Fibrillin-1 and elastin fragmentation in the pathogenesis of thoracic aortic aneurysm in Marfan syndrome
G Guo - 2011 - refubium.fu-berlin.de
Das Marfan-Syndrom (MFS) ist eine relativ häufige autosomal dominant vererbte Störung
des Bindegewebes mit zahlreichen Symptomen im Skelettsystem, am Auge, und im …
des Bindegewebes mit zahlreichen Symptomen im Skelettsystem, am Auge, und im …
Loss of elastic fiber integrity and reduction of vascular smooth muscle contraction resulting from the upregulated activities of matrix metalloproteinase-2 and-9 in the …
AWY Chung, K Au Yeung, GGS Sandor… - Circulation …, 2007 - Am Heart Assoc
Thoracic aortic aneurysm (TAA) is the life-threatening complication of Marfan syndrome
(MFS), a connective tissue disorder caused by mutations in the fibrillin-1 gene. TAA is …
(MFS), a connective tissue disorder caused by mutations in the fibrillin-1 gene. TAA is …
Marfan syndrome: A therapeutic challenge for long-term care
AH Wagner, M Zaradzki, R Arif, A Remes… - Biochemical …, 2019 - Elsevier
Marfan syndrome (MFS) is an autosomal dominant genetic disorder caused by mutations in
the fibrillin-1 gene. Acute aortic dissection is the leading cause of death in patients suffering …
the fibrillin-1 gene. Acute aortic dissection is the leading cause of death in patients suffering …
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 …
Pathogenetic sequence for aneurysm revealed in mice underexpressing fibrillin-1
L Pereira, SY Lee, B Gayraud… - Proceedings of the …, 1999 - National Acad Sciences
Dissecting aortic aneurysm is the hallmark of Marfan syndrome (MFS) and the result of
mutations in fibrillin-1, the major constituent of elastin-associated extracellular microfibrils. It …
mutations in fibrillin-1, the major constituent of elastin-associated extracellular microfibrils. It …
Aortopathy in Marfan syndrome: an update
F Romaniello, D Mazzaglia, A Pellegrino… - Cardiovascular …, 2014 - Elsevier
Marfan syndrome (MFS) is an inherited autosomal dominant multisystem disease caused by
mutations in the FBN1 gene encoding fibrillin-1, an extracellular matrix glycoprotein widely …
mutations in the FBN1 gene encoding fibrillin-1, an extracellular matrix glycoprotein widely …
The pathogenesis of aortopathy in Marfan syndrome and related diseases
JA Jones, JS Ikonomidis - Current cardiology reports, 2010 - Springer
Marfan syndrome is a systemic connective tissue disorder that is inherited in an autosomal-
dominant pattern with variable penetrance. Although there are many clinical manifestations …
dominant pattern with variable penetrance. Although there are many clinical manifestations …
[HTML][HTML] The fibrillin-1 hypomorphic mgR/mgR murine model of Marfan syndrome shows severe elastolysis in all segments of the aorta
S Schwill, P Seppelt, J Grünhagen, CE Ott… - Journal of vascular …, 2013 - Elsevier
OBJECTIVE: Fibrillin-1 hypomorphic mice (mgR/mgR) are accepted as a model of Marfan
syndrome. Phenotypic investigations of this mouse have not previously included …
syndrome. Phenotypic investigations of this mouse have not previously included …
[HTML][HTML] Indomethacin prevents the progression of thoracic aortic aneurysm in Marfan syndrome mice
Background: Marfan syndrome (MFS), an inherited disorder of connective tissue
characterized by abnormalities in the skeletal, ocular, and cardiovascular systems, is caused …
characterized by abnormalities in the skeletal, ocular, and cardiovascular systems, is caused …
Insights into elastin fiber fragmentation: Mechanisms and treatment of aortic aneurysm in Marfan syndrome
Marfan syndrome (MFS) is an autosomal dominant connective tissue disorder caused by
fibrillin 1 (FBN1) gene mutations that results in defects in the skeletal, ocular, and …
fibrillin 1 (FBN1) gene mutations that results in defects in the skeletal, ocular, and …