1EMO

NMR STUDY OF A PAIR OF FIBRILLIN CA2+ BINDING EPIDERMAL GROWTH FACTOR-LIKE DOMAINS, 22 STRUCTURES

1EMO の概要

• エントリーDOI: 10.2210/pdb1emo/pdb
• 関連するPDBエントリー: 1EMN
• 分子名称: FIBRILLIN, CALCIUM ION (2 entities in total)
• 機能のキーワード: extracellular matrix, calcium-binding, glycoprotein, multigene family, disease mutation, egf-like domain, human fibrillin-1 fragment, matrix protein
• 由来する生物種: Homo sapiens (human)
• タンパク質・核酸の鎖数: 1
• 化学式量合計: 8830.87

構造登録者

Downing, A.K.,Campbell, I.D.,Handford, P.A. (登録日: 1996-08-05, 公開日: 1996-12-23, 最終更新日: 2024-10-23)

主引用文献

Downing, A.K.,Knott, V.,Werner, J.M.,Cardy, C.M.,Campbell, I.D.,Handford, P.A.
Solution structure of a pair of calcium-binding epidermal growth factor-like domains: implications for the Marfan syndrome and other genetic disorders.
Cell(Cambridge,Mass.), 85:597-605, 1996

PubMed Abstract: The nuclear magnetic resonance structure of a covalently linked pair of calcium-binding (cb) epidermal growth factor-like (EGF) domains from human fibrillin-1, the protein defective in the Marfan syndrome, is described. The two domains are in a rigid, rod-like arrangement, stabilized by interdomain calcium binding and hydrophobic interactions. We propose a model for the arrangement of fibrillin monomers in microfibrils that reconciles structural and antibody binding data, and we describe a set of disease-causing mutations that provide the first clues to the specificity of cbEFG interactions. The residues involved in stabilizing the domain linkage are highly conserved in fibrillin, fibulin, thrombomodulin, and the low density lipoprotein receptor. We propose that the relative orientation of tandem cbEGF domains in these proteins is similar, but that in others, including Notch, pairs adopt a completely different conformation.

PubMed: 8653794
DOI: 10.1016/S0092-8674(00)81259-3

実験手法

SOLUTION NMR