Novel Aortic Dissection Model Links Endothelial Dysfunction and Immune Infiltration.

ABSTRACT:

Background

Aortic dissection (AD) is the separation of medial layers of the aorta and is a major cause of death in patients with connective tissue disorders such as Marfan syndrome. However, molecular triggers instigating AD, its temporospatial progression, and how vascular cells in each vessel layer interact and participate in the pathological process remain incompletely understood. To unravel the underlying molecular mechanisms of AD, we generated a spontaneous AD mouse model.

Methods

We incorporated a novel missense variant (p.G234D) in FBN1, the gene for fibrillin-1, identified in a patient with nonsyndromic familial AD into mice using the CRISPR/Cas9 (clustered regularly interspaced short palindromic repeats/CRISPR-associated protein 9) system. We performed molec

SUBMITTER: Kimura K 

PROVIDER: S-EPMC12175827 | biostudies-literature | 2025 Jun

REPOSITORIES: biostudies-literature