The Multiple Functions of Fibrillin-1 Microfibrils in Organismal Physiology

Keiichi Asano, Anna Cantalupo, Lauriane Sedes, Francesco Ramirez

Research output: Contribution to journalReview articlepeer-review

1 Scopus citations


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 skeletal growth, and the dislocation of the crystalline lens. The characterization of these MFS manifestations in mice, that replicate the human phenotype, have revealed that the underlying mechanisms are distinct and organ-specific. This brief review summarizes relevant findings supporting this conclusion.

Original languageEnglish
Article number1892
JournalInternational Journal of Molecular Sciences
Issue number3
StatePublished - 1 Feb 2022


  • Bone lengthening
  • Dilated cardiomyopathy
  • Fibrillin-1
  • Lens dislocation
  • Marfan syndrome
  • Osteopenia
  • Thoracic aortic aneurysm


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