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 …

Redox stress in Marfan syndrome: Dissecting the role of the NADPH oxidase NOX4 in aortic aneurysm

F Jimenez-Altayo, T Meirelles, E Crosas-Molist… - Free Radical Biology …, 2018 - Elsevier
Marfan syndrome (MFS) is characterized by the formation of ascending aortic aneurysms
resulting from altered assembly of extracellular matrix fibrillin-containing microfibrils and …

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 …

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 …

[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 …

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 …

Anatomically specific reactive oxygen species production participates in Marfan syndrome aneurysm formation

F Emrich, K Penov, M Arakawa… - Journal of cellular …, 2019 - Wiley Online Library
Marfan syndrome (MFS) is a connective tissue disorder that results in aortic root aneurysm
formation. Reactive oxygen species (ROS) seem to play a role in aortic wall remodelling in …

[HTML][HTML] In vivo phenotypic vascular dysfunction extends beyond the aorta in a mouse model for fibrillin-1 (Fbn1) mutation

T Curry, ME Barrameda, TC Thomas, M Esfandiarei - Scientific Reports, 2024 - nature.com
Abstract In individuals with Marfan Syndrome (MFS), fibrillin-1 gene (FBN1) mutations can
lead to vascular wall weakening and dysfunction. The experimental mouse model of MFS …

[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 …