Excessive glycosylation drives thoracic aortic aneurysm formation through integrated stress response.

ABSTRACT:

Background and aims

Thoracic aortic aneurysms and dissections (TAADs) are depicted by aortic medial degeneration characterized by glycan-rich matrix accumulation. Marfan syndrome (MFS) is the most common inherited connective tissue disorder associated with TAAD. Although vascular smooth muscle cell metabolic dysfunction has emerged as a pathogenic driver of TAAD, surgical repair remains the mainstay of treatment. This study aimed to investigate the role of the hexosamine biosynthetic pathway (HBP) in sporadic and genetic TAAD pathophysiology.

Methods

Hexosamine biosynthetic pathway activation was analysed in aortas from an MFS mouse model, a β-aminopropionitrile-induced non-genetic TAAD model, and patients with sporadic TAAD using transcriptomic and metabolomic approaches. A

SUBMITTER: Rochano-Ortiz A 

PROVIDER: S-EPMC12665370 | biostudies-literature | 2025 Dec

REPOSITORIES: biostudies-literature