Stabilisation of extracellular matrix is crucial to rapamycin-mediated life span increase in Marfan mgR/mgR mice

Marfan syndrome is a hereditary connective tissue disorder caused by heterozygous mutations in the fibrillin-1 gene (FBN1) and altered TGF-β signalling. Life-threatening complications involve thoracic aortic aneurysms (TAA) and dissections due to the disruption of microfibrillar assembly in the aortic wall. We previously demonstrated that Rapamycin, a typical mTOR pathway inhibitor, limits the ascending aorta elastolysis and expansion, significantly increasing lifespan in an established …