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Biochim Biophys Acta. 2000 Dec 20;1498(2-3):84-90.

Fibrillin-1, a calcium binding protein of extracellular matrix.

Author information

  • Division of Molecular and Cellular Biochemistry, Department of Biochemistry, University of Oxford, South Parks Road, OX1 3QU, Oxford, UK. penny@bioch.ox.ac.uk

Abstract

Fibrillin-1 is a large extracellular matrix glycoprotein which assembles to form 10-12 nm microfibrils in extracellular matrix. Mutations in the human fibrillin-1 gene (FBN-1) cause the connective tissue disease Marfan syndrome and related disorders, which are characterised by defects in the skeletal, cardiovascular and ocular systems of the body. Fibrillin-1 has a striking modular organisation which is dominated by multiple tandem repeats of the calcium binding epidermal growth factor-like (cbEGF) domain. This review focuses on recent studies which have investigated the structural and functional role of calcium binding to cbEGF domains in fibrillin-1 and 10-12 nm microfibrils.

PMID:
11108952
[PubMed - indexed for MEDLINE]
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