic aneurysm and dissections represent the most serious clinical manifestation of MFS …

Rationale and design of a trial evaluating the effects of losartan vs. nebivolol vs. the association of both on the progression of aortic root dilation in Marfan syndrome …

FI Gambarin, V Favalli, A Serio, M Regazzi… - Journal of …, 2009 - journals.lww.com
Background The major clinical problem of Marfan syndrome (MFS) is the aortic root
aneurysm, with risk of dissection when the root diameter approximates 5 cm. In MFS, a key …

[HTML][HTML] The evaluation of flow-mediated vasodilation in the brachial artery correlates with endothelial dysfunction evaluated by nitric oxide synthase metabolites in …

O Lomelí, I Pérez-Torres, R Márquez, S Críales… - Frontiers in …, 2018 - frontiersin.org
Marfan syndrome (MS) is of the most common connective tissue disorders. Although most
patients have mutations in the fibrillin-1 gene (FBN1) and more than 1,700 mutations have …

NADPH oxidase 4 attenuates cerebral artery changes during the progression of Marfan syndrome

Y Onetti, T Meirelles, AP Dantas… - American Journal …, 2016 - journals.physiology.org
Marfan syndrome (MFS) is a connective tissue disorder that is often associated with the
fibrillin-1 (Fbn1) gene mutation and characterized by cardiovascular alterations …

[HTML][HTML] Novel pharmacological strategies to prevent aortic complications in Marfan syndrome

P Matt, F Eckstein - Journal of geriatric cardiology: JGC, 2011 - ncbi.nlm.nih.gov
The Marfan syndrome (MFS) is a systemic connective tissue disorder caused by mutations in
the FBN1 gene. Recent molecular studies, most performed in mouse models, revealed that …

Aortopathy in Marfan syndrome: an update

F Romaniello, D Mazzaglia, A Pellegrino… - Cardiovascular …, 2014 - Elsevier
Marfan syndrome (MFS) is an inherited autosomal dominant multisystem disease caused by
mutations in the FBN1 gene encoding fibrillin-1, an extracellular matrix glycoprotein widely …