Extracellular matrix and vascular dynamics in the kidney of a murine model for Marfan syndrome.

Extracellular matrix and vascular dynamics in the kidney of a murine model for Marfan syndrome.

Fibrillin-1 is a pivotal structural component of the kidney's glomerulus and peritubular tissue. Mutations in the fibrillin-1 gene result in Marfan syndrome (MFS), an autosomal dominant disease of the connective tissue. Although the kidney is not considered a classically affected organ in MFS,...

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Bibliographic Details
Main Authors: Rodrigo Barbosa de Souza, Renan Barbosa Lemes, Orestes Foresto-Neto, Luara Lucena Cassiano, Dieter P Reinhardt, Keith M Meek, Ivan Hong Jun Koh, Philip N Lewis, Lygia V Pereira
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2023-01-01
Series:PLoS ONE
Online Access:https://doi.org/10.1371/journal.pone.0285418

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https://doi.org/10.1371/journal.pone.0285418

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