Send to

Choose Destination
See comment in PubMed Commons below
Connect Tissue Res. 2008;49(1):1-6. doi: 10.1080/03008200701820708.

Fibrillin-rich microfibrils - structural and instructive determinants of mammalian development and physiology.

Author information

Child Health Institute of New Jersey, Robert W. Johnson Medical School, New Brunswick, New Jersey, USA.


Fibrillin-rich microfibrils have emerged recently as an informative model system in which to study fundamental questions related to extracellular matrix biology and connective tissue pathophysiology. As a result, these studies have yielded novel clinical concepts and promising therapeutic strategies. These achievements have been based on the realization from studies of genetically engineered mice that mutations in fibrillin-rich microfibrils impair both the structural integrity of connective tissues and signaling events by TGF-beta/BMP superfamily members. In this view, fibrillin-rich microfibrils represent architectural assemblies that specify the concentration and timely release of local effectors of morphogenesis and tissue remodeling, in addition to conferring structural integrity to individual organ systems. This review summarizes the evidence supporting our current understanding of the structural and instructive roles that fibrillin-rich microfibrils play during embryonic development and in human diseases.

[Indexed for MEDLINE]
PubMed Commons home

PubMed Commons

How to join PubMed Commons

    Supplemental Content

    Full text links

    Icon for Taylor & Francis
    Loading ...
    Support Center