Fibulins: Multiple roles in matrix structures and tissue functions

S. De Vega, T. Iwamoto, Y. Yamada

    Research output: Contribution to journalReview articlepeer-review

    177 Citations (Scopus)

    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.

    Original languageEnglish
    Pages (from-to)1890-1902
    Number of pages13
    JournalCellular and Molecular Life Sciences
    Volume66
    Issue number11-12
    DOIs
    Publication statusPublished - 2009 Jun

    Keywords

    • Angiogenesis
    • Elastogenesis
    • Embryonic development
    • Extracellular matrix network
    • Fibulins
    • Genetic disorders
    • Protein interactions

    ASJC Scopus subject areas

    • Molecular Medicine
    • Molecular Biology
    • Pharmacology
    • Cellular and Molecular Neuroscience
    • Cell Biology

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