Laminin isoforms in development and disease

Susanne Schéele, Alexander Nyström, Madeleine Durbeej, Jan Fredrik Talts, Marja Ekblom, Peter Ekblom

    94 Citations (Scopus)

    Abstract

    The members of the laminin family of heterotrimers are major constituents of all basement membranes, sheet-like extracellular structures, present in almost all organs. The laminins bind to cell surface receptors and thereby tightly connect the basement membrane to the adjacent cell layer. This provides for the specific basement membrane functions to stabilize cellular structures, to serve as effective physical barriers, and furthermore, to govern cell fate by inducing intracellular signalling cascades. Many different types of diseases involve basement membrances and laminins. Metastasizing solid tumors must pass through basement membranes to reach the vascular system, and various microbes and viruses enter the cells through direct interaction with laminins. Furthermore, whereas mutations in one specific laminin chain lead to a muscular disorder, mutations of other laminin chains cause skin blistering and kidney defects, respectively. This review summarizes recent progress concerning the molecular mechanisms of laminins in development and disease. The current knowledge may lead to clinical treatment of lamininopathies and may include stem-cell approaches as well as gene therapy.
    Original languageEnglish
    JournalJournal of Molecular Medicine
    Volume85
    Issue number8
    Pages (from-to)825-836
    Number of pages12
    ISSN0946-2716
    DOIs
    Publication statusPublished - 2007

    Keywords

    • Former LIFE faculty
    • Lamininopathies
    • Integrin
    • Dystroglycan
    • Basement membranes

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