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- W2167790003 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." @default.
- W2167790003 created "2016-06-24" @default.
- W2167790003 creator A5061413790 @default.
- W2167790003 date "2000-12-01" @default.
- W2167790003 modified "2023-10-03" @default.
- W2167790003 title "Fibrillin-1, a calcium binding protein of extracellular matrix" @default.
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- W2167790003 doi "https://doi.org/10.1016/s0167-4889(00)00085-9" @default.
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