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Glycoprotein Defects Responsible for Abnormal Platelet Function in Inherited Platelet Disorders

  • Alan T. Nurden

Abstract

The study of congenital bleeding syndromes has yielded much information on how normal platelets function. In particular, the characterization of the molecular defects in disorders of platelet adhesion and aggregation has resulted in specific roles being proposed for different membrane glycoproteins and α-granule proteins in these processes. In this chapter, we will highlight three major inherited disorders of platelet function: Bemard-Soulier syndrome (BSS), gray platelet syndrome (GPS), and Glanzmann’s thrombasthenia (GT). Each disorder will be introduced by a brief description of the platelet function abnormalities present. This will be followed by a detailed discussion of the specific molecular defects that distinguish the platelets of each syndrome. Emphasis will be placed on reviewing recent data, although an attempt will be made to interpret previous studies in the context of the new advances. Where possible, the different molecular abnormalities will be assessed in terms of how their presence leads to modifications of platelet adhesion and aggregation mechanisms in hemostasis.

Keywords

Platelet Glycoprotein Normal Platelet Clot Retraction Disulfide Reduction Platelet Membrane Glycoprotein 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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Copyright information

© Plenum Press, New York 1985

Authors and Affiliations

  • Alan T. Nurden
    • 1
  1. 1.Unité 150 INSERMHôpital LariboisièreParis, Cedex 10France

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