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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.

Type

Journal article

Journal

Biochim Biophys Acta

Publication Date

20/12/2000

Volume

1498

Pages

84 - 90

Keywords

Animals, Calcium, Calcium Chloride, Calcium-Binding Proteins, Egtazic Acid, Epidermal Growth Factor, Extracellular Matrix Proteins, Fibrillin-1, Fibrillins, Fibroblasts, Humans, Marfan Syndrome, Microfibrils, Microfilament Proteins, Mutation, Tandem Repeat Sequences