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 …
dilatation, dissection, and rupture. Few studies reported the blood calcium and lipid profile of …
Fibrillin-1 mutation contributes to Marfan syndrome by inhibiting Cav1. 2-mediated cell proliferation in vascular smooth muscle cells
W Lin, J Xiong, Y Jiang, H Liu, J Bian, J Wang, Y Shao… - Channels, 2023 - Taylor & Francis
Marfan syndrome (MFS) is an autosomal dominant connective tissue disorder caused by
mutation in fibrillin-1 (FBN1). However, the molecular mechanism underlying MFS remains …
mutation in fibrillin-1 (FBN1). However, the molecular mechanism underlying MFS remains …
Characterizing the cardiovascular phenotype of a new zebrafish model of Marfan syndrome
PY Sips, R Le Roux, L Caboor… - European Heart …, 2021 - academic.oup.com
Background Marfan syndrome (MFS) is a rare disease caused by a defect in the fibrillin-1
gene (FBN1), with potentially severe cardiovascular manifestations. MFS patients are …
gene (FBN1), with potentially severe cardiovascular manifestations. MFS patients are …
[HTML][HTML] Renal cystic disease in the Fbn1C1039G/+ Marfan mouse is associated with enhanced aortic aneurysm formation
S Hibender, S Wanga, I van der Made, M Vos… - Cardiovascular …, 2019 - Elsevier
Marfan syndrome (MFS) is a connective tissue disorder caused by mutations in the fibrillin-1
gene (FBN1), resulting in aortic aneurysm formation and dissections. Interestingly, variable …
gene (FBN1), resulting in aortic aneurysm formation and dissections. Interestingly, variable …
[HTML][HTML] Analysis of disease progression-associated gene expression profile in fibrillin-1 mutant mice: new insight into molecular pathogenesis of Marfan syndrome
KL Kim, C Choi, W Suh - Biomolecules & Therapeutics, 2014 - ncbi.nlm.nih.gov
Marfan syndrome (MFS) is a dominantly inherited connective tissue disorder caused by
mutations in the gene encoding fibrillin-1 (FBN1) and is characterized by aortic dilatation …
mutations in the gene encoding fibrillin-1 (FBN1) and is characterized by aortic dilatation …
[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 …
lead to vascular wall weakening and dysfunction. The experimental mouse model of MFS …
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 …
The genetic cause of this syndrome is the mutation of the FBN1 gene, encoding the …
Fibroblast-secreted phosphoprotein 1 mediates extracellular matrix deposition and inhibits smooth muscle cell contractility in Marfan syndrome aortic aneurysm
M Zhou, Y Zhu, Z Zhou, F Qi, S Zheng, S Gao… - Journal of …, 2022 - Springer
Abstract Fibrillin 1 (Fbn1) mutation causes Marfan syndrome (MFS) with thoracic aortic
aneurysm (TAA) as the main complication. The mechanisms for extracellular matrix (ECM) …
aneurysm (TAA) as the main complication. The mechanisms for extracellular matrix (ECM) …
[HTML][HTML] Endothelial dysfunction in Marfan syndrome mice is restored by resveratrol
A Mieremet, M van der Stoel, S Li, E Coskun… - Scientific reports, 2022 - nature.com
Patients with Marfan syndrome (MFS) develop thoracic aortic aneurysms as the aorta
presents excessive elastin breaks, fibrosis, and vascular smooth muscle cell (vSMC) death …
presents excessive elastin breaks, fibrosis, and vascular smooth muscle cell (vSMC) death …
[HTML][HTML] Functional analysis of an intronic FBN1 pathogenic gene variant in a family with marfan syndrome
K Hu, Y Wan, FT Lee, J Chen, H Wang, H Qu… - Frontiers in …, 2022 - frontiersin.org
Marfan syndrome (MFS) is an autosomal dominant connective tissue disorder that
canonically affects the ocular, skeletal, and cardiovascular system, in which aortic tear and …
canonically affects the ocular, skeletal, and cardiovascular system, in which aortic tear and …