title: Changes in von Willebrand factor-cleaving protease (ADAMTS13) activity after infusion of desmopressin.
abstract: von Willebrand factor-cleaving protease (ADAMTS13) cleaves von Willebrand factor (VWF) and regulates its physiologic function. To investigate the relation between ADAMTS13 activity and VWF, we compared ADAMTS13 activity with the VWF-related parameters VWF antigen (VWF:Ag), VWF collagen-binding activity (VWF:CBA), VWF-propeptide, proVWF, and VWF multimeric composition in 10 healthy volunteers and 3 patients with type 1 von Willebrand disease before and after infusing 0.3 microg/kg desmopressin. The VWF-related parameters in the volunteers increased 60 minutes after start of infusion by 3.7-fold for VWF:Ag, 7.2-fold for propeptide, and 2.2-fold for VWF:CBA. Unusually large VWF multimers and traces of proVWF appeared. The ADAMTS13 activity decreased to about half the initial value. After 24 hours values returned to baseline. Patients with type 1 von Willebrand disease showed similar results. We conclude that the inverse correlation between ADAMTS13 and VWF-related parameters suggests a consumption of ADAMTS13 after the desmopressin-induced release of higher multimers of VWF.
authors.affiliation: Department of Clinical Pharmacology, University of Vienna, Austria.
authors.forename: Rosemarie A
authors.initials: RA
authors.lastname: Reiter
authors.forename: Paul
authors.initials: P
authors.lastname: Knöbl
authors.forename: Katalin
authors.initials: K
authors.lastname: Varadi
authors.forename: Peter L
authors.initials: PL
authors.lastname: Turecek
mesh_terms: D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; D000328:Adult; D016022:Case-Control Studies; D003094:Collagen / Q000378:metabolism; D003894:Deamino Arginine Vasopressin / Q000008:administration & dosage / Q000494:pharmacology*; D019281:Dimerization; D005260:Female; D006801:Humans; D007263:Infusions, Parenteral; D008297:Male; D008666:Metalloendopeptidases / Q000187:drug effects* / Q000378:metabolism; D008875:Middle Aged; D014842:von Willebrand Diseases / Q000188:drug therapy / Q000201:enzymology; D014841:von Willebrand Factor / Q000187:drug effects / Q000378:metabolism
chemical_list: D014841:von Willebrand Factor; D003094:Collagen; D051722:ADAM Proteins; D008666:Metalloendopeptidases; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human; D003894:Deamino Arginine Vasopressin