[HTML][HTML] Aortic and cardiac structure and function using high-resolution echocardiography and optical coherence tomography in a mouse model of Marfan syndrome

L Lee, JZ Cui, M Cua, M Esfandiarei, X Sheng… - PloS one, 2016 - journals.plos.org
Marfan syndrome (MFS) is an autosomal-dominant disorder of connective tissue caused by
mutations in the fibrillin-1 (FBN1) gene. Mortality is often due to aortic dissection and rupture …

[HTML][HTML] Oxidative stress-related susceptibility to aneurysm in Marfan's syndrome

J Rysz, A Gluba-Brzózka, R Rokicki, B Franczyk - Biomedicines, 2021 - mdpi.com
The involvement of highly reactive oxygen-derived free radicals (ROS) in the genesis and
progression of various cardiovascular diseases, including arrhythmias, aortic dilatation …

[HTML][HTML] Aortic pathology from protein kinase G activation is prevented by an antioxidant vitamin B12 analog

GK Schwaerzer, H Kalyanaraman, DE Casteel… - Nature …, 2019 - nature.com
People heterozygous for an activating mutation in protein kinase G1 (PRKG1, p. Arg177Gln)
develop thoracic aortic aneurysms and dissections (TAAD) as young adults. Here we report …

A review of integrin-mediated endothelial cell phenotype in the design of cardiovascular devices

A Post, E Wang, E Cosgriff-Hernandez - Annals of biomedical engineering, 2019 - Springer
Sustained biomaterial thromboresistance has long been a goal and challenge in blood-
contacting device design. Endothelialization is one of the most successful strategies to …

Quantification of aortic and cutaneous elastin and collagen morphology in Marfan syndrome by multiphoton microscopy

JZ Cui, AY Tehrani, KA Jett, P Bernatchez… - Journal of structural …, 2014 - Elsevier
In a mouse model of Marfan syndrome, conventional Verhoeff-Van Gieson staining displays
severe fragmentation, disorganization and loss of the aortic elastic fiber integrity. However …

[HTML][HTML] Effectiveness of combination of losartan potassium and doxycycline versus single-drug treatments in the secondary prevention of thoracic aortic aneurysm in …

HHC Yang, JM Kim, E Chum, C van Breemen… - The Journal of thoracic …, 2010 - Elsevier
OBJECTIVE: Losartan potassium (INN losartan), an antihypertensive drug, has been shown
to prevent thoracic aortic aneurysm in Marfan syndrome through the inhibition of …

NOing the heart: role of nitric oxide synthase-3 in heart development

Y Liu, Q Feng - Differentiation, 2012 - Elsevier
Congenital heart disease is the most common birth defect in humans. Identifying factors that
are critical to embryonic heart development could further our understanding of the disease …

[HTML][HTML] Influence of resveratrol on the cardiovascular health effects of chronic kidney disease

JY Song, TC Shen, YC Hou, JF Chang, CL Lu… - International journal of …, 2020 - mdpi.com
Cardiovascular disease (CVD) is closely related to chronic kidney disease (CKD), and
patients with CKD have a high risk of CVD-related mortality. Traditional CVD risk factors …

[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] The multiple functions of fibrillin-1 microfibrils in organismal physiology

K Asano, A Cantalupo, L Sedes, F Ramirez - International Journal of …, 2022 - mdpi.com
Fibrillin-1 is the major structural component of the 10 nm-diameter microfibrils that confer key
physical and mechanical properties to virtually every tissue, alone and together with elastin …