Update on ADAMTS13 and VWF in cardiovascular and hematological disorders

被引：33

作者：

Akyol, Omer ^{[1
,2
]}

Akyol, Sumeyya ^{[1
]}

Chen, Chu-Huang ^{[1
,2
,3
,4
]}

机构：

[1] Texas Heart Inst, Vasc & Med Res, Houston, TX 77025 USA

[2] Hacettepe Univ, Dept Med Biochem, Fac Med, Ankara, Turkey

[3] Kaohsiung Med Univ, Lipid Sci & Aging Res Ctr, Kaohsiung, Taiwan

[4] Kaohsiung Med Univ Hosp, Ctr Lipid Biosci, Kaohsiung, Taiwan

来源：

CLINICA CHIMICA ACTA | 2016年 / 463卷

关键词：

ADAMTS13; von Willebrand factor; Thrombotic thrombocytopenic purpura; Arterial thrombosis; Cardiovascular diseases; Hematological diseases; VON-WILLEBRAND-FACTOR; THROMBOTIC THROMBOCYTOPENIC PURPURA; FACTOR-CLEAVING PROTEASE; ACUTE MYOCARDIAL-INFARCTION; CONGENITAL HEART-DISEASE; UPSHAW-SCHULMAN-SYNDROME; VASCULAR ENDOTHELIAL-CELLS; C-REACTIVE PROTEIN; ATRIAL-FIBRILLATION; ISCHEMIC-STROKE;

D O I：

10.1016/j.cca.2016.10.017

中图分类号：

R446 [实验室诊断]; R-33 [实验医学、医学实验];

学科分类号：

1001 ;

摘要：

Endothelial cells (EC) respond to injury by releasing numerous factors, including von Willebrand factor (VWF). High circulating levels of unusually large VWF multimers (UL-VWFM) have strong procoagulant activity and facilitate platelet adhesion and aggregation by interacting with platelets after an acute event superimposed on peripheral arterial disease and coronary artery disease. ADAMTS13-a disintegrin-like metalloproteinase with thrombospondin motif type 1 member 13 regulates a key physiological process of coagulation in the circulation by cleaving VWF multimers into small, inactive fragments. Low levels of ADAMTS13 in the blood may play a role in cardiovascular and hematological disorders, and clarifying its role may help improve disease management. The genetic, pharmacological, physiological, and pathological aspects related to ADAMTS13/VWF have been extensively investigated. Here, we provide an update on recent findings of the relationship between ADAMTS13 and hematological/cardiovascular disorders, including thrombotic thrombocytopenic purpura, arterial thrombosis, thrombotic microangiopathy, myocardial infarction, ischemic stroke, heart failure, and hypertension. (C) 2016 Elsevier B.V. All rights reserved.

引用

页码：109 / 118

页数：10

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