Article
The Multiple Functions of Fibrillin-1 Microfibrils in Organismal Physiology.
International journal of molecular sciences - 8 Feb 2022
Asano Keiichi, Cantalupo Anna, Sedes Lauriane, Ramirez Francesco
Abstract excerpt
Fibrillin-1 is the major structural component of the 10 nm-diameter microfibrils that confer key physical and mechanical properties to virtually every tissue, alone and together with elastin in the elastic fibers. Mutations in fibrillin-1 cause pleiotropic manifestations in Marfan syndrome (MFS), including dissecting thoracic aortic aneurysms, myocardial dysfunction, progressive bone loss, disproportionate...
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