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Pathophysiology and Therapeutics of Thoracic Aortic Aneurysm in Marfan Syndrome

Department of Pharmacological Sciences, Icahn School of Medicine at Mount Sinai, New York, NY 10029, USA
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Author to whom correspondence should be addressed.
Academic Editor: Hisashi Sawada
Biomolecules 2022, 12(1), 128; https://doi.org/10.3390/biom12010128
Received: 30 November 2021 / Revised: 4 January 2022 / Accepted: 6 January 2022 / Published: 14 January 2022
About 20% of individuals afflicted with thoracic aortic disease have single-gene mutations that predispose the vessel to aneurysm formation and/or acute aortic dissection often without associated syndromic features. One widely studied exception is Marfan syndrome (MFS) in which mutations in the extracellular protein fibrillin-1 cause additional abnormalities in the heart, eyes, and skeleton. Mouse models of MFS have been instrumental in delineating major cellular and molecular determinants of thoracic aortic disease. In spite of research efforts, translating experimental findings from MFS mice into effective drug therapies for MFS patients remains an unfulfilled promise. Here, we describe a series of studies that have implicated endothelial dysfunction and improper angiotensin II and TGFβ signaling in driving thoracic aortic disease in MFS mice. We also discuss how these investigations have influenced the way we conceptualized possible new therapies to slow down or even halt aneurysm progression in this relatively common connective tissue disorder. View Full-Text
Keywords: angiotensin II; arterial disease; endothelial dysfunction; Marfan syndrome; NO signaling; oxidative stress; TGFβ; thoracic aortic aneurysm angiotensin II; arterial disease; endothelial dysfunction; Marfan syndrome; NO signaling; oxidative stress; TGFβ; thoracic aortic aneurysm
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MDPI and ACS Style

Asano, K.; Cantalupo, A.; Sedes, L.; Ramirez, F. Pathophysiology and Therapeutics of Thoracic Aortic Aneurysm in Marfan Syndrome. Biomolecules 2022, 12, 128. https://doi.org/10.3390/biom12010128

AMA Style

Asano K, Cantalupo A, Sedes L, Ramirez F. Pathophysiology and Therapeutics of Thoracic Aortic Aneurysm in Marfan Syndrome. Biomolecules. 2022; 12(1):128. https://doi.org/10.3390/biom12010128

Chicago/Turabian Style

Asano, Keiichi, Anna Cantalupo, Lauriane Sedes, and Francesco Ramirez. 2022. "Pathophysiology and Therapeutics of Thoracic Aortic Aneurysm in Marfan Syndrome" Biomolecules 12, no. 1: 128. https://doi.org/10.3390/biom12010128

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