Disturbed nitric oxide signalling gives rise to congenital bicuspid aortic valve and aortopathy.

ABSTRACT: Patients with a congenital bicuspid aortic valve (BAV), a valve with two instead of three aortic leaflets, have an increased risk of developing thoracic aneurysms and aortic dissection. The mechanisms underlying BAV-associated aortopathy are poorly understood. This study examined BAV-associated aortopathy in Nos3^-/- mice, a model with congenital BAV formation. A combination of histological examination and in vivo ultrasound imaging was used to investigate aortic dilation and dissections in Nos3^-/- mice. Moreover, cell lineage analysis and single-cell RNA sequencing were used to observe the molecular anomalies within vascular smooth muscle cells (VSMCs) of Nos3^-/- mice. Spontaneous aortic dissections were found in ascending aortas

SUBMITTER: Peterson JC 

PROVIDER: S-EPMC7541347 | biostudies-literature | 2020 Sep

REPOSITORIES: biostudies-literature