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 …

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 …

[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 …

[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 …

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 …

[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 …

[HTML][HTML] Induction of macrophage chemotaxis by aortic extracts from patients with Marfan syndrome is related to elastin binding protein

G Guo, P Gehle, S Doelken, JL Martin-Ventura… - PloS one, 2011 - journals.plos.org
Marfan syndrome is an autosomal dominantly inherited disorder of connective tissue with
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 …

[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 …

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 …