FN (Fibronectin)-Integrin α5 Signaling Promotes Thoracic Aortic Aneurysm in a Mouse Model of Marfan Syndrome.

ABSTRACT:

Background

Marfan syndrome, caused by mutations in the gene for fibrillin-1, leads to thoracic aortic aneurysms (TAAs). Phenotypic modulation of vascular smooth muscle cells (SMCs) and ECM (extracellular matrix) remodeling are characteristic of both nonsyndromic and Marfan aneurysms. The ECM protein FN (fibronectin) is elevated in the tunica media of TAAs and amplifies inflammatory signaling in endothelial and SMCs through its main receptor, integrin α5β1. We investigated the role of integrin α5-specific signals in Marfan mice in which the cytoplasmic domain of integrin α5 was replaced with that of integrin α2 (denoted α5/2 chimera).

Methods

We crossed α5/2 chimeric mice with Fbn1^mgR/mgR mice (mgR model of Marfan syndrome) to evaluate the survival rate and

SUBMITTER: Chen M 

PROVIDER: S-EPMC10133209 | biostudies-literature | 2023 May

REPOSITORIES: biostudies-literature