Update on ADAMTS13 and VWF in cardiovascular and hematological disorders

作者：Akyol Omer^*; Akyol Sumeyya; Chen Chu Huang^*

来源：Clinica Chimica Acta, 2016, 463: 109-118.

DOI：10.1016/j.cca.2016.10.017

• ADAMTS13
• von Willebrand factor
• Thrombotic thrombocytopenic purpura
• Arterial thrombosis
• Cardiovascular diseases
• Hematological diseases

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.

• 出版日期2016-12-1