Decreased stability and structural heterogeneity of the residual platelet glycoprotein IIb/IIIa complex in a variant of Glanzmann's thrombasthenia.
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 degrees 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.
Modderman
P W
PW
Central Laboratory of The Netherlands Red Cross Blood Transfusion Service, Amsterdam.
van Mourik
J A
JA
van Berkel
W
W
Cordell
J L
JL
Morel
M C
MC
Kaplan
C
C
Ouwehand
W H
WH
Huisman
J G
JG
von dem Borne
A E
AE
D000328:Adult; D000911:Antibodies, Monoclonal / Q000276:immunology; D001791:Blood Platelet Disorders / Q000097:blood*; D004355:Drug Stability; D004591:Electrophoresis, Polyacrylamide Gel; D005260:Female; D006801:Humans; D007123:Immunoelectrophoresis, Two-Dimensional; D015394:Molecular Structure; D010979:Platelet Function Tests; D010980:Platelet Membrane Glycoproteins / Q000276:immunology; D013915:Thrombasthenia / Q000097:blood*
D000911:Antibodies, Monoclonal; D010980:Platelet Membrane Glycoproteins