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Rapid Diagnosis of the Bernard-Soulier Syndrome Using a Monoclonal Antibody Against Glycoprotein Ib

  • E. Taaning
  • S. Stenbjerg
  • J. Ingerslev
Conference paper

Abstract

The Bernard-Soulier syndrome is an autosomal recessive trait characterized by deficient synthesis of platelet surface glycoprotein Ib (GP Ib) resulting in impaired platelet function and bleeding tendency [1]. GP Ib is a link protein for assembly of platelet and von Willebrand factor [2] during haemostasis. The development of a monoclonal antibody specific for GP Ib, denoted AN51, was reported by McMichael and coworkers [3]. The antibody is now commercially available. Employing this antibody and using a previously reported ELISA method for detection of platelet surface proteins [4], we developed a simple technique for direct detection of GP Ib.

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References

  1. 1.
    Degos L, Tobelem G, Lethielleux P, Levy-Tolando S, Caen JP, Colombani (1977) Molecular defects in platelets from patients with Bernard-Soulier syndrome. Blood 50:899–903PubMedGoogle Scholar
  2. 2.
    Weiss HJ (1980) Congenital disorders of platelet function. Seminars in Haematology 17:228–241Google Scholar
  3. 3.
    McMichael AJ, Rust NA, Pilch JR, Sochinsky R, Morton J, Mason DY, Ruan C, Tobelem G, Caen J (1981) Monoclonal antibody to human platelet glycoprotein Ib: I. Immunological studies. Br J Haematol 49:511–519PubMedCrossRefGoogle Scholar
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    Taaning E (1985) Microplate enzyme immuno-assay for detection of platelet antibodies. Tissue Antig 25:19–27CrossRefGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 1988

Authors and Affiliations

  • E. Taaning
    • 1
  • S. Stenbjerg
    • 1
  • J. Ingerslev
    • 1
  1. 1.Copenhagen, AarhusDenmark

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