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• W4313205300 abstract "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 occurred before aortic dilatation. To reverse EC morphology, we performed treatment with resveratrol. This restored EC blood flow alignment, junctional linearity, phospho-eNOS expression, and improved the structural integrity of the internal elastic lamina of Fbn1 C1041G/+ mice. In conclusion, these experiments identify the involvement of ECs and underlying internal elastic lamina in MFS aortic pathology, which could act as potential target for future MFS pharmacotherapies." @default.
• W4313205300 created "2023-01-06" @default.
• W4313205300 creator A5002187107 @default.
• W4313205300 creator A5048416392 @default.
• W4313205300 creator A5051494885 @default.
• W4313205300 creator A5060265848 @default.
• W4313205300 creator A5068007728 @default.
• W4313205300 creator A5073397759 @default.
• W4313205300 creator A5084610580 @default.
• W4313205300 date "2022-12-28" @default.
• W4313205300 modified "2023-09-26" @default.
• W4313205300 title "Endothelial dysfunction in Marfan syndrome mice is restored by resveratrol" @default.
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