Microfibrils and fibrillin-1 induce integrin-mediated signaling, proliferation and migration in human endothelial cells
B Mariko, Z Ghandour, S Raveaud… - … of Physiology-Cell …, 2010 - journals.physiology.org
Microfibrils are macromolecular complexes associated with elastin to form elastic fibers that
endow extensible tissues, such as arteries, lungs, and skin, with elasticity property. Fibrillin …
endow extensible tissues, such as arteries, lungs, and skin, with elasticity property. Fibrillin …
Dysfunction of endothelial and smooth muscle cells in small arteries of a mouse model of Marfan syndrome
HT Syyong, AWY Chung, HHC Yang… - British journal of …, 2009 - Wiley Online Library
Background and purpose: Marfan syndrome, a connective tissue disorder caused by
mutations in FBN1 encoding fibrillin‐1, results in life‐threatening complications in the aorta …
mutations in FBN1 encoding fibrillin‐1, results in life‐threatening complications in the aorta …
[HTML][HTML] Promising novel therapies in the treatment of aortic and visceral aneurysms
TM Stougiannou, KC Christodoulou… - Journal of Clinical …, 2023 - mdpi.com
Aortic and visceral aneurysms affect large arterial vessels, including the thoracic and
abdominal aorta, as well as visceral arterial branches, such as the splenic, hepatic, and …
abdominal aorta, as well as visceral arterial branches, such as the splenic, hepatic, and …
[HTML][HTML] Aortic dimensions, biophysical properties, and plasma biomarkers in children and adults with marfan or loeys-dietz syndrome
JZ Cui, KC Harris, K Raedschelders, Z Hollander… - CJC open, 2021 - Elsevier
Background Aortic dilation, stiffening, and dissection are common and potentially lethal
complications of Marfan syndrome (MFS) and Loeys-Dietz syndrome (LDS), which involve …
complications of Marfan syndrome (MFS) and Loeys-Dietz syndrome (LDS), which involve …
An exploration of alternative therapeutic targets for aortic disease in Marfan syndrome
LJF Van Den Heuvel, S Peeters, JAN Meester… - Drug Discovery …, 2024 - Elsevier
Highlights•No medical intervention can completely arrest aneurysmal growth.•Drugs with
immediate translational potential in Marfan syndrome include resveratrol, doxycycline …
immediate translational potential in Marfan syndrome include resveratrol, doxycycline …
[HTML][HTML] In vivo characterization of doxycycline-mediated protection of aortic function and structure in a mouse model of Marfan syndrome-associated aortic aneurysm
JZ Cui, L Lee, X Sheng, F Chu, CP Gibson… - Scientific reports, 2019 - nature.com
Aortic aneurysm is the most life-threatening complication in Marfan syndrome (MFS)
patients. Doxycycline, a nonselective matrix metalloproteinases inhibitor, was reported to …
patients. Doxycycline, a nonselective matrix metalloproteinases inhibitor, was reported to …
[HTML][HTML] Induction of macrophage chemotaxis by aortic extracts from patients with Marfan syndrome is related to elastin binding protein
Marfan syndrome is an autosomal dominantly inherited disorder of connective tissue with
prominent skeletal, ocular, and cardiovascular manifestations. Aortic aneurysm and …
prominent skeletal, ocular, and cardiovascular manifestations. Aortic aneurysm and …
The RNA-binding fragile-X mental retardation protein and its role beyond the brain
C Malecki, BD Hambly, RW Jeremy, EN Robertson - Biophysical Reviews, 2020 - Springer
It is well-established that variations of a CGG repeat expansion in the gene FMR1, which
encodes the fragile-X mental retardation protein (FMRP), cause the neurocognitive disorder …
encodes the fragile-X mental retardation protein (FMRP), cause the neurocognitive disorder …
[HTML][HTML] Characterization of doxycycline-mediated inhibition of Marfan syndrome-associated aortic dilation by multiphoton microscopy
AY Tehrani, JZ Cui, T Bucky Jones, E Hotova… - Scientific reports, 2020 - nature.com
Marfan syndrome (MFS) is a connective tissue disorder that results in aortic root widening
and aneurysm if unmanaged. We have previously reported doxycycline, a nonselective …
and aneurysm if unmanaged. We have previously reported doxycycline, a nonselective …
Infusion of Hibiscus sabdariffa L. Modulates Oxidative Stress in Patients with Marfan Syndrome
ME Soto, A Zuñiga-Muñoz… - Mediators of …, 2016 - Wiley Online Library
Marfan syndrome (MFS) is associated with progressive aortic dilatation, endothelial
dysfunction, and oxidative stress that contribute to the early acute dissection of the vessel …
dysfunction, and oxidative stress that contribute to the early acute dissection of the vessel …