Article

A Marfan-Associated FBN1 Nonsense Mutation Mouse Model Reveals Adventitial Inflammation During Aneurysm Progression

2025-09-25

Abstract excerpt

Marfan syndrome (MFS) is a genetic disorder caused by mutations in fibrillin-1(FBN1) , which encodes FBN1, a key structural component of the extracellular matrix. Mutations in FBN1 influence the severity of aortic disease and therapeutic responses, with aortic aneurysm being the leading cause of mortality in patients with MFS. To investigate the mechanisms driving aneurysm progression, we generated a mouse model...

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Literature Corpus work: a811d86c-275c-5384-8512-f72ddf3c91f3
DOI: 10.1101/2025.09.23.678101

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