Fibrillin‐1 genetic deficiency leads to pathological ageing of arteries in mice
B Mariko, M Pezet, B Escoubet, S Bouillot… - The Journal of …, 2011 - Wiley Online Library
Fibrillin‐1, the major component of extracellular microfibrils that associate with insoluble
elastin in elastic fibres, is mainly synthesized during development and postnatal growth and …
elastin in elastic fibres, is mainly synthesized during development and postnatal growth and …
Aortic regurgitation is associated with ascending aortic remodeling in the nondilated aorta
B Balint, JM Federspiel, T Schwab… - … , and vascular biology, 2021 - Am Heart Assoc
Objective: The probability of aortic complications in patients with bicuspid aortic valve is
higher in association with aortic regurgitation (AR) compared with aortic stenosis (AS) or …
higher in association with aortic regurgitation (AR) compared with aortic stenosis (AS) or …
[HTML][HTML] Intrinsic cardiomyopathy in Marfan syndrome: results from in-vivo and ex-vivo studies of the Fbn1C1039G/+ model and longitudinal findings in humans
Background: Mild intrinsic cardiomyopathy in patients with Marfan syndrome (MFS) has
consistently been evidenced by independent research groups. So far, little is known about …
consistently been evidenced by independent research groups. So far, little is known about …
[HTML][HTML] Differences in the thoracic aorta by region and sex in a murine model of Marfan syndrome
F Jiménez-Altayó, AM Siegert, F Bonorino… - Frontiers in …, 2017 - frontiersin.org
Marfan syndrome (MFS) is a hereditary disorder of the connective tissue that causes life-
threatening aortic aneurysm, which initiates at the aortic root and can progress into the …
threatening aortic aneurysm, which initiates at the aortic root and can progress into the …
[HTML][HTML] The potential beneficial effects of resveratrol on cardiovascular complications in Marfan syndrome patients–insights from rodent-based animal studies
MM van Andel, M Groenink, AH Zwinderman… - International journal of …, 2019 - mdpi.com
Marfan syndrome (MFS) patients are at risk for cardiovascular disease. In particular, for
aortic aneurysm formation, which ultimately can result in a life-threatening aortic dissection …
aortic aneurysm formation, which ultimately can result in a life-threatening aortic dissection …
Losartan attenuates degradation of aorta and lung tissue micromechanics in a mouse model of severe Marfan syndrome
JJ Lee, J Galatioto, S Rao, F Ramirez… - Annals of biomedical …, 2016 - Springer
Marfan syndrome (MFS) is an autosomal dominant disease of the connective tissue due to
mutations in the fibrillin-1 gene (FBN1). This study aimed at characterizing microelastic …
mutations in the fibrillin-1 gene (FBN1). This study aimed at characterizing microelastic …
[PDF][PDF] 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 …
Patients with MFS notably suffer from aortic aneurysm and dissection. Despite considerable …
Proteomics reveals Rictor as a noncanonical TGF-β signaling target during aneurysm progression in Marfan mice
The objective of the present study was to 1) analyze the ascending aortic proteome within a
mouse model of Marfan syndrome (MFS; Fbn1 C1041G/+) at early and late stages of …
mouse model of Marfan syndrome (MFS; Fbn1 C1041G/+) at early and late stages of …
[HTML][HTML] Leukotriene-C4 synthase, a critical enzyme in the activation of store-independent Orai1/Orai3 channels, is required for neointimal hyperplasia
Leukotriene-C 4 synthase (LTC 4 S) generates LTC 4 from arachidonic acid metabolism.
LTC 4 is a proinflammatory factor that acts on plasma membrane cysteinyl leukotriene …
LTC 4 is a proinflammatory factor that acts on plasma membrane cysteinyl leukotriene …
Long‐term effects of losartan on structure and function of the thoracic aorta in a mouse model of Marfan syndrome
HHC Yang, JM Kim, E Chum… - British journal of …, 2009 - Wiley Online Library
Background and purpose: During development of thoracic aortic aneurysms in a mouse
model of Marfan syndrome, upregulation of matrix metalloproteinase (MMP)‐2 and‐9 was …
model of Marfan syndrome, upregulation of matrix metalloproteinase (MMP)‐2 and‐9 was …