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

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) phe...

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Detalles Bibliográficos
Autores principales: Dale, Matthew, Fitzgerald, Matthew P., Liu, Zhibo, Meisinger, Trevor, Karpisek, Andrew, Purcell, Laura N., Carson, Jeffrey S., Harding, Paul, Lang, Haili, Koutakis, Panagiotis, Batra, Rishi, Mietus, Constance J., Casale, George, Pipinos, Iraklis, Baxter, B. Timothy, Xiong, Wanfen
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5645122/
https://www.ncbi.nlm.nih.gov/pubmed/29040313
http://dx.doi.org/10.1371/journal.pone.0186603