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

Journal article

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.

Authors: Handford, P

• Publication status: Published
• Journal: Biochimica et biophysica acta
• Volume: 1498
• Issue: 2-3
• Pages: 84-90
• Publication date: 2000-12-01
• DOI: 10.1016/s0167-4889(00)00085-9
• ISSN: 0006-3002
• Language: English
• Keywords: Microfilament Proteins; Humans; Microfibrils; Epidermal Growth Factor; Calcium-Binding Proteins; Fibroblasts; Extracellular Matrix Proteins; Tandem Repeat Sequences; Mutation; Egtazic Acid; Calcium; Marfan Syndrome; Calcium Chloride; Animals