title: Update on ADAMTS13 and VWF in cardiovascular and hematological disorders.
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.
authors.affiliation: Vascular and Medicinal Research, Texas Heart Institute, Houston, TX, USA; Department of Medical Biochemistry, Faculty of Medicine, Hacettepe University, Ankara, Turkey. Electronic address: oakyol@texasheart.org.
authors.forename: Omer
authors.initials: O
authors.lastname: Akyol
authors.affiliation: Vascular and Medicinal Research, Texas Heart Institute, Houston, TX, USA; Medical Biochemist, TUBITAK/BIDEB Scholar, Ankara, Turkey.
authors.forename: Sumeyya
authors.initials: S
authors.lastname: Akyol
authors.affiliation: Vascular and Medicinal Research, Texas Heart Institute, Houston, TX, USA; Lipid Science and Aging Research Center, Kaohsiung Medical University, Kaohsiung, Taiwan; Center for Lipid Biosciences, Kaohsiung Medical University Hospital, Kaohsiung, Taiwan.
authors.forename: Chu-Huang
authors.initials: CH
authors.lastname: Chen
mesh_terms: D000071120:ADAMTS13 Protein / Q000097:blood / Q000378:metabolism*; D002318:Cardiovascular Diseases / Q000097:blood / Q000378:metabolism*; D006402:Hematologic Diseases / Q000097:blood / Q000378:metabolism*; D006801:Humans; D014841:von Willebrand Factor / Q000378:metabolism*
chemical_list: D014841:von Willebrand Factor; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human
keywords: ADAMTS13; Arterial thrombosis; Cardiovascular diseases; Hematological diseases; Thrombotic thrombocytopenic purpura; von Willebrand factor