Decreased stability and structural heterogeneity of the residual platelet glycoprotein IIb/IIIa complex in a variant of Glanzmann's thrombasthenia

P. W. Modderman; J. A. van           Mourik; W. van Berkel; J. L. Cordell; M. C. Morel; C. Kaplan; W. H. Ouwehand; J. G. Huisman; A. E. G. K. von dem           Borne

doi:10.1111/j.1365-2141.1989.tb00290.x

Decreased stability and structural heterogeneity of the residual platelet glycoprotein IIb/IIIa complex in a variant of Glanzmann's thrombasthenia

P. W. Modderman^*
, J. A. van Mourik
, W. van Berkel
, J. L. Cordell
, M. C. Morel
, C. Kaplan
, W. H. Ouwehand
, J. G. Huisman
, A. E. G. K. von dem Borne

^*Corresponding author for this work

University of Amsterdam
University of Oxford
Institut national de la transfusion sanguine

Research output: Contribution to journal › Article › Academic › peer-review

Abstract

A patient is described with a disturbance of platelet function comparable to that in Glanzmann's thrombasthenia. Platelet aggregation and binding of fibrinogen to the patient's platelets were defective and thrombin‐induced clot retraction was absent. The platelet fibrinogen content was only moderately reduced. As measured by monoclonal antibody binding in the presence of divalent cations, the platelets contained about 15% of the normal amount of GPIIb and GPIIIa and only 6% of the normal amount of intact GPIIb/IIIa complex. The residual GPIIb/IIIa complex exhibited a decreased stability as shown by the lack of binding of a complex‐dependent anti‐GPIIb/IIIa antibody to platelets incubated with ethylene diamine tetraacetic acid (EDTA) at 22°C. Crossed immunoelectrophoresis (CIE) in the presence of divalent cations showed partial dissociation of GPIIb/IIIa as well as the presence of two forms of the residual intact GPIIb/IIIa complex. In addition, both CIE in the presence of the EDTA and two‐dimensional sodium dodecyl sulphate (SDS) gel electrophoresis showed the presence of two forms of GPIIb. This form of thrombasthenia is characterized by a defective platelet function, a marked reduction of GPIIb and GPIIIa, decreased stability of the residual GPIIb/IIIa complex and structural heterogeneity of GPIIb. Copyright © 1989, Wiley Blackwell. All rights reserved

Original language	English
Pages (from-to)	514-521
Journal	British journal of haematology
Volume	73
Issue number	4
DOIs	https://doi.org/10.1111/j.1365-2141.1989.tb00290.x
Publication status	Published - 1989
Externally published	Yes

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Modderman, P. W., van Mourik, J. A., Berkel, W. V., Cordell, J. L., Morel, M. C., Kaplan, C., Ouwehand, W. H., Huisman, J. G., & von dem Borne, A. E. G. K. (1989). Decreased stability and structural heterogeneity of the residual platelet glycoprotein IIb/IIIa complex in a variant of Glanzmann's thrombasthenia. British journal of haematology, 73(4), 514-521. https://doi.org/10.1111/j.1365-2141.1989.tb00290.x

@article{86138fbee244477bb348e4f9f779b337,

title = "Decreased stability and structural heterogeneity of the residual platelet glycoprotein IIb/IIIa complex in a variant of Glanzmann's thrombasthenia",

abstract = "A patient is described with a disturbance of platelet function comparable to that in Glanzmann's thrombasthenia. Platelet aggregation and binding of fibrinogen to the patient's platelets were defective and thrombin‐induced clot retraction was absent. The platelet fibrinogen content was only moderately reduced. As measured by monoclonal antibody binding in the presence of divalent cations, the platelets contained about 15\% of the normal amount of GPIIb and GPIIIa and only 6\% of the normal amount of intact GPIIb/IIIa complex. The residual GPIIb/IIIa complex exhibited a decreased stability as shown by the lack of binding of a complex‐dependent anti‐GPIIb/IIIa antibody to platelets incubated with ethylene diamine tetraacetic acid (EDTA) at 22°C. Crossed immunoelectrophoresis (CIE) in the presence of divalent cations showed partial dissociation of GPIIb/IIIa as well as the presence of two forms of the residual intact GPIIb/IIIa complex. In addition, both CIE in the presence of the EDTA and two‐dimensional sodium dodecyl sulphate (SDS) gel electrophoresis showed the presence of two forms of GPIIb. This form of thrombasthenia is characterized by a defective platelet function, a marked reduction of GPIIb and GPIIIa, decreased stability of the residual GPIIb/IIIa complex and structural heterogeneity of GPIIb. Copyright {\textcopyright} 1989, Wiley Blackwell. All rights reserved",

author = "Modderman, \{P. W.\} and \{van Mourik\}, \{J. A.\} and Berkel, \{W. van\} and Cordell, \{J. L.\} and Morel, \{M. C.\} and C. Kaplan and Ouwehand, \{W. H.\} and Huisman, \{J. G.\} and \{von dem Borne\}, \{A. E. G. K.\}",

year = "1989",

doi = "10.1111/j.1365-2141.1989.tb00290.x",

language = "English",

volume = "73",

pages = "514--521",

journal = "British journal of haematology",

issn = "0007-1048",

publisher = "Wiley Blackwell",

number = "4",

}

Modderman, PW, van Mourik, JA, Berkel, WV, Cordell, JL, Morel, MC, Kaplan, C, Ouwehand, WH, Huisman, JG & von dem Borne, AEGK 1989, 'Decreased stability and structural heterogeneity of the residual platelet glycoprotein IIb/IIIa complex in a variant of Glanzmann's thrombasthenia', British journal of haematology, vol. 73, no. 4, pp. 514-521. https://doi.org/10.1111/j.1365-2141.1989.tb00290.x

TY - JOUR

T1 - Decreased stability and structural heterogeneity of the residual platelet glycoprotein IIb/IIIa complex in a variant of Glanzmann's thrombasthenia

AU - Modderman, P. W.

AU - van Mourik, J. A.

AU - Berkel, W. van

AU - Cordell, J. L.

AU - Morel, M. C.

AU - Kaplan, C.

AU - Ouwehand, W. H.

AU - Huisman, J. G.

AU - von dem Borne, A. E. G. K.

PY - 1989

Y1 - 1989

N2 - A patient is described with a disturbance of platelet function comparable to that in Glanzmann's thrombasthenia. Platelet aggregation and binding of fibrinogen to the patient's platelets were defective and thrombin‐induced clot retraction was absent. The platelet fibrinogen content was only moderately reduced. As measured by monoclonal antibody binding in the presence of divalent cations, the platelets contained about 15% of the normal amount of GPIIb and GPIIIa and only 6% of the normal amount of intact GPIIb/IIIa complex. The residual GPIIb/IIIa complex exhibited a decreased stability as shown by the lack of binding of a complex‐dependent anti‐GPIIb/IIIa antibody to platelets incubated with ethylene diamine tetraacetic acid (EDTA) at 22°C. Crossed immunoelectrophoresis (CIE) in the presence of divalent cations showed partial dissociation of GPIIb/IIIa as well as the presence of two forms of the residual intact GPIIb/IIIa complex. In addition, both CIE in the presence of the EDTA and two‐dimensional sodium dodecyl sulphate (SDS) gel electrophoresis showed the presence of two forms of GPIIb. This form of thrombasthenia is characterized by a defective platelet function, a marked reduction of GPIIb and GPIIIa, decreased stability of the residual GPIIb/IIIa complex and structural heterogeneity of GPIIb. Copyright © 1989, Wiley Blackwell. All rights reserved

AB - A patient is described with a disturbance of platelet function comparable to that in Glanzmann's thrombasthenia. Platelet aggregation and binding of fibrinogen to the patient's platelets were defective and thrombin‐induced clot retraction was absent. The platelet fibrinogen content was only moderately reduced. As measured by monoclonal antibody binding in the presence of divalent cations, the platelets contained about 15% of the normal amount of GPIIb and GPIIIa and only 6% of the normal amount of intact GPIIb/IIIa complex. The residual GPIIb/IIIa complex exhibited a decreased stability as shown by the lack of binding of a complex‐dependent anti‐GPIIb/IIIa antibody to platelets incubated with ethylene diamine tetraacetic acid (EDTA) at 22°C. Crossed immunoelectrophoresis (CIE) in the presence of divalent cations showed partial dissociation of GPIIb/IIIa as well as the presence of two forms of the residual intact GPIIb/IIIa complex. In addition, both CIE in the presence of the EDTA and two‐dimensional sodium dodecyl sulphate (SDS) gel electrophoresis showed the presence of two forms of GPIIb. This form of thrombasthenia is characterized by a defective platelet function, a marked reduction of GPIIb and GPIIIa, decreased stability of the residual GPIIb/IIIa complex and structural heterogeneity of GPIIb. Copyright © 1989, Wiley Blackwell. All rights reserved

UR - https://www.scopus.com/pages/publications/0024809994

UR - https://www.ncbi.nlm.nih.gov/pubmed/2611137

U2 - 10.1111/j.1365-2141.1989.tb00290.x

DO - 10.1111/j.1365-2141.1989.tb00290.x

M3 - Article

C2 - 2611137

SN - 0007-1048

VL - 73

SP - 514

EP - 521

JO - British journal of haematology

JF - British journal of haematology

IS - 4

ER -

Decreased stability and structural heterogeneity of the residual platelet glycoprotein IIb/IIIa complex in a variant of Glanzmann's thrombasthenia

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