The aorta in Marfan syndrome: from molecular mechanisms to mechanobiological dysfunction.

Abstract excerpt

Marfan syndrome (MFS) is an autosomal dominant connective tissue disorder that affects cardiovascular, musculoskeletal, and ocular tissues, with premature death often resulting from dissection of the thoracic aorta. MFS results from pathogenic variants in FBN1, which encodes fibrillin-1, a glycoprotein that promotes elastic fibre organization and stability and contributes to smooth muscle cell mechano-sensing of...

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