Abstract.
The fibulins are a family of secreted glycoproteins associated with basement membranes, elastic fibers, and other matrices. They are expressed in a variety of tissues. Association with these matrix structures is mediated by their ability to interact with many extracellular matrix constituents. The seven members of the family are defined by the presence of two structural modules, a tandem repeat of epidermal growth factor-like modules and a unique C-terminal fibulin-type module. They act not only as intermolecular bridges within the extracellular matrix to form supramolecular structures, but also as mediators for cellular processes and tissue remodeling. These important functions of fibulins in a wide range of biological processes have been shown in in vitro systems, gene knockout mice, and human genetic disorders. In this review, we describe the structure and function of these proteins and discuss the implication of fibulins in development and diseases.
Similar content being viewed by others
Author information
Authors and Affiliations
Corresponding author
Additional information
Received 3 October 2008; received after revision 9 January 2009; accepted 16 January 2009
Rights and permissions
About this article
Cite this article
de Vega, S., Iwamoto, T. & Yamada, Y. Fibulins: Multiple roles in matrix structures and tissue functions. Cell. Mol. Life Sci. 66, 1890–1902 (2009). https://doi.org/10.1007/s00018-009-8632-6
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00018-009-8632-6