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Thromb Haemost 1985; 54(03): 626-629
DOI: 10.1055/s-0038-1660085
Original Article
Schattauer GmbH Stuttgart

Diversity of Glycoprotein Deficiencies in Glanzmann’s Thrombasthenia

M Meyer
1   The Department of Medical Genetics, Medical Academy Erfurt, German Democratic Republic, G. D. R.
,
F H Herrmann
2   The Institute of Medical Genetics, Ernst-Moritz-Arndt University, Greifswald, G. D. R.
› Author Affiliations
Further Information

Publication History

Received 25 February 1985

Accepted 29 July 1985

Publication Date:
19 July 2018 (online)

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Summary

The platelet proteins of 9 thrombasthenic patients from 7 families were analysed by high resolution two-dimensional gel electrophoresis (HR-2DE) and crossed immunoelectrophoresis (CIE). In 7 patients both glycoproteins (GPs) IIb and Ilia were absent or reduced to roughly the same extent. In two related patients only a trace of GP Ilb-IIIa complex was detected in CIE, but HR-2DE revealed a glycopeptide in the position of GP Ilia in an amount comparable to type II thrombasthenia. This GP Ilia-like component was neither recognized normally by anti-GP Ilb-IIIa antibodies nor labeled by surface iodination. In unreduced-reduced two-dimensional gel electrophoresis two components were observed in the region of GP Ilia. The assumption of a structural variant of GP Ilia in the two related patients is discussed.

Keywords

Thrombasthenia - Platelet membrane glycoproteins - Glycoprotein IIb-IIIa complex
 

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