Fibrillin-1, a calcium binding protein of extracellular matrix

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 syndro me and related disorders, which are characterised by defects in the skeleta l, cardiovascular and ocular systems of the body. Fibrillin-1 has a strikin g modular organisation which is dominated by multiple tandem repeats of the calcium binding epidermal growth factor-like (cbEGF) domain. This review f ocuses on recent studies which have investigated the structural and functio nal role of calcium binding to cbEGF domains in fibrillin-1 and 10-12 nm mi crofibrils. (C) 2000 Elsevier Science B.V. All rights reserved.