Article

Intrinsic Mechanics of Human Stem Cell Derived Aortic Smooth Muscle Cells Support a Developmental Basis for Aneurysm Localization in Marfan Syndrome

2023-10-04

Abstract excerpt

Marfan Syndrome (MFS), a connective tissue disorder caused by a mutation in the fibrillin-1 gene, occurs in approximately 1 in 5,000 people worldwide. As an important constituent of the extracellular matrix, mutated fibrillin-1 in Marfan Syndrome leads to aortic medial degeneration, aneurysm, and dissection. TGFβ in the matrix, which is controlled by fibrillin-1, is known to cause pathological effects in smooth mu...

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Literature Corpus work: 29820f7d-1d22-5942-9c71-fad7698e485a
DOI: 10.1101/2023.10.03.560723

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