Platelet activation and aggregation induced by recombinant von Willebrand factors reproducing four type 2B von Willebrand disease missense mutations

Thrombosis and Haemostasis

Volumn 79, Issue 1, 1998, Pages 211-216

  De Romeuf, Christophe ^a,b   Hilbert, Lysiane ^a   Mazurier, Claudine ^a  

^a Laboratoire Francais du Fractionnement et des Biotechnologies   (France)

^b Lab Francais Fraction Biotechnologie   (France)

Author keywords

[No Author keywords available]

Indexed keywords

AURINTRICARBOXYLIC ACID; EDETIC ACID; GLYCOPROTEIN IB; MONOCLONAL ANTIBODY; RISTOCETIN; VON WILLEBRAND FACTOR;

ARTICLE; HUMAN; MISSENSE MUTATION; NORMAL HUMAN; PRIORITY JOURNAL; THROMBOCYTE ACTIVATION; THROMBOCYTE AGGREGATION; VON WILLEBRAND DISEASE;

EID: 0031974270     PISSN: 03406245     EISSN: None     Source Type: Journal    
DOI: 10.1055/s-0037-1614241     Document Type: Article

References (27)

1. Ruggeri ZM, Ware J. The structure and function of von Willebrand factor. Thromb Haemost 1992; 67: 594-9.
2. Sixma JJ, De Groot PG. Regulation of platelet adhesion to the vessel wall. Ann NY Acad Sci 1994; 714: 190-9.
3. Sadler JE. A revised classification of von Willebrand disease. For the subcommittee on von Willebrand factor of the Scientific and Standardization Committee of the International Society on Thrombosis and Haemostasis. Thromb Haemost 1994; 71: 520-5.
4. Ruggeri ZM, Pareti FI, Mannucci PM, Ciavarella N, Zimmerman TS. Heightened interaction between platelets and factor VIII/von Willebrand factor in a new subtype of von Willebrand's disease. New Engl J Med 1980; 302: 11047-51.
5. Murata M, Fukuyama M, Satoh K, Fujimura K, Yoshioka A, Takahashi H, Handa M, Kawai Y, Watanabe K, Ikeda Y. Low shear stress can initiate von Willebrand factor-dependent platelet aggregation in patients with type-IIB and platelet-type von Willebrand disease. J Clin Invest 1993; 92: 1555-8.
6. Ginsburg D, Sadler JE. Von Willebrand disease: a database of point mutations, insertions and deletions. Thromb Haemost 1993; 64: 326-32.
7. Fujimura Y, Titani K, Holland LZ, Robert JR, Elder JH, Ruggeri ZM, Zimmerman TS. Von Willebrand factor: a reduced and alkylated 52/48 kDa fragment beginning at amino acid residue 449 contains the domain interacting with platelet glycoprotein Ib. J Biol Chem 1986; 261: 381-5.
8. De Romeuf C, Mazurier C. Interest of a simple and fast method for platelet von Willebrand factor characterization. Thromb Res 1996; 83: 287-98.
9. Hilbert L, Gaucher C, de Romeuf C, Horellou MH, Vink T, Mazurier C. Leu 697 → Val mutation in mature von Willebrand factor is responsible for type IIB von Willebrand disease. Blood 1994; 83: 1542-50.
10. Hilbert L, Gaucher C and Mazurier C. Effects of different amino-acid substitutions in the Leucine 694-Proline 708 segment of recombinant von Willebrand factor. Br J Haematol 1995; 91: 983-90.
11. Hilbert L, Gaucher C, de Romeuf C, Parquet A, Abgrall JF, Trzeciak C, Mazurier C. Identification of "new" type 2B von Willebrand disease mutation: R543Q, R545P and R578L. Br J Haematol 1996; 93 suppl 2: abstract 1175.
12. Bonthron D, Orr EC, Mitsock LM, Ginsburg D, Handin RI, Orkin SH. Nucleotide sequence of the pre-pro-von Willebrand factor cDNA. Nucleic Acid Res 1986; 14: 7125-7.
13. Mazurier C, Parquet-Gernez A, Goudemand M. Dosage de l'antigène lié au facteur VIII par la technique ELISA. Intérêt dans l'étude de la maladie de Willebrand. Path Biol 1977; 25: 18-24.
14. Mazurier C, Samor B, Goudemand M. Improved characterization of plasma von Willebrand factor heterogeneity when using 2.5% agarose gel electrophoresis. Thromb Haemost 1986; 55: 61-4.
15. Rabinowitz I, Randi AM, Shindler KS, Tuley EA, Ruatogi PK and Sadler JE. Type IIB mutation His 505→Asp implicates a new segment in the control of von Willebrand factor binding to platelet glycoprotein Ib. J Biol Chem 1993; 268: 20497-501.
16. Gralnick HR, Williams SB and Morisato DK. Effect of multimeric structure of the factor VIII/vWF protein on binding to platelets. Blood 1981; 58: 387-97.
17. Federici AB, Bader R, Pagani S, Colibretti AL, De Marco L, Mannucci PM. Binding of von Willebrand factor (vWF) to glycoproteins (GP) Ib and IIb-IIIa complex: affinity is related to multimeric size. Br J Haematol 1987; 73: 93-9.
18. Gralnick HR, Williams SB, Mc Keown LP, Rick ME, Maisonneuve P, Jeanneau C, Sultan Y. Von Willebrand's disease with spontaneous platelet aggregation induced by an abnormal plasma von Willebrand factor. J Clin Invest 1985; 76: 1522-9.
19. De Marco L, Girolami A, Zimmerman TS, Ruggeri ZM. Interaction of purified IIB von Willebrand factor with the platelet membrane glycoprotein Ib induces fibrinogen binding to the glycoprotein IIb-IIIa complex and initiates aggregation. Proc Natl Acad Sci 1985; 82: 7424-8.
20. De Marco L, Mazzuccato M, Del Ben MG, Budde U, Federici AB, Girolami A, Ruggeri ZM. Type IIB von Willebrand factor with normal sialic acid content induces platelet aggregation in the absence of ristocetin. Role of platelet activation, fibrinogen and two distinct membrane receptors. J Clin Invest 1987; 80: 475-82.
21. Ikeda Y, Handa M, Kawano K, Kamata T, Murata M, Araki Y, Anbo H, Kawai Y, Watanabe K, Itagaki I, Sakai K, Ruggeri ZM. The role of von Willebrand factor and fibrinogen in platelet aggregation under varying shear stress. J Clin Invest 1991; 87: 1234-40.
22. 2+ concentration. Thromb Haemost 1993; 70: 697-701.
23. Chow TW, Hellums JD, Moake JL, Kroll MH. Shear stress-induced von Willebrand factor binding to platelet glycoprotein-Ib initiates calcium influx associated with aggregation. Blood 1992; 80: 113-20.
24. Philipp DR, Charo IF, Scarborough RM. GpIIb-IIIa: the responsive integrin. Cell 1991; 65: 359-65.
25. 2+ binding properties of the platelet glycoprotein IIb ligand domain. J Biol Chem 1992; 267: 1001-7.
26. Cooney KA, Ginsburg D. Comparative analysis of type 2b von Willebrand disease mutations : implications for the mechanism of von Willebrand factor binding to platelets. Blood 1996; 87: 2322-8.
27. Gaucher C, de Romeuf C. Rauïs-Morret M, Corrazza F, Fondu P, Mazurier C. Diagnosis of subtype 2B von Willebrand disease in a patient with 2A phenotype of plasma von Willebrand factor. Thromb Haemost 1995; 73: 610-6.