Aortic & peripheral vascular disease
Excessive glycosylation drives thoracic aortic aneurysm formation through integrated stress response
Authors' conclusion
The HBP-ISR axis drives medial degeneration in TAAD.
Study context
- Population
- Marfan syndrome (MFS) mouse model, β-aminopropionitrile-induced non-genetic TAAD model, and patients with sporadic TAAD
- Comparison
- Hexosamine biosynthetic pathway (HBP) or integrated stress response (ISR) inhibition vs no inhibition in TAAD models
- Outcome
- HBP role in TAAD
Methodological detailsTranscriptomic and metabolomic analyses; aortic dilatation monitored by ultrasound; pharmacological inhibition of HBP and ISR
- Transcriptomic and metabolomic analyses; aortic dilatation monitored by ultrasound; pharmacological inhibition of HBP and ISR
Main finding
As reported in the abstract
- HBP up-regulated in MFS mouse model and β-aminopropionitrile-induced TAAD, and in aortic samples from MFS and sporadic TAAD patients
- Enhanced HBP activity contributed to aortic dilatation and medial degeneration via vascular smooth muscle cell dysfunction and ISR activation
- Inhibition of HBP or ISR reversed these effects in the MFS model
Original publication
Excessive glycosylation drives thoracic aortic aneurysm formation through integrated stress response. Eur Heart J. Published 28 July 2025. doi:10.
Summarised from the published abstract; every number is checked against it