Aortic & peripheral vascular disease
PIEZO1 attenuates Marfan syndrome aneurysm development through TGF-β signaling pathway inhibition via TGFBR2
Authors' conclusion
Piezo1 deficiency aggravates MFS aneurysms by promoting TGF-β signalling pathway activation via TGF-β receptor 2 endocytosis and a decrease in autophagy.
Study context
- Population
- Marfan syndrome (MFS) mice (Fbn1C1041G/+) and patients with MFS
- Comparison
- Piezo1 VSMC conditional knockout MFS mice vs MFS mice
Main finding
As reported in the abstract
- PIEZO1 expression decreased in aortas of MFS mice
- MFS × CKO mice showed aggravated thoracic aortic aneurysm (TAA), inflammation, extracellular matrix remodelling and TGF-β pathway activation vs MFS mice
- PIEZO1 knockout exacerbated TGF-β pathway activation by inhibiting Rab GTPase 3C-mediated endocytosis and autophagy of TGF-β receptor 2
- Pharmacological PIEZO1 activation with Yoda1 prevented TGF-β pathway activation and reversed TAA in MFS mice
Original publication
PIEZO1 attenuates Marfan syndrome aneurysm development through TGF-β signaling pathway inhibition via TGFBR2. Eur Heart J. Published 25 November 2024. doi:10.
Summarised from the published abstract; every number is checked against it