Sex, pregnancy and aortic disease in Marfan syndrome.

ABSTRACT:

Background

Sex-related differences as well as the adverse effect of pregnancy on aortic disease outcome are well-established phenomena in humans with Marfan syndrome (MFS). The underlying mechanisms of these observations are largely unknown.

Objectives

In an initial (pilot) step we aimed to confirm the differences between male and female MFS patients as well as between females with and without previous pregnancy. We then sought to evaluate whether these findings are recapitulated in a pre-clinical model and performed in-depth cardiovascular phenotyping of mutant male and both nulliparous and multiparous female Marfan mice. The effect of 17β-estradiol on fibrillin-1 protein synthesis was compared in vitro using human aortic smooth muscle cells and fibroblasts.

Results

SUBMITTER: Renard M 

PROVIDER: S-EPMC5510874 | biostudies-literature | 2017

REPOSITORIES: biostudies-literature