title: ADAMTS13 in health and disease.
abstract: We raised a set of monoclonal antibodies (mAb) against recombinant human ADAMTS13, constructed the first antigen test fully based on mAbs and compared ADAMTS13 antigen and enzymatic activity levels in a large set of plasma samples collected from different patients and healthy controls. Assessing both ADAMTS13 antigen and activity helps to understand whether or not the protease is fully active in pathological conditions, e.g. liver cirrhosis, inflammatory bowel disease, cardiac surgery, pregnancy and oral contraceptive intake, in the neonatal state and in normal individuals. Our ADAMTS13 antigen assay showed less variability than the collagen binding-based activity assay. Antigen values correlated well with activity in normal individuals, but differed to various degrees in neonates, pregnancies at later maternal age and cardiac surgery. No discrepancies were noted in liver cirrhosis and inflammatory bowel disease, which were both associated with low plasma levels of ADAMTS13. In conclusion, parallel measurement of ADAMTS13 activity and antigen provides a new tool for understanding the behavior of the VWF-cleaving protease in health and disease.
authors.affiliation: Laboratory for Thrombosis Research, Interdisciplinary Research Center, Catholic University of Leuven, Campus Kortrijk, E. Sabbelaan 53, Kortrijk, Belgium.
authors.forename: Hendrik B
authors.initials: HB
authors.lastname: Feys
authors.forename: Hans
authors.initials: H
authors.lastname: Deckmyn
authors.forename: Karen
authors.initials: K
authors.lastname: Vanhoorelbeke
mesh_terms: D051722:ADAM Proteins / Q000097:blood* / Q000172:deficiency / Q000276:immunology / Q000502:physiology; D000071120:ADAMTS13 Protein; D000328:Adult; D000911:Antibodies, Monoclonal / Q000276:immunology; D006348:Cardiac Surgical Procedures; D003276:Contraceptives, Oral; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D015212:Inflammatory Bowel Diseases / Q000201:enzymology; D008103:Liver Cirrhosis / Q000201:enzymology; D008297:Male; D011247:Pregnancy; D011697:Purpura, Thrombotic Thrombocytopenic / Q000201:enzymology; D011994:Recombinant Proteins / Q000276:immunology; D014841:von Willebrand Factor / Q000032:analysis / Q000378:metabolism
chemical_list: D000911:Antibodies, Monoclonal; D003276:Contraceptives, Oral; D011994:Recombinant Proteins; D014841:von Willebrand Factor; D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human