Endothelial dysfunction in Marfan syndrome mice is restored by resveratrol.

ABSTRACT: Patients with Marfan syndrome (MFS) develop thoracic aortic aneurysms as the aorta presents excessive elastin breaks, fibrosis, and vascular smooth muscle cell (vSMC) death due to mutations in the FBN1 gene. Despite elaborate vSMC to aortic endothelial cell (EC) signaling, the contribution of ECs to the development of aortic pathology remains largely unresolved. The aim of this study is to investigate the EC properties in Fbn1^C1041G/+ MFS mice. Using en face immunofluorescence confocal microscopy, we showed that EC alignment with blood flow was reduced, EC roundness was increased, individual EC surface area was larger, and EC junctional linearity was decreased in aortae of Fbn1^C1041G/+ MFS mice. This modified EC phenotype was most prominent in the ascending aorta and

SUBMITTER: Mieremet A 

PROVIDER: S-EPMC9797556 | biostudies-literature | 2022 Dec

REPOSITORIES: biostudies-literature