The activity of the von Willebrand factor cleaving protease ADAMTS-13 in newborn infants

Journal of Thrombosis and Haemostasis

Volumn 2, Issue 2, 2004, Pages 228-233

  Schmugge, M ^a   Dunn, M S ^b,e   Amankwah, K S ^b,e   Blanchette, V S ^c,e   Freedman, J ^d,f   Rand, Margaret L ^c,e  

^a UNIVERSITY CHILDREN S HOSPITAL   (Switzerland)

^b SUNNYBROOK RESEARCH INSTITUTE   (Canada)

^c HOSPITAL FOR SICK CHILDREN   (Canada)

^d ST MICHAEL'S HOSPITAL   (Canada)

^e UNIVERSITY OF TORONTO   (Canada)

^f UNIVERSITY OF TORONTO   (Canada)

Author keywords

ADAMTS 13; Neonates; von Willebrand factor; VWF cleaving protease

Indexed keywords

COLLAGEN; POLYMER; VON WILLEBRAND FACTOR; VON WILLEBRAND FACTOR CLEAVING PROTEINASE; ADAM PROTEIN; ADAMTS13 PROTEIN, HUMAN; METALLOPROTEINASE;

ADULT; ARTICLE; BLOOD CLOTTING; BLOOD SAMPLING; CHILD; CLINICAL ARTICLE; ENZYME ACTIVATION; ENZYME RELEASE; FEMALE; HUMAN; INFANT; MALE; MOLECULAR WEIGHT; NEWBORN; PRIORITY JOURNAL; PROTEIN BINDING; PROTEIN LOCALIZATION; PROTEIN PROTEIN INTERACTION; PROTEIN SYNTHESIS; THROMBOTIC THROMBOCYTOPENIC PURPURA; UMBILICAL CORD BLOOD; AGE; BLOOD; COMPARATIVE STUDY; DIMERIZATION; FETUS BLOOD; HEMOLYTIC UREMIC SYNDROME; METABOLISM; VEIN;

ADAM PROTEINS; ADULT; AGE FACTORS; CHILD; DIMERIZATION; FEMALE; FETAL BLOOD; HEMOLYTIC-UREMIC SYNDROME; HUMANS; INFANT, NEWBORN; MALE; METALLOENDOPEPTIDASES; PURPURA, THROMBOTIC THROMBOCYTOPENIC; VEINS; VON WILLEBRAND FACTOR;

EID: 4644327577     PISSN: 15387933     EISSN: None     Source Type: Journal    
DOI: 10.1046/j.1538-7933.2003.00575.x     Document Type: Article

References (46)

1. Sadler JE. Biochemistry and genetics of von Willebrand factor. Ann Rev Biochem 1998; 67: 395-424.
2. Ruggeri ZM. Structure and function of von Willebrand factor. Thromb Haemost 1999; 82: 576-84.
3. Keularts IMLW, Hamulyak K, Hemker HC, Beguin S. The effect of DDAVP infusion on thrombin generation in platelet-rich plasma of von Willebrand Type 1 and mild haemophilia A patients. Thromb Haemost 2000; 84: 638-42.
4. Gerritsen HE, Robles R, Lammle B, Furlan M. Partial amino acid sequence of purified von Willebrand factor-cleaving protease. Blood 2001; 98: 1654-61.
5. Fujikawa K, Suzuki H, McMullen B, Chung D. 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. Levy GG, Nichols WC, Lian EC, Foroud T, McClintick JN, McGee BM, Yang AY, Siemieniak DR, Stark KR, Gruppo R, Sarode R, Shurin SB, Chandrasekaran V, Stabler SP, Sabio H, Bouhassira EE, Upshaw JD Jr, Ginsburg D, Tsai HM. Mutations in a member of the ADAMTS gene family cause thrombotic thrombocytopenic purpura. Nature 2001; 413: 488-94.
7. Plaimauer B, Zimmermann K, Volkel D, Antoine G, Kerschbaumer R, Jenab P, Furlan M, Gerritsen H, Lammle B, Schwarz HP, Scheiflinger F. Cloning, expression, and functional characterization of the von Willebrand factor-cleaving protease (ADAMTS13). Blood 2002; 100 3626-32.
8. Tsai HM. Deficiency of ADAMTS13 causes thrombotic thrombocytopenic purpura. Arterioscler Thromb Vasc Biol 2003; 23 388-96.
9. Dent JA, Berkowitz SD, Ware J, Kasper CK, Ruggeri ZM. Identification of a cleavage site directing the immunochemical detection of molecular abnormalities in type IIA von Willebrand factor. Proc Natl Acad Sci USA 1990; 87: 6306-10.
10. Furlan M, Robles R, Lamie 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.
11. 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.
12. Moake JL, Turner NA, Stathopoulos NA, Nolasco LH, Hellums JD. 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.
13. Tsai HM, Sussman II, Nagel RL. Shear stress enhances the proteolysis of von Willebrand factor in normal plasma. Blood 1994; 83: 2171-9.
14. McCrary JK, Nolasco LH, Hellums JD, Kroll MH, Turner NA, Moake JL. Direct demonstration of radiolabeled von Willebrand factor binding to platelet glycoprotein Ib and IIb-IIIa in the presence of shear stress. Ann Biomed Eng 1995; 23: 787-93.
15. Moake JL. Thrombotic thrombocytopenic purpura. Thromb Haemost 1995; 74: 240-5.
16. Furlan M, Robles R, Galbusera M, Remuzzi G, Kyrle PA, Brenner B, Krause M, Scharrer I, Aumann V, Mittler U, Solenthaler M, Lammle B. von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura and the hemolytic-uremic syndrome. N Engl J Med 1998; 339: 1578-84.
17. Tsai HM, Lian EC. Antibodies to von Willebrand factor-cleaving protease in acute thrombotic thrombocytopenic purpura. N Engl J Med 1998; 339: 1585-94.
18. Schneppenheim R, Budde U, Oyen F, Angerhaus D, Aumann V, Drewke E, Hassenpflug W, Häberle J, Kentouche K, Kohne E, Kurnik K, Mueller-Wiefel D, Obser T, Santer R, Sykora KW. von Willebrand factor cleaving protease and ADAMTS13 mutations in childhood TTP. Blood 2003; 101: 1845-50.
19. Andrew M. Developmental hemostasis: relevance to thromboembolic complications in pediatric patients. In: Andrew M, Monagle PT, Brooker L, eds. Thromboembolic Complications During Infancy and Childhood. Hamilton, B.C.: Decker, 2000: 5-46.
20. deVeber G. Canadian pediatric ischemic stroke registry: analysis of children with arterial ischemic stroke. Ann Neurol 2000; 48: 514 (Abstract).
21. Schmidt B, Andrew M. Neonatal thrombosis: report of a prospective Canadian and international registry. Pediatrics 1996; 96: 939-43.
22. Nowak-Göttl U, von Kries R, Göbel U. Neonatal symptomatic thromboembolism in Germany: two year survey. Arch Dis Child 1997; 76: F163-F167.
23. Katz JA, Moake JL, McPherson PD, Weinstein MJ, Moise KJ, Carpenter RJ, Sala DJ. Relationship between human development and disappearance of unusually large von Willebrand factor multimers from plasma. Blood 1989; 73: 1851-8.
24. Weinstein J, Blanchard R, Moake J, Vosburgh E, Moise K. Fetal and neonatal von Willebrand factor is unusually large and similar to the VWF in patients with TTP. Br J Haematol 1989; 72: 68-72.
25. Shenkman B, Linder N, Savion N, Tamarin I, Dardik R, Kennet G, German B, Varon D. Increased neonatal platelet deposition on sub-endothelium under flow conditions. The role of plasma von Willebrand factor. Pediatr Res 1999; 45: 270-5.
26. Mannucci PM, Canciani MT, Forza I, Lussana F, Lattuada A, Rossi E. Changes in health and disease of the metalloprotease that cleaves von Willebrand factor. Blood 2001; 98: 2730-5.
27. Takahashi Y, Kawaguchi C, Hanesaka Y, Fujimura Y, Yoshioka A. Plasma von Willebrand factor-cleaving protease is low in the newborns. Thromb Haemost 2001; 86(Suppl.): 285 (Abstract).
28. Tsai HM, Sarode R, Downes KA. Ultralarge von Willebrand factor multimers and normal ADAMTS13 activity in the umbilical cord blood. Thromb Res 2003; 108: 121-5.
29. Aronson DL, Krizek DM, Rick ME. A rapid assay for the VWF protease. Thromb Haemost 2001; 85: 184-5.
30. Ledford MR, Kent JW, Civantos F. A comparative study of three methods for the visualization of von Willebrand factor multimers. Thromb Haemost 1990; 64: 569-75.
31. Aihara M, Sawada Y, Ueno K, Morimoto S, Yoshida Y, de Serres M, Cooper HA, Wagner RH. Visualization of von Willebrand factor multimers by immunoenzymatic stain using avidin-biotin peroxidase complex. Thromb Haemost 1986; 55: 263-7.
32. Schmugge M, Rand ML, Bang KWA, Mody M, Dunn MS, Amankwah KS, Blanchette VS, Freedman J. Relationship of von Willebrand factor binding to activated platelets from healthy neonates and adults. Pediatr Res 2003; 54: 474-9.
33. Veyradier A, Obert B, Houllier A, Meyer D, Girma JP. Specific von Willebrand factor-cleaving protease in thrombotic microangiopathies: a study of 111 cases. Blood 2001; 98: 1765-72.
34. Park YD, Yoshioka A, Kawa K, Ishizashi H, Yagi H, Yamamoto Y, Matsumoto M, Fujimura Y. Impaired activity of plasma von Willebrand factor-cleaving protease may predict the occurrence of hepatic veno-occlusive disease after stem cell transplantation. Bone Marrow Transplant 2002; 29: 789-94.
35. Sahud MA, Claster S, Liu L, Ero M, Harris K, Furlan M. von Willebrand factor-cleaving protease inhibitor in a patient with human immunodeficiency syndrome-associated thrombotic thrombocytopenic purpura. Br J Haematol 2002; 116: 909-11.
36. Yagi H, Konno M, Kinoshita S, Matsumoto M, Ishizashi H, Matsui T, Titani K, Fujimura Y. Plasma of patients with Upshaw-Schulman syndrome, a congenital deficiency of von Willebrand factor-cleaving protease activity, enhances the aggregation of normal platelets under high shear stress. Br J Haematol 2001; 115: 991-7.
37. Moore JC, Hayward CP, Warkentin TE, Kelton JG. Decreased von Willebrand factor protease activity associated with thrombocytopenic disorders. Blood 2001; 98: 1842-6.
38. Gungor T, Furlan M, Lammle B, Kuhn F, Seger RA. Acquired deficiency of von Willebrand factor-cleaving protease in a patient suffering from acute systemic lupus erythematosus. Rheumatology 2001; 40: 940-2 (letter).
39. Bianchi V, Robles R, Alberio L, Furlan M, Lämmle B. Von Willebrand factor-cleaving protease (ADAMTS13) in thrombocytopenic disorders: severely deficient activity is specific for thrombotic thrombocytopenic purpura. Blood 2002; 100: 710-2.
40. Gerritsen HE, Turecek PL, Schwarz HP, Lammle B, Furlan M. 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.
41. Aronson DL, Krizek DM, Rick ME. Partial purification and characterization of the von Willebrand factor protease. Thromb Haemost 2001; 86 (Suppl. ): 1076 (Abstract).
42. Dong JF, Moake JL, Nolasco L, Bernardo A, Arceneaux W, Shrimpton CN, Schade AJ, McIntire LV, Fujikawa K, Lopez JA. ADAMTS-13 rapidly cleaves newly secreted ultralarge von Willebrand factor multimers on the endothelial surface under flowing conditions. Blood 2002; 100: 4033-9.
43. Wagner DD. The Weibel-Palade body: the storage granule for von Willebrand factor and P-selectin. Thromb Haemost 1993; 70 105-10.
44. Parker RI, Gralnick HR. Identification of platelet glycoprotein IIb/IIIa as the major binding site for released platelet-von Willebrand factor. Blood 1986; 68: 732-6.
45. Gralnick HR, Williams SB, McKeown LP, Magruder L, Hansmann K, Vail M, Parker RI. Platelet von Willebrand factor. Mayo Clin Proc 1991; 66: 634-40.
46. Shankaran H, Alexandridis P, Neelamegham S. Aspects of hydrodynamic shear regulating shear-induced platelet activation and self association of von Willebrand factor in suspension. Blood 2003; 101: 2637-45.