Fibrillin-1, a calcium binding protein of extracellular matrix

被引：72

作者：

Handford, PA ^{[1
]}

机构：

[1] Univ Oxford, Dept Biochem, Div Mol & Cellular Biochem, Oxford OX1 3QU, England

来源：

BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH | 2000年 / 1498卷 / 2-3期

关键词：

fibrillin-1; calcium; calcium binding epidermal growth factor-like domain; extracellular matrix; Marfan syndrome;

D O I：

10.1016/S0167-4889(00)00085-9

中图分类号：

Q5 [生物化学]; Q7 [分子生物学];

学科分类号：

071010 ; 081704 ;

摘要：

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. (C) 2000 Elsevier Science B.V. All rights reserved.

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收藏

页码：84 / 90

页数：7

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