Cellular and Molecular Life Sciences

, Volume 64, Issue 18, pp 2437–2446

Extracellular microfibrils in development and disease

  • F. Ramirez
  • L. Y. Sakai
  • D. B. Rifkin
  • H. C. Dietz
Review

DOI: 10.1007/s00018-007-7166-z

Cite this article as:
Ramirez, F., Sakai, L.Y., Rifkin, D.B. et al. Cell. Mol. Life Sci. (2007) 64: 2437. doi:10.1007/s00018-007-7166-z

Abstract.

Fibrillins are the structural components of extracellular microfibrils that impart physical properties to tissues, alone or together with elastin as elastic fibers. Genetic studies in mice have revealed that fibrillin-rich microfibrils are also involved in regulating developmental programs and homeostatic processes through the modulation of TGF-β/BMP signaling events. A new paradigm has thus emerged whereby the spatiotemporal organization of microfibrils dictates both the cellular activities and physical properties of connective tissues. These observations have paved the way to novel therapeutic approaches aimed at counteracting the life-threatening complications in human conditions caused by dysfunctions of fibrillin-rich microfibrils.

Keywords.

Aortic aneurysm development and morphogenesis emphysema fibrillin extracellular matrix Marfan syndrome myopathy TGF-β/BMP signaling molecules 

Copyright information

© Birkhäuser Verlag, Basel 2007

Authors and Affiliations

  • F. Ramirez
    • 1
  • L. Y. Sakai
    • 2
  • D. B. Rifkin
    • 3
  • H. C. Dietz
    • 4
  1. 1.Department of PediatricsChild Health Institute of New Jersey, UMDNJ-Robert Wood Johnson Medical SchoolNew BrunswickUSA
  2. 2.Research DepartmentShriners Hospital for ChildrenPortlandUSA
  3. 3.Department of Cell BiologyNew York University School of MedicineNew YorkUSA
  4. 4.Departments of Pediatrics, Medicine and Molecular Biology and Genetics, and Howard Hughes Medical InstituteJohn Hopkins University School of MedicineBaltimoreUSA