PMID: 8949396Aug 1, 1996Paper

Giant platelets, megakaryocytes and the expression of glycoprotein Ib-IX complexes

Comptes rendus de l'Académie des sciences. Série III, Sciences de la vie
Paquita Nurden, Alan T Nurden

Abstract

The role of GP Ib-IX complexes in platelet adhesion was discovered from studies on patients with the Bernard-Soulier Syndrome (BSS). In this inherited disorder, the bulk of the platelets are round and giant. Furthermore, the platelet count is decreased, often severely so. The relationship between these abnormalities and the deficiency of GP Ib-IX is not well understood. In normal discoid platelets, the bulk of GP Ib-IX is found on the plasma membrane. After platelet activation by thrombin, this distribution changes and the majority of GP Ib-IX complexes are located within the surface-connected canalicular system (SCCS). The platelets now possess pseudopods and are spheroid. Cytoskeletal modifications accompany these changes. We now report that platelets of a BSS variant with a qualitative defect of GP Ib show no translocation of GP Ib in response to thrombin, suggesting that the linkage with the cytoskeleton is impaired. Morphological studies of megakaryocytes (MK) from BSS patients show an altered maturation and an abnormal development of the membrane systems, implying a role for GP Ib-IX in megakaryocytopoiesis. In a case of Epstein syndrome, where giant platelets and thrombocytopenia are associated with deafness and renal dy...Continue Reading

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