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Article

Premature aortic smooth muscle cell differentiation contributes to matrix dysregulation in Marfan Syndrome.

PloS one - 1 Jan 2017

Dale Matthew, Fitzgerald Matthew P, Liu Zhibo, Meisinger Trevor, Karpisek Andrew, Purcell Laura N, Carson Jeffrey S, Harding Paul, Lang Haili, Koutakis Panagiotis, Suh Melissa, Batra Rishi, Mietus Constance J, Casale George, Pipinos Iraklis, Baxter B Timothy, Xiong Wanfen

Abstract excerpt

Thoracic aortic aneurysm and dissection are life-threatening complications of Marfan syndrome (MFS). Studies of human and mouse aortic samples from late stage MFS demonstrate increased TGF-β activation/signaling and diffuse matrix changes. However, the role of the aortic smooth muscle cell (SMC) phenotype in early aneurysm formation in MFS has yet to be fully elucidated. As our objective, we investigated whether...

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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Identifiers

PMID: 29040313
DOI: 10.1371/journal.pone.0186603
PMCID: PMC5645122

Genes

KLF4

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