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 (...

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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: Humans; Fibroblasts; Egtazic Acid; Calcium; Mutation; Extracellular Matrix Proteins; Calcium-Binding Proteins; Calcium Chloride; Epidermal Growth Factor; Microfibrils; Marfan Syndrome; Microfilament Proteins; Animals; Tandem Repeat Sequences