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 …

[HTML][HTML] Inhibition of Marfan Syndrome aortic root dilation by losartan: Role of angiotensin II receptor type 1–independent activation of endothelial function

SL Sellers, N Milad, R Chan, M Mielnik… - The American journal of …, 2018 - Elsevier
Marfan syndrome (MFS) is a genetic disorder that frequently leads to aortic root dissection
and aneurysm. Despite promising preclinical and pilot clinical data, a recent large-scale …

Molecular mechanisms in genetic aortopathy–signaling pathways and potential interventions

CX Dong, C Malecki, E Robertson, B Hambly… - International Journal of …, 2023 - mdpi.com
Thoracic aortic disease affects people of all ages and the majority of those aged< 60 years
have an underlying genetic cause. There is presently no effective medical therapy for …

AKT2 confers protection against aortic aneurysms and dissections

YH Shen, L Zhang, P Ren, MT Nguyen, S Zou… - Circulation …, 2013 - Am Heart Assoc
Rationale: Aortic aneurysm and dissection (AAD) are major diseases of the adult aorta
caused by progressive medial degeneration of the aortic wall. Although the overproduction …

Mild aerobic exercise blocks elastin fiber fragmentation and aortic dilatation in a mouse model of Marfan syndrome associated aortic aneurysm

C Gibson, C Nielsen, R Alex… - Journal of Applied …, 2017 - journals.physiology.org
Regular low-impact physical activity is generally allowed in patients with Marfan syndrome,
a connective tissue disorder caused by heterozygous mutations in the fibrillin-1 gene …

Vasomotor dysfunction in the thoracic aorta of Marfan syndrome is associated with accumulation of oxidative stress

HHC Yang, C van Breemen, AWY Chung - Vascular Pharmacology, 2010 - Elsevier
We have described that the progression of thoracic aortic aneurysm in Marfan syndrome is
accompanied with aortic vascular dysfunction. In the present study, we hypothesized that the …

The NO signalling pathway in aortic aneurysm and dissection

M Toral, A de la Fuente‐Alonso… - British Journal of …, 2022 - Wiley Online Library
Recent studies have shown that NO is a central mediator in diseases associated with
thoracic aortic aneurysm, such as Marfan syndrome. The progressive dilation of the aorta in …

[HTML][HTML] Allopurinol blocks aortic aneurysm in a mouse model of Marfan syndrome via reducing aortic oxidative stress

I Rodríguez-Rovira, C Arce, K De Rycke… - Free Radical Biology …, 2022 - Elsevier
Background Increasing evidence indicates that redox stress participates in MFS aortopathy,
though its mechanistic contribution is little known. We reported elevated reactive oxygen …

Resveratrol Inhibits Aortic Root Dilatation in the Fbn1C1039G/+ Marfan Mouse Model

S Hibender, R Franken, C Van Roomen… - … , and vascular biology, 2016 - Am Heart Assoc
Objective—Marfan syndrome (MFS) is a connective tissue disorder caused by mutations in
the fibrillin-1 gene. Patients with MFS are at risk of aortic aneurysm formation and dissection …

[PDF][PDF] The emerging role of endothelial cells in the pathogenesis of thoracic aortic aneurysm and dissection

A Verstraeten, I Fedoryshchenko… - European Heart …, 2023 - academic.oup.com
The emerging role of endothelial cells in the pathogenesis of thoracic aortic aneurysm and
dissection Page 1 European Heart Journal (2023) 44, 1262–1264 https://doi.org/10.1093/eurheartj/ehac771 …