An iPSC-derived vascular model of Marfan syndrome identifies key mediators of smooth muscle cell death

A Granata, F Serrano, WG Bernard, M McNamara… - Nature …, 2017 - nature.com
Marfan syndrome (MFS) is a heritable connective tissue disorder caused by mutations in
FBN1, which encodes the extracellular matrix protein fibrillin-1. To investigate the …

Vascular smooth muscle cells in Marfan syndrome aneurysm: the broken bricks in the aortic wall

GL Perrucci, E Rurali, A Gowran, A Pini… - Cellular and Molecular …, 2017 - Springer
Marfan syndrome (MFS) is a connective tissue disorder with multiple organ manifestations.
The genetic cause of this syndrome is the mutation of the FBN1 gene, encoding the …

Characterization of the thoracodorsal artery: morphology and reactivity

M Billaud, AW Lohman, AC Straub, T Parpaite… - …, 2012 - Wiley Online Library
Please cite this paper as: Billaud M, Lohman AW, Straub AC, Parpaite T, Johnstone SR,
Isakson BE. Characterization of the thoracodorsal artery: morphology and reactivity …

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 …

Calcium promotes vascular smooth muscle cell phenotypic switching in Marfan syndrome

Y Yang, E Xie, Y Liu, Z Peng, C Yu, K Hua… - … and Biophysical Research …, 2023 - Elsevier
Abstract Fibrillin 1 (Fbn1) mutations cause Marfan syndrome (MFS), with aortic root
dilatation, dissection, and rupture. Few studies reported the blood calcium and lipid profile of …

Intrinsic Mechanics of Human Stem Cell Derived Aortic Smooth Muscle Cells Support a Developmental Basis for Aneurysm Localization in Marfan Syndrome

RJ Wiener, H Orins, KD Costa - bioRxiv, 2023 - biorxiv.org
Marfan Syndrome (MFS), a connective tissue disorder caused by a mutation in the fibrillin-1
gene, occurs in approximately 1 in 5,000 people worldwide. As an important constituent of …

[HTML][HTML] Structure-related blockage of calcium channels by vasodilator alkamides in mice mesenteric artery

DCG Garcia, AC Pereira, SJC Gutierrez… - Vascular …, 2016 - Elsevier
The development of new calcium channel blockers is still relevant for the understanding of
their physiological role and pharmacological and therapeutic purposes. For this task, natural …

[HTML][HTML] Activation of Cav1. 2 and BKCa is involved in the downregulation of caffeine-induced contraction in mice mesenteric arteries

DCG Garcia, MJ Lopes, UC Mbiakop, VS Lemos… - Life sciences, 2019 - Elsevier
Aims Caffeine is a methylxanthine with multiple actions in vascular smooth muscle cells
(VSMCs), including the increase in the intracellular Ca 2+(i Ca 2+) concentration by the …

Developing 3-D Human in vitro Models of Vascular Mechanobiology in Marfan Syndrome

NM Allen - 2019 - search.proquest.com
Marfan Syndrome (MFS) is a genetic disorder affecting connective tissue. Progression of
MFS leads to changes in aortic stiffness, contributing to aneurysm and dissection; major …

Articles in PresS. Am J Physiol Heart Circ Physiol (March 4, 2016). doi: 10.1152/ajpheart. 00770.2015

Y Onetti, T Meirelles, AP Dantas, K Schröder, E Vila… - 2016 - journals.physiology.org
Marfan syndrome (MFS) is a connective tissue disorder that is often associated with 25
fibrillin-1 (Fbn1) gene mutation and characterized by cardiovascular alterations, 26 …