Relation between ADAMTS13 activity and ADAMTS13 antigen levels in healthy donors and patients with thrombotic microangiopathies (TMA)

Thrombosis and Haemostasis

Volumn 95, Issue 2, 2006, Pages 212-220

  Rieger, Manfred ^a   Ferrari, Silvia ^a   Kremer Hovinga, Johanna A ^b   Konetschny, Christian ^a   Herzog, Andrea ^a   Koller, Letizia ^a   Weber, Alfred ^a   Remuzzi, Guiseppe ^c   Dockal, Michael ^a   Plaimauer, Barbara ^a   Scheiflinger, Friedrich ^a,d  

^a BAXTER BIOSCIENCE   (Austria)

^b UNIVERSITY OF BERN   (Switzerland)

^c MARIO NEGRI INSTITUTE FOR PHARMACOLOGICAL RESEARCH   (Italy)

^d Azienda Ospedaliera ^*  (Austria)

Author keywords

ADAMTS13; ADAMTS13 antigen; ELISA; HUS; TTP

Indexed keywords

AUTOANTIBODY; IMMUNOGLOBULIN G; POLYCLONAL ANTIBODY; VON WILLEBRAND FACTOR; VON WILLEBRAND FACTOR CLEAVING PROTEINASE;

ANTIGEN ANTIBODY COMPLEX; ANTIGEN DETECTION; ARTICLE; BLOOD ANALYSIS; BLOOD DONOR; COHORT ANALYSIS; CONCENTRATION (PARAMETERS); CONTROLLED STUDY; DIAGNOSTIC VALUE; ENZYME ACTIVITY; ENZYME BINDING; ENZYME DEFICIENCY; ENZYME LINKED IMMUNOSORBENT ASSAY; GENE MUTATION; HEMOLYTIC UREMIC SYNDROME; HUMAN; HUMAN CELL; HUMAN TISSUE; MAJOR CLINICAL STUDY; PATHOGENESIS; PLASMAPHERESIS; PREDICTION; PRIORITY JOURNAL; RABBIT; REMISSION; THROMBOTIC THROMBOCYTOPENIC PURPURA;

ADAM PROTEINS; ANIMALS; ANTIGEN-ANTIBODY COMPLEX; ANTIGENS; BLOOD DONORS; CASE-CONTROL STUDIES; ENZYME-LINKED IMMUNOSORBENT ASSAY; HUMANS; PURPURA, THROMBOTIC THROMBOCYTOPENIC; RABBITS;

EID: 33645092626     PISSN: 03406245     EISSN: None     Source Type: Journal    
DOI: 10.1160/TH05-08-0550     Document Type: Article

References (35)

1. Moake JL, Turner NA, Stathopoulos NA et al.. Involvement of large plasma von Willebrand factor (vWF) multimers and unusually large vWF forms derived from endothelial cells in shear stress-induced platelet aggregation. J Clin Invest 1986; 78: 1456-61.
2. Furlan M, Robles R, Lämmle B. Partial purification and characterization of a protease from human plasma cleaving von Willebrand factor to fragments produced by in vivo proteolysis. Blood 1996; 87: 4223-34.
3. Tsai HM. Physiologic cleavage of von Willebrand factor by a plasma protease is dependent on its conformation and requires calcium ion. Blood 1996; 87: 4235-44.
4. Gerritsen HE, Robles R, Lämmle B et al.. Partial amino acid sequence of purified von Willebrand factor-cleaving protease. Blood 2001; 98: 1654-61.
5. Fujikawa K, Suzuki H, McMullen B et al.. Purification of human von Willebrand factor-cleaving protease and its identification as a new member of the metalloproteinase family. Blood 2001; 98: 1662-6.
6. Zheng X, Chung D, Takayama TK et al.. Structure of von Willebrand factor-cleaving protease (ADAMTS13), a metalloprotease involved in thrombotic thrombocytopenic purpura. J Biol Chem 2001; 276: 41059-63.
7. Soejima K, Mimura N, Hirashima M et al.. A novel human metalloprotease synthesized in the liver and secreted into the blood: possibly, the von Willebrand factor-cleaving protease? J Biochem 2001; 130: 475-80.
8. Levy GG, Nichols WC, Lian EC et al.. Mutations in a member of the ADAMTS gene family cause thrombotic thrombocytopenic purpura. Nature 2001; 413: 488-94.
9. Porter S, Clark IM, Kevorkian L et al.. The ADAMTS metalloproteinases. Biochem J 2005; 386: 15-27.
10. Moake JL, Rudy CK, Troll JH et al.. Unusually large plasma factor VIII: von Willebrand factor multimers in chronic relapsing thrombotic thrombocytopenic putpura. N Engl J Med 1982; 307: 1432-5.
11. Furlan M, Robles R, Lämmle B. Partial purification and characterization of a protease from human plasma cleaving von Willebrand factor to fragments produced by in vivo proteolysis. Blood 1996; 87: 4223-34.
12. Furlan M, Robles R, Solenthaler M et al.. Deficient activity of von Willebrand factor-cleaving protease in chronic relapsing thrombotic thrombocytopenic purpura. Blood 1997; 89: 3097-103.
13. Furlan M, Robles R, Galbusera M et al.. von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura and the hemolytic-uremic syndrome. N Engl J Med 1998; 339: 1578-84.
14. Tsai HM. Physiologic cleavage of von Willebrand factor by a plasma protease is dependent on its conformation and requires calcium ion. Blood 1996; 87: 4235-44.
15. Tsai HM, Lian EC. Antibodies to von Willebrand factor-cleaving protease in acute thrombotic thrombocytopenic purpura. N Engl J Med 1998; 339: 1585-94.
16. Remuzzi G, Galbusera M, Noris M et al.. Italian Registry of Recurrent and Familial HUS/TTP. Thrombotic thrombocytopenic purpura/hemolytic uremic syndrome: von Willebrand factor cleaving protease (ADAMTS13) is deficient in recurrent and familial thrombotic thrombocytopenic purpura and hemolytic uremic syndrome. Blood 2002; 100: 778-85.
17. Remuzzi G. Is ADAMTS-13 deficiency specific for thrombotic thrombocytopenic purpura? No. J Thromb Haemost 2003; 1: 632-4.
18. Moake JL. Thrombotic microangiopathies. N Engl J Med 2002; 347: 589-600.
19. Manuelian T, Hellwage J, Meri S et al.. Mutations in factor H reduce binding affinity to C3b and heparin and surface attachment to endothelial cells in hemolytic uremic syndrome. J Clin Invest 2003; 111: 1181-90.
20. Caprioli J, Castelletti F, Bucchioni S et al.. International Registry of Recurrent and Familial HUS/TTP. Complement factor H mutations and gene polymorphisms in haemolytic uraemic syndrome: the C-257T, the A2089G and the G2881T polymorphisms are strongly associated with the disease. Hum Mol Genet 2003; 12: 3385-95.
21. Noris M, Brioschi S, Caprioli J et al.. International Registry of Recurrent and Familial HUS/TTP. Familial haemolytic uraemic syndrome and an MCP mutation. Lancet 2003; 362: 1542-7.
22. Raife T, Atkinson B, Montgomery R et al.. Severe deficiency of VWF-cleaving protease (ADAMTS13) activity defines a distinct population of Thrombotic microangiopathy patients. Transfusion 2004; 44:146-50.
23. Veyradier A, Girma JP. Assays of ADAMTS-13 activity. Semin Hematol 2004; 41: 41-7.
24. Gerritsen HE, Turecek PL, Schwarz HP et al.. Assay of von Willebrand factor (VWF)-cleaving protease based on decreased collagen binding affinity of degraded vWF: a tool for the diagnosis of thrombotic thrombocytopenic purpura (TTP). Thromb Haemost 1999; 82: 1386-9.
25. Studt JD, Böhm M, Budde U et al.. Measurement of von Willebrand factor-cleaving protease (ADAMTS-13) activity in plasma: a multicenter comparison of different assay methods. J Thromb Haemost 2003; 1: 1882-7.
26. Böhm M, Vigh T, Scharrer I. Evaluation and clinical application of a new method for measuring activity of von Willebrand factor-cleaving metalloprotease (ADAMTS13). Ann Hemato 2002; 81: 430-5.
27. Scheiflinger F, Knobl P, Tratner B et al.. Nonneutralizing IgM and IgG antibodies to von Willebrand factor-cleaving protease (ADAMTS-13) in a patient with thrombotic thrombocytopenic purpura. Blood 2003; 102: 3241-3.
28. Rieger M, Mannucci PM, Hovinga JA et al.. ADAMTS13 autoantibodies in patients with thrombotic microangiopathies and other immunomediated diseases. Blood 2005; 106: 1262-7.
29. Plaimauer B, Zimmermann K, Volkel D et al.. Cloning, expression, and functional characterization of the von Willebrand factor-cleaving protease (ADAMTS13). Blood 2002; 100: 3626-32.
30. Food and Drug Administration and Center for Veterinary Medicine. Guidance for industry. Validation of analytical procedures: Methodology. VICH GL2, October 1998.
31. Findlay JW, Smith WC, Lee JW et al.. Validation of immunoassays for bioanalysis: a pharmaceutical industry perspective. J Pharm Biomed Anal 2000; 21: 1249-73.
32. Klaus C, Plaimauer B, Studt JD et al.. Epitope mapping of ADAMTS13 autoantibodies in acquired thrombotic thrombocytopenic purpura. Blood 2004; 103: 4514-9.
33. Crawley J, Lam J, Rance J et al.. Proteolytic inactivation of ADAMTS13 by thrombin and plasmin. Blood 2005; 105: 1085-93.
34. Zheng X, Nishio K, Majerus EM et al.. Cleavage of von Willebrand factor requires the spacer domain of the metalloprotease ADAMTS13. J Biol Chem 2003; 278: 30136-41.
35. Soejima K, Matsumoto M, Kokame K et al.. ADAMTS13 cysteine-rich/spacer domains are functionally essential for von Willebrand factor cleavage. Blood 2003; 102: 3232-7.