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Thromb Haemost 1997; 78(01): 571-576
DOI: 10.1055/s-0038-1657591
New treatments of von Wiltebrand disease
Schattauer GmbH Stuttgart

Recombinant von Willebrand Factor

Hans Peter Schwarz
1   Immuno AG, Vienna, Austria
,
Peter L Turecek
1   Immuno AG, Vienna, Austria
,
Ludwig Pichler
1   Immuno AG, Vienna, Austria
,
Artur Mitterer
1   Immuno AG, Vienna, Austria
,
Wolfgang Mundt
1   Immuno AG, Vienna, Austria
,
Friedrich Dorner
1   Immuno AG, Vienna, Austria
,
Jacqueline Roussi
2   Hôpital Raymond-Pointcaré, Garches
,
Ludovic Drouet
3   Hôpital Lariboisière, Paris, France
› Author Affiliations
Further Information

Publication History

Publication Date:
12 July 2018 (online)

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  • References

  • 1 Sadler JE. A revised classification of von Willebrand disease. Thromb Haemost 1994; 71: 520-525
    Article in Thieme ConnectPubMedGoogle Scholar
  • 2 Ginsburg D, Sadler JE. Von Willebrand disease: a database of point mutations, insertions, and deletions. Thromb Haemost 1993; 69: 177
    Article in Thieme ConnectPubMedGoogle Scholar
  • 3 Sadler JE, Davie EW, Hemophilia A, hemophilia B. von Willebrand disease. In: Stamatoyannopoulos G, Nienhuis AW, Majerus PW, Varmus H. (eds). The Molecular Basis of Blood Diseases, 2nd ed. Philadelphia: Saunders; 1995: 657-700
    Google Scholar
  • 4 Montgomery RR, Coller BS. Von Willebrand disease. In: Colman RW, Hirsh J, Marder VJ, Salzman EW. (eds). Hemostasis and Thrombosis: Basic Principles and Clinical Practice, 3rd ed. Philadelphia: Lippincott; 1994: 134-168
    Google Scholar
  • 5 Weiss HJ, Sussman II, Hoyer LW. Stabilization of factor VIII in plasma by the von Willebrand factor. J Clin Invest 1977; 60: 390-404
    CrossrefPubMedGoogle Scholar
  • 6 Koppelman SJ, van Hoeij M, Lankhof H, Schiphorst ME, Damas C, Vlot AJ, Wise R, Bouma BN, Sixma JJ. Requirements of von Willebrand factor to protect factor VIII from inactivation by activated protein C. Blood 1996; 87: 2292-2300
    PubMedGoogle Scholar
  • 7 Ruggeri ZM, Ware J. Von Willebrand factor. FASEB J 1993; 7: 308-316
    CrossrefPubMedGoogle Scholar
  • 8 Lethagen S. Pathogenesis, clinical picture and treatment of von Willebrand’s disease. Ann Med 1995; 27: 641-651
    CrossrefPubMedGoogle Scholar
  • 9 Paper R. Women with bleeding disorders. Presented at the 48th Annual Meeting of the National Hemophilia Foundation. 1996. San Diego; CA:
    Google Scholar
  • 10 Scott JP, Montgomery RR. Therapy of von Willebrand disease. Semin Thromb Hemost 1993; 19: 37-47
    Article in Thieme ConnectPubMedGoogle Scholar
  • 11 Fischer BE, Schlokat U, Mitterer A, Reiter M, Mundt W, Turecek PL, Schwarz HP, Domer F. Structural analysis of recombinant von Willebrand factor produced at industrial scale fermentation of transformed CHO cells co-expressing recombinent furin. FEBS Lett 1995; 375: 259-262
    CrossrefPubMedGoogle Scholar
  • 12 Schlokat U, Fischer B, Mitterer A, Falkner F, Reiter M, Grillberger L, Mundt W, Preininger A, Mohr G, Siekmann J, Turecek P, Schwarz HP, Domer F. Herstellung und Charakterisierung von rekombinantem Willebrand-Faktor. In: Scharrer I, Schramm W. (eds). 26. Hamophilie-Symposion Hamburg 1995. Berlin; Springer Verlag: 1997: 147-158
    Google Scholar
  • 13 Furlan M, Robles R, Affolter D, Meyer D, Baillod P. Triplet structure of von Willebrand factor reflects proteolytic degradation of high molecular weight multimers. Proc Natl Acad Sci USA 1993; 90: 7503-7507
    CrossrefPubMedGoogle Scholar
  • 14 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: 4223-4234
    CrossrefPubMedGoogle Scholar
  • 15 Tsai HM. Physiologic cleavage of von Willebrand factor by a plasma protease is dependent on its conformation and requires calcium ion. Blood 1996; 87: 4235-4244
    PubMedGoogle Scholar
  • 16 Turecek PL, Furlan M, Lämmle B, Richter G, Gritsch H, Siekmann J, Schwarz HP. Cleavage of recombinant von Willebrand factor (vWF) by a vWF-depolymerizing protease. Blood. 1996. 88. 326a (abst)
    Google Scholar
  • 17 Stoddart JH, Andersen J, Lynch DC. Clearance of normal and type 2A von Willebrand factor in the rat. Blood 1996; 88: 1692-1699
    CrossrefPubMedGoogle Scholar
  • 18 Sakariassen KS, Aarts PAMM, DeGroot PG, Houdijk WPM, Sixma JJ. A perfusion chamber developed to investigate platelet interaction in flowing blood with human vessel wall cells, their extracellular matrix and purified components. J Lab Clin Med 1983; 102: 522
    PubMedGoogle Scholar
  • 19 Fretto LJ, Fowler WE, McCaslin DR, Erickson HP, McKee PA. Substructure of human von Willebrand factor. J Biol Chem 1986; 261: 15679-15689
    PubMedGoogle Scholar
  • 20 Ohmori K, Fretto LJ, Harrison RL, Switzer MEP, Erickson HP, McKee PA. Electro microscopy of human factor Vlll/von Willebrand glycoprotein: Effect of reducing reagents on structure and function. J Cell Biol. 1982: 632-640
    Google Scholar
  • 21 Furlan M. Von Willebrand factor: molecular size and functional activity. Ann Hematol 1996; 72: 341-348
    CrossrefPubMedGoogle Scholar
  • 22 Turecek PL, Gritsch H, Richter G, Auer W, Pichler L, Schwarz HP. Assessment of bleeding for the evaluation of therapeutic preparations in small animal models of antibody-induced hemophilia and von Willebrand disease. Thromb Haemost. in press
    Google Scholar
  • 23 Slappendel RJ. Von Willebrand’s disease in Dutch Kooiger dogs. Vet Quart 1995; 17: 21-22
    PubMedGoogle Scholar
  • 24 Drouet L, Roussi J, Bonneau M. et al The effect of recombinant human von Willebrand factor in pigs with severe von Willebrand’s disease. Blood 1995; 86 (Suppl. 01) 612a (abst)
    PubMedGoogle Scholar
  • 25 Stokol T, Parry BW, Mansell PD. Factor VIII activity in canine von Willebrand disease. Vet Clin Pathol 1995; 24: 81-90
    CrossrefPubMedGoogle Scholar
  • 26 Sodetz JM, Pizzo S, McKee P. Relationship of sialic acid to function and in vivo survival of human Factor Vlll/von Willebrand factor protein. J Biol Chem 1977; 252: 5538-5546
    PubMedGoogle Scholar
  • 27 Fischer BE, Turecek PL, Mitterer A, Schlokat U, Falkner FG, Schwarz HP, Domer F. Multimeric composition and structural analysis of a recombinant von Willebrand factor (rvWF). Thromb Haemost 1995; 73: 1166 (abst.)
    PubMedGoogle Scholar
  • 28 Giles AR, Tinlin S, Greenwood R. A canine model of hemophilic (factor VIII: C deficiency) bleeding. Blood 1982; 60: 727-730
    PubMedGoogle Scholar
  • 29 Mertz ET. The anomaly of a normal duke’s and a very prolonged saline bleeding time in swine suffering from an inherited bleeding disease. Am J Physiol 1942; 136: 360-362
    PubMedGoogle Scholar