A variant of Glanzmann's thrombasthenia with abnormal glycoprotein IIb-IIIa complexes in the platelet membrane.
J Clin Invest
; 79(3): 962-9, 1987 Mar.
Article
em En
| MEDLINE
| ID: mdl-3818957
ABSTRACT
Patient C.M. presented platelet function defects symptomatic of Glanzmann's thrombasthenia. However, analysis of surface-labeled platelets by SDS-polyacrylamide gel electrophoresis revealed the usual presence of the major glycoproteins, including GP IIb and GP IIIa. Platelet fibrinogen was not detected. Analysis of Triton X-100 extracts of Ca2+-washed C.M. platelets by crossed immunoelectrophoresis (CIE) showed normal amounts of GP IIb-IIIa complexes. However, when samples were electrophoresed through an agarose gel containing 125I-fibrinogen, the usual binding of fibrinogen to GP IIb-IIIa did not occur. Furthermore, the GP IIb-IIIa complexes showed an increased sensitivity to dissociation with EDTA, either after Triton X-100 solubilization or in the intact platelet membrane. For example, after incubation with EDTA at room temperature, the patient's platelets bound little of the monoclonal antibodies AP-2 or T10 (anti-GP IIb-IIIa complex) although normally binding Tab (anti-GP IIb). Patient C.M. appears to represent a subgroup of thrombasthenia where platelets contain unstable GP IIb-IIIa complexes unable to support fibrinogen binding.
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Transtornos Plaquetários
/
Plaquetas
/
Trombastenia
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Glicoproteínas da Membrana de Plaquetas
Limite:
Adult
/
Female
/
Humans
Idioma:
En
Revista:
J Clin Invest
Ano de publicação:
1987
Tipo de documento:
Article