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Redox Dysregulation of Vascular Smooth Muscle Sirtuin-1 in Thoracic Aortic Aneurysm in Marfan Syndrome.

Arterioscler Thromb Vasc Biol

August 2023

Vascular Biology Section (E.B., S.S.P.D.L., Y.T., H.L., Y.H., Y.W., P.M.S., X.Y., J.B.G., X.W., J.H., F.S.), Department of Medicine, Boston University Chobenian & Avedisian School of Medicine, MA.

Background: Thoracic aortic aneurysms (TAAs) are abnormal aortic dilatations and a major cardiovascular complication of Marfan syndrome. We previously demonstrated a critical role for vascular smooth muscle (VSM) SirT1 (sirtuin-1), a lysine deacetylase, against maladaptive aortic remodeling associated with chronic oxidative stress and aberrant activation of MMPs (matrix metalloproteinases).

Methods: In this study, we investigated whether redox dysregulation of SirT1 contributed to the pathogenesis of TAA using fibrillin-1 hypomorphic mice (Fbn1), an established model of Marfan syndrome prone to aortic dissection/rupture.

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