Related Experiment Video
Updated: Mar 13, 2026

Investigating von Willebrand Factor Pathophysiology Using a Flow Chamber Model of von Willebrand Factor-platelet String Formation
Published on: August 14, 2017
Update on ADAMTS13 and VWF in cardiovascular and hematological disorders
Omer Akyol1, Sumeyya Akyol2, Chu-Huang Chen3
1Vascular and Medicinal Research, Texas Heart Institute, Houston, TX, USA; Department of Medical Biochemistry, Faculty of Medicine, Hacettepe University, Ankara, Turkey.
Abstract:
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.
Related Concept Videos
Disorders of Hemostasis
Thromboembolic Disorders
Two factors primarily cause thromboembolic conditions.
Anticoagulant Drugs: Low-Molecular-Weight Heparins
Coagulation
During the coagulation phase, clotting factors, or procoagulants, play a vital role in initiating and progressing the coagulation cascade. This cascade is a series of reactions...
Formation of the Platelet Plug
As the injured blood vessel contracts, endothelial cells undergo contraction, revealing collagen fibers in the basement membrane and underlying connective tissue. Furthermore, the plasma membrane of endothelial cells becomes adhesive, preparing the site for platelet adhesion. Platelets...
Extrinsic and Intrinsic Pathways of Hemostasis
The Extrinsic Pathway
The extrinsic pathway of coagulation is typically initiated by tissue damage that exposes blood to tissue factor (TF), a protein released by the damaged tissue cells outside the blood vessels—this interaction with TF triggers biochemical reactions involving specific clotting factors. The key player here is Factor VII, which...
Vascular Spasm

