• 1
    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: 630610.
  • 2
    Tsai HM. Physiologic cleavage of von Willebrand factor by a plasma protease is dependent on its conformation and requires calcium ion. Blood 1996; 87: 423544.
  • 3
    Furlan M, Robles R, Lammle 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: 422334.
  • 4
    Moake JL, Rudy CK, Troll JH, Weinstein MJ, Colannino NM, Azocar J, Seder RH, Hong SL, Deykin D. Unusually large plasma factor VIII:von Willebrand factor multimers in chronic relapsing thrombotic thrombocytopenic purpura. N Engl J Med 1982; 307: 14325.
  • 5
    Moake JL, Rudy CK, Troll JH, Schafer AI, Weinstein MJ, Colannino NM, Hong SL. Therapy of chronic relapsing thrombotic thrombocytopenic purpura with prednisone and azathioprine. Am J Hematol 1985; 20: 739.
  • 6
    Moake J, Chintagumpala M, Turner N, McPherson P, Nolasco L, Steuber C, Santiago-Borrero P, Horowitz M, Pehta J. Solvent/detergent-treated plasma suppresses shear-induced platelet aggregation and prevents episodes of thrombotic thrombocytopenic purpura. Blood 1994; 84: 4907.
  • 7
    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: 27305.
  • 8
    Loof AH, Van Vliet HH, Kappers-Klunne MC. Low activity of von Willebrand factor-cleaving protease is not restricted to patients suffering from thrombotic thrombocytopenic purpura. Br J Haematol 2001; 112: 10878.
  • 9
    Bianchi V, Robles R, Alberio L, Furlan M, Lammle B. Von Willebrand factor-cleaving protease (ADAMTS13) in thrombocytopenic disorders: a severely deficient activity is specific for thrombotic thrombocytopenic purpura. Blood 2002; 100: 7103.
  • 10
    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: 157884.
  • 11
    Tsai HM, Lian EC. Antibodies to von Willebrand factor-cleaving protease in acute thrombotic thrombocytopenic purpura. N Engl J Med 1998; 339: 158594.
  • 12
    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: 13869.
  • 13
    He S, Cao H, Magnusson CG, Eriksson-Berg M, Mehrkash M, Schenck-Gustafsson K, Blomback M. Are increased levels of von Willebrand factor in chronic coronary heart disease caused by decrease in von Willebrand factor cleaving protease activity? A study by an immunoassay with antibody against intact bond 842Tyr-843Met of the von Willebrand factor protein. Thromb Res 2001; 103: 2418.
  • 14
    Obert B, Tout H, Veyradier A, Fressinaud E, Meyer D, Girma JP. Estimation of the von Willebrand factor-cleaving protease in plasma using monoclonal antibodies to vWF. Thromb Haemost 1999; 82: 13825.
  • 15
    Kirzek DM, Rick ME. Clinical application of a rapid method using agarose gel electrophoresis and Western blotting to evaluate von Willebrand factor protease activity. Electrophoresis 2001; 22: 9469.
  • 16
    Aronson DL, Krizek DM, Rick ME. A rapid assay for the vWF protease. Thromb Haemost 2001; 85: 1845.
  • 17
    Bohm M, Vigh T, Scharrer I. Evaluation and clinical application of a new method for measuring activity of von Willebrand factor-cleaving metalloprotease (ADAMTS13). Ann Hematol 2002; 81: 4305.
  • 18
    Shenkman B, Inbal A, Tamarin I, Lubetsky A, Savion N, Varon D. Diagnosis of thrombotic thrombocytopenic purpura based on modulation by patient plasma of normal platelet adhesion under flow condition. Br J Haematol 2003; 120: 597604.
  • 19
    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: 40339.
  • 20
    Cruz MA, Whitelock JL, Dong JF. Evaluation of ADAMTS-13 activity in plasma using recombinant von Willebrand factor A2 domain polypeptide as substrate. Thromb Haemost 2003; 90: 12349.
  • 21
    Dong JF, Moake JL, Bernardo A, Fujikawa K, Ball C, Nolasco L, Lopez JA, Cruz MA. Adamts-13 metalloprotease interacts with the endothelial cell-derived ultra-large von Willebrand factor. J Biol Chem 2003; 278: 296339.
  • 22
    Lankhof H, Damas C, Schiphorst ME, Ijsseldijk MJ, Bracke M, Furlan M, Tsai HM, De Groot PG, Sixma JJ, Vink T. von Willebrand factor without the A2 domain is resistant to proteolysis. Thromb Haemost 1997; 77: 100813.
  • 23
    Zimmerman TS, Dent JA, Ruggeri ZM, Nannini LH. Subunit composition of plasma von Willebrand factor. Cleavage is present in normal individuals, increased in IIA and IIB von Willebrand disease, but minimal in variants with aberrant structure of individual oligomers (types IIC, IID, and IIE). J Clin Invest 1986; 77: 94751.
  • 24
    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: 16626.
  • 25
    Zheng X, Chung D, Takayama TK, Majerus EM, Sadler JE, Fujikawa K. Structure of von Willebrand factor-cleaving protease (ADAMTS13), a metalloprotease involved in thrombotic thrombocytopenic purpura. J Biol Chem 2001; 276: 4105963.
  • 26
    Gerritsen HE, Robles R, Lammle B, Furlan M. Partial amino acid sequence of purified von Willebrand factor-cleaving protease. Blood 2001; 98: 165461.
  • 27
    Moake JL. Thrombotic microangiopathies. N Engl J Med 2002; 347: 589600.
  • 28
    Tsai HM. Deficiency of ADAMTS13 and thrombotic thrombocytopenic purpura. Blood 2002; 100: 383940.
  • 29
    Tsai HM, Sussman II, Nagel RL. Shear stress enhances the proteolysis of von Willebrand factor in normal plasma. Blood 1994; 83: 21719.