Structure and function of von Willebrand factor: the protein that is deficient and/or abnormal in inherited von Willebrand disease

被引：53

作者：

Hassan, Md Imtaiyaz ^{[1
]}

Saxena, Aditya ^{[1
]}

Ahmad, Faizan ^{[1
]}

机构：

[1] Jamia Millia Islamia, Ctr Interdisciplinary Res Basic Sci, New Delhi 110025, India

来源：

BLOOD COAGULATION & FIBRINOLYSIS | 2012年 / 23卷 / 01期

关键词：

biomarker; blood clotting; cell adhesion; fibrinogen-binding; hemostasis; integrin GPIb; von Willebrand diseases; von Willebrand factor; AMINO-ACID-RESIDUES; FACTOR A1 DOMAIN; HUMAN VONWILLEBRAND-FACTOR; GLYCOPROTEIN IB-ALPHA; ALANINE-SCANNING MUTAGENESIS; COAGULATION-FACTOR-VIII; IMPROVED PERFORMANCE-CHARACTERISTICS; COFACTOR ACTIVITY ASSAY; HUMAN ENDOTHELIAL-CELLS; COLLAGEN-BINDING ASSAY;

D O I：

10.1097/MBC.0b013e32834cb35d

中图分类号：

R5 [内科学];

学科分类号：

100201 [内科学];

摘要：

von Willebrand factor (VWF) is a long plasma protein that contains many domains and each domain has its own function. VWF exists in a multimeric form and performs varieties of functions in the human body, including thrombus formation and blood coagulation. The crystal structures of three subdomains are known, and, interestingly, all three domains share identical three-dimensional fold with alpha-beta-alpha sandwiched model. VWF is directly associated with different types of von Willebrand disease. In this review, our aim is to gather recent developments on structure and functions of VWF and its clinical relevance. Blood Coagul Fibrinolysis 23: 11-22 (C) 2011 Wolters Kluwer Health vertical bar Lippincott Williams & Wilkins.

引用

页码：11 / 22

页数：12

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