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Thromb Haemost 1986; 55(03): 338-341
DOI: 10.1055/s-0038-1661559
Original Article
Schattauer GmbH Stuttgart

Further Studies on Aggregation of Platelet-Type von Willebrand’s Disease Platelets by Human von Willebrand Factor

H Takahashi
The First Department of Internal Medicine, Niigata University School of Medicine, Niigata, Japan
,
W Tatewaki
The First Department of Internal Medicine, Niigata University School of Medicine, Niigata, Japan
,
M Hanano
The First Department of Internal Medicine, Niigata University School of Medicine, Niigata, Japan
,
R Nagayama
The First Department of Internal Medicine, Niigata University School of Medicine, Niigata, Japan
,
A Shibata
The First Department of Internal Medicine, Niigata University School of Medicine, Niigata, Japan
› Author Affiliations
Further Information

Publication History

Received 21 January 1986

Accepted 21 March 1986

Publication Date:
18 July 2018 (online)

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Summary

Platelet-type von Willebrand’s disease (vWD) is a bleeding disorder characterized by a heightened interaction between platelets and von Willebrand factor (vWF) as the result of an intrinsic platelet abnormality (probably in GPIb). Platelet aggregability was nearly normal in response to thrombin, wheat germ agglutinin and Ricinus communis agglutinin in this disorder. Unmodified platelets showed no aggregation upon the addition of peanut agglutinin. Partially purified human vWF induced little aggregation of washed patient platelets, but the aggregation was greatly enhanced in the presence of plasma devoid of vWF. Monoclonal antibodies directed against GPIb and GPIIb/IIIa as well as EDTA completely inhibited vWF-induced aggregation. These results indicate that human vWF induces aggregation of platelet-type vWD platelets in the presence of divalent cations and some plasma cofactor(s), and that both GPIb and GPIIb/IIIa are involved in this aggregation.

Keywords

Platelet-type von Willebrand’s disease - von Willebrand factor - Platelet membrane glycoprotein (GP) Ib and GPIIb/IIIa - Receptor - Platelet aggregation
 

  • References

  • 1 Ruggeri ZM, Zimmerman TS. Variant von Willebrand’s disease. Characterization of two subtypes by analysis of multimeric composition of factor VUI/von Willebrand factor in plasma and platelets. J Clin Invest 1980; 65: 1318-1325
    CrossrefPubMedGoogle Scholar
  • 2 Hoyer LW, Shainoff JR. Factor-VUI-related protein circulates in normal human plasma as high molecular weight multimers. Blood 1980; 55: 1056-1059
    PubMedGoogle Scholar
  • 3 Fowler WE, Fretto LJ, Hamilton KK, Erickson HP, McKee PA. Substructure of human von Willebrand factor. J Clin Invest 1985; 76: 1491-1500
    CrossrefPubMedGoogle Scholar
  • 4 Kirby EP, Mills DC B. The interaction of bovine factor VIII with human platelets. J Clin Invest 1975; 56: 491-502
    CrossrefPubMedGoogle Scholar
  • 5 Cooper HA, Clemetson KJ, Lüscher EF. Human platelet membrane receptor for bovine von Willebrand factor (platelet aggregating factor): An integral membrane glycoprotein. Proc Natl Acad Sci USA 1979; 76: 1069-1073
    CrossrefPubMedGoogle Scholar
  • 6 Kao K-J, Pizzo SV, McKee PA. Demonstration and characterization of specific binding sites for factor VIII/von Willebrand factor on human platelets. J Clin Invest 1979; 63: 656-664
    CrossrefPubMedGoogle Scholar
  • 7 Kao K-J, Pizzo SV, McKee PA. Platelet receptors for human factor VIII/von Willebrand factor protein: Functional correlation of receptor occupancy and ristocetin-induced platelet aggregation. Proc Natl Acad Sci USA 1979; 76: 5317-5320
    CrossrefPubMedGoogle Scholar
  • 8 Moake JL, Olson JD, Troll JH, Tang SS, Funicella T, Peterson DM. Binding of radioiodinated human von Willebrand factor to Bernard-Soulier, thrombasthenic and von Willebrand’s disease platelets. Thromb Res 1980; 19: 21-27
    CrossrefPubMedGoogle Scholar
  • 9 Coller BS, Peerschke EI, Scudder LE, Sullivan CA. Studies with a murine monoclonal antibody that abolishes ristocetin-induced binding of von Willebrand factor to platelets: Additional evidence in support of GPIb as a platelet receptor for von Willebrand factor. Blood 1983; 61: 99-110
    PubMedGoogle Scholar
  • 10 Ruggeri ZM, De Marco L, Gatti L, Bader R, Montgomery RR. Platelets have more than one binding site for von Willebrand factor. J Clin Invest 1983; 72: 1-12
    CrossrefPubMedGoogle Scholar
  • 11 Schullek J, Jordan J, Montgomery RR. Interaction of von Willebrand factor with human platelets in the plasma milieu. J Clin Invest 1984; 73: 421-428
    CrossrefPubMedGoogle Scholar
  • 12 Plow EF, Srouji AH, Meyer D, Marguerie G, Ginsberg MH. Evidence that three adhesive proteins interact with a common recognition site on activated platelets. J Biol Chem 1984; 259: 5388-5391
    PubMedGoogle Scholar
  • 13 Takahashi H. Studies on the pathophysiology and treatment of von Willebrand’s disease. IV. Mechanism of increased ristocetin-induced platelet aggregation in von Willebrand’s disease. Thromb Res 1980; 19: 857-867
    CrossrefPubMedGoogle Scholar
  • 14 Takahashi H, Handa M, Watanabe K, Ando Y, Nagayama R, Hattori A, Shibata A, Federici AB, Ruggeri ZM, Zimmerman TS. Further characterization of platelet-type von Willebrand’s disease in Japan. Blood 1984; 64: 1254-1262
    PubMedGoogle Scholar
  • 15 Weiss HJ, Meyer D, Rabinowitz R, Pietu G, Girma J-P, Vicic WJ, Rogers J. PSeudo-von Willebrand’s disease. An intrinsic platelet defect with aggregation by unmodified human factor VIII/von Willebrand factor and enhanced adsorption of its high-molecular-weight multimers. N Engl J Med 1982; 306: 326-333
    CrossrefPubMedGoogle Scholar
  • 16 Miller JL, Castella A. Platelet-type von Willebrand’s disease: Characterization of a new bleeding disorder. Blood 1982; 60: 790-794
    PubMedGoogle Scholar
  • 17 Takahashi H, Nagayama R, Hattori A, Shibata A. Botrocetin- and Polybrene-induced platelet aggregation in platelet-type von Willebrand’s disease. Am J Hematol 1985; 18: 179-189
    CrossrefPubMedGoogle Scholar
  • 18 Takahashi H, Shibata A. Agglutination of platelet-type von Willebrand’s disease platelets by bovine von Willebrand factor. Thromb Haemostas 1985; 53: 204-207
    PubMedGoogle Scholar
  • 19 Miller JL, Kupinski JM, Castella A, Ruggeri ZM. von Willebrand factor binds to platelets and induces aggregation in platelet-type but not type IIB von Willebrand’s disease. J Clin Invest 1983; 72: 1532-1542
    CrossrefPubMedGoogle Scholar
  • 20 Born GV R. Aggregation of blood platelets by adenosine diphosphate and its reversal. Nature 1962; 194: 927-929
    PubMedGoogle Scholar
  • 21 Hardisty RM, Macpherson JC. A one-stage factor VIII (anti-haemophilic globulin) assay and its use on venous and capillary plasma. Thrombos Diathes Haemorrh 1962; 7: 215-229
    PubMedGoogle Scholar
  • 22 Amiral J, Adalbert B, Adam M. Application of enzyme immunoassays to coagulation testing. Clin Chem 1984; 30: 1512-1516
    PubMedGoogle Scholar
  • 23 Macfarlane DE, Stibbe J, Kirby EP, Zucker MB, Grant RA, McPherson J. A method for assaying von Willebrand factor (ristocetin cofactor). Thrombos Diathes Haemorrh 1975; 34: 306-308
    PubMedGoogle Scholar
  • 24 Jenkins CS P, Phillips DR, Clemetson KJ, Meyer D, Larrieu M-J, Lüscher EF. Platelet membrane glycoproteins implicated in ristocetin-induced aggregation. Studies of the proteins on platelets from patients with Bernard-Soulier syndrome and von Willebrand’s disease. J Clin Invest 1976; 57: 112-124
    CrossrefPubMedGoogle Scholar
  • 25 Nurden AT, Dupuis D, Kunicki TJ, Caen JP. Analysis of the glycoprotein and protein composition of Bernard-Soulier platelets by single and two-dimensional sodium dodecyl sulfate-polyacrylamide gel electrophoresis. J Clin Invest 1981; 67: 1431-1440
    CrossrefPubMedGoogle Scholar
  • 26 Greenberg J, Packham MA, Cazenave J-P, Reimers H-J, Mustard JF. Effects on platelet function of removal of platelet sialic acid by neuraminidase. Lab Invest 1975; 32: 476-484
    PubMedGoogle Scholar
  • 27 Naim HY, Clemetson KJ, Lüscher EF. Effects of galactose-binding lectins on human blood platelets: Identity of the peanut agglutinin receptor with the von Willebrand factor receptor. Thromb Res 1982; 26: 431-441
    CrossrefPubMedGoogle Scholar
  • 28 Moroi M, Jung SM. Selective staining of human platelet glycoproteins using nitrocellulose transfer of electrophoresed protein and peroxidase-conjugated lectins. Biochim Biophys Acta 1984; 798: 295-301
    CrossrefPubMedGoogle Scholar
  • 29 Nachman RL, Tarasov E, Weksler BB, Ferris B. Wheat germ agglutinin affinity chromatography of human platelet membrane gylcoproteins. Thromb Res 1977; 12: 91-104
    PubMedGoogle Scholar
  • 30 Okumura T, Hasitz M, Jamieson GA. Platelet glycocalicin. Interaction with thrombin and role as thrombin receptor of the platelet surface. J Biol Chem 1978; 253: 3435-3443
    PubMedGoogle Scholar
  • 31 McGregor JL, Brochier J, Wild F, Follea G, Trzeciak M-C, James E, Dechavanne M, McGregor L, Clemetson KJ. Monoclonal antibodies against platelet membrane glycoproteins. Characterization and effect on platelet function. Eur J Biochem 1983; 131: 427-436
    CrossrefPubMedGoogle Scholar
  • 32 Miller JL, Lyle VA. The role of membrane glycoproteins Ib and IIb/ IIIa in aggregation induced by von Willebrand factor or by low concentrations of ristocetin in platelet-type von Willebrand’s disease. Thromb Haemostas 1985 54. 138 (Abstr)
    PubMedGoogle Scholar
  • 33 Gralnick HR, Williams SB, Coller BS. Asialo von Willebrand factor interactions with platelets. Interdependence of glycoproteins Ib and IIb/IIIa for binding and aggregation. J Clin Invest 1985; 75: 19-25
    CrossrefPubMedGoogle Scholar
  • 34 De Marco L, Girolami A, Russell S, Ruggeri ZM. Interaction of asialo von Willebrand factor with glycoprotein Ib induces fibrinogen binding to the glycoprotein IIb/IIIa complex and mediates platelet aggregation. J Clin Invest 1985; 75: 1198-1203
    CrossrefPubMedGoogle Scholar
  • 35 Takahashi H, Hattori A, Nagayama R, Hanano M, Fuse I, Takizawa S, Takeshige T, Shibata A. Morphological studies of platelet aggregates induced by human von Willebrand factor in platelet-type von Willebrand’s disease. Thromb Res 1985; 38: 433-438
    CrossrefPubMedGoogle Scholar
  • 36 Zimmerman TS, Ruggeri ZM, Fulcher CA. Factor VIII/von Willebrand factor. Prog Hematol 1983; 13: 279-309
    PubMedGoogle Scholar
  • 37 Takahashi H, Nagayama R, Hattori A, Shibata A. Platelet aggregation induced by DDAVP in platelet-type von Willebrand’s disease. N Engl J Med 1984; 310: 722-723
    PubMedGoogle Scholar
  • 38 Miller JL, Boselli BD, Kupinski JM. In vivo interaction of von Willebrand factor with platelets following cryoprecipitate transfusion in platelet-type von Willebrand’s disease. Blood 1984; 63: 226-230
    PubMedGoogle Scholar