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Exploring thoracic aorta ECM alterations in Marfan syndrome: insights into aorta wall structure

Abstract Marfan syndrome is a connective tissue disorder caused by FBN1 mutations, leading to aortic wall fragility and increased susceptibility to aneurysm and dissection. This study investigated microstructural and molecular alterations in the thoracic aorta of Fbn1mgΔlpn mice, with a focus on the...

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Principais autores: Rodrigo Barbosa de Souza, Luara Lucena Cassiano, Philipp Barnowski, Sara Ventura, Walter Miguel Turato, Suelen Cristina Russafa Nascimento, Giovanna Lodi Mignanelli, Waldir Caldeira, Ana Maria Cristina Rebelo Pinto da Fonseca Martins, Flavio de Carvalho Luposeli, Francisco Rafael Martins Laurindo, Dieter P. Reinhardt, Gerhard Sengle, Ivan Hong Jun Koh, Keith M. Meek, Philip N. Lewis
Formato: Artigo
Idioma:Inglês
Publicado em: Nature Portfolio 2025-07-01
Colecção:Scientific Reports
Assuntos:
Marfan syndrome
Aorta
Extracellular matrix
Fibrillin-1
Aneurysm
Collagen
Acesso em linha:https://doi.org/10.1038/s41598-025-09665-w
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