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 …
FBN1, which encodes the extracellular matrix protein fibrillin-1. To investigate the …
[HTML][HTML] p38 MAPK is an early determinant of promiscuous Smad2/3 signaling in the aortas of fibrillin-1 (Fbn1)-null mice
L Carta, S Smaldone, L Zilberberg, D Loch… - Journal of Biological …, 2009 - ASBMB
Excessive transforming growth factor-β (TGF-β) signaling characterizes the progression of
aortic aneurysm in mouse models of Marfan syndrome, a systemic disorder of the connective …
aortic aneurysm in mouse models of Marfan syndrome, a systemic disorder of the connective …
Targetting of the gene encoding fibrillin–1 recapitulates the vascular aspect of Marfan syndrome
L Pereira, K Andrikopoulos, J Tian, SY Lee… - Nature …, 1997 - nature.com
Aortic aneurysm and dissection account for about 2% of all deaths in industrialized
countries; they are also components of several genetic diseases, including Marfan …
countries; they are also components of several genetic diseases, including Marfan …
Skeletogenic phenotype of human Marfan embryonic stem cells faithfully phenocopied by patient-specific induced-pluripotent stem cells
N Quarto, B Leonard, S Li… - Proceedings of the …, 2012 - National Acad Sciences
Marfan syndrome (MFS) is a heritable connective tissue disorder caused by mutations in the
gene coding for FIBRILLIN-1 (FBN1), an extracellular matrix protein. MFS is inherited as an …
gene coding for FIBRILLIN-1 (FBN1), an extracellular matrix protein. MFS is inherited as an …
Marfan syndrome; A connective tissue disease at the crossroads of mechanotransduction, TGFβ signaling and cell stemness
F Ramirez, C Caescu, E Wondimu, J Galatioto - Matrix Biology, 2018 - Elsevier
Mutations in fibrillin-1 cause Marfan syndrome (MFS), the most common heritable disorder of
connective tissue. Fibrillin-1 assemblies (microfibrils and elastic fibers) represent a unique …
connective tissue. Fibrillin-1 assemblies (microfibrils and elastic fibers) represent a unique …
Single-cell transcriptomic profiling of vascular smooth muscle cell phenotype modulation in Marfan syndrome aortic aneurysm
Objective: To delineate temporal and spatial dynamics of vascular smooth muscle cell
(SMC) transcriptomic changes during aortic aneurysm development in Marfan syndrome …
(SMC) transcriptomic changes during aortic aneurysm development in Marfan syndrome …
Aortopathy in a mouse model of Marfan syndrome is not mediated by altered transforming growth factor β signaling
H Wei, JH Hu, SN Angelov, K Fox, J Yan… - Journal of the …, 2017 - Am Heart Assoc
Background Marfan syndrome (MFS) is caused by mutations in the gene encoding fibrillin‐1
(FBN 1); however, the mechanisms through which fibrillin‐1 deficiency causes MFS …
(FBN 1); however, the mechanisms through which fibrillin‐1 deficiency causes MFS …
Human Genome and Diseases:¶ The molecular pathogenesis of the Marfan syndrome
PN Robinson, P Booms - Cellular and Molecular Life Sciences CMLS, 2001 - Springer
The Marfan syndrome (MFS) is an autosomal dominant heritable disorder of connective
tissue with highly variable clinical manifestations including aortic dilatation and dissection …
tissue with highly variable clinical manifestations including aortic dilatation and dissection …
[HTML][HTML] A New Mouse Model for Marfan Syndrome Presents Phenotypic Variability Associated with the Genetic Background and Overall Levels of Fbn1 Expression
BL Lima, EJC Santos, GR Fernandes, C Merkel… - PloS one, 2010 - journals.plos.org
Marfan syndrome is an autosomal dominant disease of connective tissue caused by
mutations in the fibrillin-1 encoding gene FBN1. Patients present cardiovascular, ocular and …
mutations in the fibrillin-1 encoding gene FBN1. Patients present cardiovascular, ocular and …
Marfan syndrome: from gene to therapy
N Bolar, L Van Laer, BL Loeys - Current opinion in pediatrics, 2012 - journals.lww.com
Marfan syndrome: from gene to therapy : Current Opinion in Pediatrics Marfan syndrome: from
gene to therapy : Current Opinion in Pediatrics Log in or Register Subscribe to journalSubscribe …
gene to therapy : Current Opinion in Pediatrics Log in or Register Subscribe to journalSubscribe …
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