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

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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Main Authors: 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
Format: Article
Language:English
Published: Nature Portfolio 2025-07-01
Series:Scientific Reports
Subjects:
Marfan syndrome
Aorta
Extracellular matrix
Fibrillin-1
Aneurysm
Collagen
Online Access:https://doi.org/10.1038/s41598-025-09665-w
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