Abstract
Fibulins are a newly recognized family of extracellular matrix proteins. The five known members of the family share an elongated structure and many calcium-binding sites, owing to the presence of tandem arrays of epidermal growth factor-like domains. They have overlapping binding sites for several basement-membrane proteins, tropoelastin, fibrillin, fibronectin and proteoglycans, and they participate in diverse supramolecular structures. New insights into their biological roles are now emerging from studies of transgenic mice and of some inherited human diseases.
Publication types
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Research Support, Non-U.S. Gov't
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Research Support, U.S. Gov't, P.H.S.
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Review
MeSH terms
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Alternative Splicing
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Animals
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Binding Sites
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Calcium / metabolism
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Calcium-Binding Proteins / genetics
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Calcium-Binding Proteins / metabolism*
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Calcium-Binding Proteins / physiology*
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Cell Division
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Chromosome Mapping
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Dimerization
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Exons
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Extracellular Matrix / metabolism*
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Introns
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Ligands
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Mice
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Mice, Transgenic
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Models, Biological
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Models, Molecular
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Protein Binding
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Protein Isoforms
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Protein Structure, Tertiary
Substances
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Calcium-Binding Proteins
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Ligands
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Protein Isoforms
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fibulin
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Calcium