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Meyer M, Thieme A, Jablonka B, et al. A new variant of Glanzmann's thrombasthenia with defective activation-dependent fibrinogen binding and altered expression of epitopes for several monoclonal antibodies against GP IIb-IIIa. Platelets. 1996;7(4):215-24doi: 10.3109/09537109609023581.
Meyer, M., Thieme, A., Jablonka, B., Just, M., Ströhl, C., Schellenberg, I., & Kirchmaier, C. M. (1996). A new variant of Glanzmann's thrombasthenia with defective activation-dependent fibrinogen binding and altered expression of epitopes for several monoclonal antibodies against GP IIb-IIIa. Platelets, 7(4), 215-24. https://doi.org/10.3109/09537109609023581
Meyer, M, et al. "A new variant of Glanzmann's thrombasthenia with defective activation-dependent fibrinogen binding and altered expression of epitopes for several monoclonal antibodies against GP IIb-IIIa." Platelets vol. 7,4 (1996): 215-24. doi: https://doi.org/10.3109/09537109609023581
Meyer M, Thieme A, Jablonka B, Just M, Ströhl C, Schellenberg I, Kirchmaier CM. A new variant of Glanzmann's thrombasthenia with defective activation-dependent fibrinogen binding and altered expression of epitopes for several monoclonal antibodies against GP IIb-IIIa. Platelets. 1996;7(4):215-24. doi: 10.3109/09537109609023581. PMID: 21043690.
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Platelets. 1996;7(4):215-24. doi: 10.3109/09537109609023581.

A new variant of Glanzmann's thrombasthenia with defective activation-dependent fibrinogen binding and altered expression of epitopes for several monoclonal antibodies against GP IIb-IIIa.

Platelets

M Meyer, A Thieme, B Jablonka, M Just, C Ströhl, I Schellenberg, C M Kirchmaier

Affiliations

  1. Institut für vaskulär Biologie and Medizin. Erfurt; B. Jablonka, M. Just, Casella AG, Frankfurt/M.; C. M. Kirchmaier, Red Cross Blood Transfusion Service Hessen, J.W. Goethe University Frankfurt/M, Germany.

PMID: 21043690 DOI: 10.3109/09537109609023581

Abstract

In a family, a moderate bleeding disorder in two patients has been specified as Glanzmann's thrombasthenia because of characteristic defects in platelet function. Analysis of platelet membrane glycoproteins revealed about a 50% decrease in the amount of GP IIb-IIIa complex (α(11b)β(3) integrin), which appeared normal with respect to electrophoretic mobility, apparent M, and isoelectric behaviour of GP IIb and GP IIIa. Content of platelet fibrinogen (Fg) was normal. [(125)I]Fg binding to ADP-stimulated platelets was strongly reduced but K(d) values indicated a much higher affinity of the residual receptors for both [(125)I]Fg and RGD peptide. Fg bound to the isolated complex as detected by crossed immunoelectrophoresis and there was substantial expression of endogenous Fg on the surface of washed thrombin-stimulated platelets. RGD-peptide induced increased binding of conformation-specific monoclonal antibodies (Mabs) LIBS 1 and PMI-1. Flow cytometric analysis revealed defective binding of nine Mabs, among them two out of three tested antibodies specific for GP IIIa (C 17, AP 5). Results indicate a genetic variant of GP IIb-IIIa complex with the structural abnormality possibly related to defective conformational change upon activation.

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