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 …

Participation of oleic acid in the formation of the aortic aneurysm in Marfan syndrome patients

ME Soto, AVI Hernández, VG Lans… - Prostaglandins & other …, 2016 - Elsevier
Marfan syndrome (MFS) is associated with progressive aortic dilatation and endothelial
dysfunction that lead to early acute dissection and rupture of the aorta and sudden death …

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 …

Selective loss of basal but not receptor-stimulated relaxation by endothelial nitric oxide synthase after isolation of the mouse aorta

J Van Langen, P Fransen, CE Van Hove… - European journal of …, 2012 - Elsevier
Bioavailability of nitric oxide (NO) is mostly studied in isolated blood vessels. We
investigated changes in basal and receptor-stimulated endothelial NO synthase (eNOS) …

An Imidazoline 2 Receptor Ligand Relaxes Mouse Aorta via Off-Target Mechanisms Resistant to Aging

F Jiménez-Altayó, A Cabrera, A Bagán… - Frontiers in …, 2022 - frontiersin.org
Imidazoline receptors (IR) are classified into three receptor subtypes (I1R, I2R, and I3R) and
previous studies showed that regulation of I2R signaling has neuroprotective potential. In …

Recent molecular biological progress in Marfan syndrome and Marfan-associated disorders

LG Gao, F Luo, RT Hui, XL Zhou - Ageing research reviews, 2010 - Elsevier
Marfan syndrome (MFS) is a connective tissue disorder with autosomal dominant
inheritance. Advances in medicine and surgery have increased the average lifespan of …

A phenotypic screen of Marfan syndrome iPSC-derived vascular smooth muscle cells uncovers GSK3β as a new target

H Davaapil, M McNamara, A Granata, RGC Macrae… - Stem Cell Reports, 2023 - cell.com
Marfan syndrome (MFS) is a rare connective tissue disorder caused by mutations in FBN1.
Patients with MFS notably suffer from aortic aneurysm and dissection. Despite considerable …

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 …

Comprehensive Characterization of Arterial and Cardiac Function in Marfan Syndrome—Can Biomarkers Help Improve Outcome?

CG Weismann, J Hlebowicz, A Åkesson… - Frontiers in …, 2022 - frontiersin.org
Background: Marfan Syndrome (MFS) has been associated with increased aortic stiffness
and left ventricular dysfunction. The latter may be due to the underlying genotype and/or …

Reversible cerebral vasoconstriction syndrome and posterior reversible encephalopathy syndrome in a boy with Loeys‐Dietz syndrome

Y Akazawa, Y Inaba, A Hachiya… - American Journal of …, 2015 - Wiley Online Library
Loeys‐Dietz syndrome (LDS) is an autosomal dominant connective tissue disorder, caused
by heterozygous mutations in TGFBR1 or TGFBR2 and characterized by vascular …