• 1
    Wick TM, Moake JL, Udden MM, Eskin SG, Sears DA, McIntire LV. Unusually large von Willebrand factor multimers increase adhesion of sickle erythrocytes to human endothelial cells under controlled flow. J Clin Invest 1987; 80: 90510.
  • 2
    Tsai HM, Sussman II, Nagel RL, Kaul DK. Desmopressin induces adhesion of normal human erythrocytes to the endothelial surface of a perfused microvascular preparation. Blood 1990; 75: 2615.
  • 3
    Kaul DK, Nagel RL, Chen D, Tsai HM. Sickle erythrocyte–endothelial interactions in microcirculation: the role of von Willebrand factor and implications for vasoocclusion. Blood 1993; 81: 242938.
  • 4
    Tsai HM, Nagel RL, Hatcher VB, Sussman II. Multimeric composition of endothelial cell-derived von Willebrand factor. Blood 1989; 73: 20746.
  • 5
    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.
  • 6
    Tsai HM, Nagel RL, Hatcher VB, Sussman II. Endothelial cell-derived high molecular weight von Willebrand factor is converted into the plasma multimer pattern by granulocyte proteases. Biochem Biophys Res Commun 1989; 158: 9805.
  • 7
    Phillips MD, Vu C, Nolasco L, Moake JL. Granulocyte proteases do not process endothelial cell-derived unusually large von Willebrand factor multimers to plasma vWF in vivo. Am J Hematol 1991; 37: 803.
  • 8
    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.
  • 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: 630610.
  • 10
    Wagner DD, Lawrence SO, Ohlsson-Wilhelm BM, Fay PJ, Marder VJ. Topology and order of formation of interchain disulfide bonds in von Willebrand factor. Blood 1987; 69: 2732.
  • 11
    Tsai HM, Sussman II, Nagel RL. Shear stress enhances the proteolysis of von Willebrand factor in normal plasma. Blood 1994; 83: 21719.
  • 12
    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.
  • 13
    Siedlecki CA, Lestini BJ, Kottke-Marchant KK, Eppell SJ, Wilson DL, Marchant RE. Shear-dependent changes in the three-dimensional structure of human von Willebrand factor. Blood 1996; 88: 293950.
  • 14
    Gralnick HR, Williams SB, McKeown LP, Maisonneuve P, Jenneau C, Sultan Y, Rick ME. In vitro correction of the abnormal multimeric structure of von Willebrand factor in type IIa von Willebrand's disease. Proc Natl Acad Sci USA 1985; 82: 596872.
  • 15
    Batlle J, Lopez Fernandez MF, Campos M, Justica B, Berges C, Navarro JL, Diaz Cremades JM, Kasper CK, Dent JA, Ruggeri ZM. The heterogeneity of type IIA von Willebrand's disease: studies with protease inhibitors. Blood 1986; 68: 120712.
  • 16
    Lyons SE, Bruck ME, Bowie EJ, Ginsburg D. Impaired intracellular transport produced by a subset of type IIA von Willebrand disease mutations. J Biol Chem 1992; 267: 442430.
  • 17
    Tsai HM, Sussman II, Ginsburg D, Lankhof H, Sixma JJ, Nagel RL. Proteolytic cleavage of recombinant type 2A von Willebrand factor mutants R834W and R834Q: inhibition by doxycycline and by monoclonal antibody VP-1. Blood 1997; 89: 195462.
  • 18
    Tsai HM. Von Willebrand factor, ADAMTS13, and thrombotic thrombocytopenic purpura. J Mol Med 2002; 80: 63947.DOI: 10.1007/s00109-002-0369-8
  • 19
    Bukowski RM. Thrombotic thrombocytopenic purpura. A review. Prog Hemost Thromb 1982; 6: 287337.
  • 20
    Bukowski RM, King JW, Hewlett JS. Plasmapheresis in the treatment of thrombotic thrombocytopenic purpura. Blood 1977; 50: 4137.
  • 21
    Byrnes JJ, Khurana M. Treatment of thrombotic thrombocytopenic purpura with plasma. N Engl J Med 1977; 297: 13869.
  • 22
    Asada Y, Sumiyoshi A, Hayashi T, Suzumiya J, Kaketani K. Immunohistochemistry of vascular lesion in thrombotic thrombocytopenic pupura, with special reference to factor VIII related antigen. Thromb Res 1985; 38: 46779.
  • 23
    Tsai HM, Lian EC. Antibodies to von Willebrand factor-cleaving protease in acute thrombotic thrombocytopenic purpura. N Engl J Med 1998; 339: 158594.DOI: 10.1056/NEJM199811263392203
  • 24
    Tsai HM, Chandler WL, Sarode R, Hoffman R, Jelacic S, Habeeb RL, Watkins SL, Wong CS, Williams GD, Tarr PI. Von Willebrand Factor and Von Willebrand Factor-Cleaving Metalloprotease Activity in Escherichia coli O157: H7–Associated Hemolytic Uremic Syndrome. Pediatr Res 2001; 49: 6539.
  • 25
    Tsai HM, Rice L, Sarode R, Chow TW, Moake JL. Antibody inhibitors to von Willebrand factor metalloproteinase and increased binding of von Willebrand factor to platelets in ticlopidine- associated thrombotic thrombocytopenic purpura. Ann Intern Med 2000; 132: 7949.
  • 26
    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: 48894.DOI: 10.1038/35097008
  • 27
    Upshaw JD Jr. Congenital deficiency of a factor in normal plasma that reverses microangiopathic hemolysis and thrombocytopenia. N Engl J Med 1978; 298: 13502.
  • 28
    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: 422334.
  • 29
    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.DOI: 10.1056/NEJM199811263392202
  • 30
    Gerritsen HE, Robles R, Lammle B, Furlan M. Partial amino acid sequence of purified von Willebrand factor-cleaving protease. Blood 2001; 98: 165461.
  • 31
    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.
  • 32
    Soejima K, Mimura N, Hirashima M, Maeda H, Hamamoto T, Nakagaki T, Nozaki C. A novel human metalloprotease synthesized in the liver and secreted into the blood: possibly, the von Willebrand factor-cleaving protease? J Biochem (Tokyo) 2001; 130: 47580.