Skip to main content
PMC home page
The Journal of Clinical Investigation logoLink to The Journal of Clinical Investigation
. 1982 Aug;70(2):304–311. doi: 10.1172/JCI110618

Characterization of the platelet membrane glycoprotein abnormalities in Bernard-Soulier syndrome and comparison with normal by surface-labeling techniques and high-resolution two-dimensional gel electrophoresis.

K J Clemetson, J L McGregor, E James, M Dechavanne, E F Lüscher
PMCID: PMC371237  PMID: 6284798

Abstract

The platelets from three patients with Bernard-Soulier syndrome have been analyzed by surface-labeling coupled with two-dimensional gel electrophoresis and compared with normals. As well as the previously described absence or deficiency in glycoprotein (GP) Ib(alpha) it could be shown that GP Ib beta and an additional low molecular weight glycoprotein GP17 were not detectable using carbohydrate-labeling methods or deficient to the same extent as the GPIb alpha subunit. In addition, the thrombin cleavable glycoprotein could not be detected using carbohydrate-labeling methods in two patients and was deficient in a third. This finding was confirmed in a fourth patient by one-dimensional gel electrophoresis. Thus, the changes in the membrane of Bernard-Soulier platelets are more complex than previously thought.

Full text

PDF
308

Images in this article

306Image
on p.306
307Image
on p.307
307Image
on p.307
308Image
on p.308
308Image
on p.308
309Image
on p.309
309Image
on p.309

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Ali-Briggs E. F., Clemetson K. J., Jenkins C. S. Antibodies against platelet membrane glycoproteins. I. Crossed immunoelectrophoresis studies with antibodies that inhibit ristocetin-induced platelet aggregation. Br J Haematol. 1981 Jun;48(2):305–318. doi: 10.1111/j.1365-2141.1981.tb08464.x. [DOI] [PubMed] [Google Scholar]
  2. Apitz-Castro R., Michelena V., Sorribes V., Rodríguez P. Metabolic isotopic labelling of plasma membrane glycoproteins from normal, Glanzmann's and Bernard-Soulier platelets. Thromb Res. 1979;16(5-6):715–725. doi: 10.1016/0049-3848(79)90215-9. [DOI] [PubMed] [Google Scholar]
  3. BERNARD J., SOULIER J. P. Sur une nouvelle variété de dystrophie thrombocytairehémoŗragipare congénitale. Sem Hop. 1948 Dec 28;24(Spec No):3217–3223. [PubMed] [Google Scholar]
  4. Berndt M. C., Phillips D. R. Purification and preliminary physicochemical characterization of human platelet membrane glycoprotein V. J Biol Chem. 1981 Jan 10;256(1):59–65. [PubMed] [Google Scholar]
  5. Bithell T. C., Parekh S. J., Strong R. R. Platelet-function studies in the Bernard-Soulier syndrome. Ann N Y Acad Sci. 1972 Oct 27;201:145–160. doi: 10.1111/j.1749-6632.1972.tb16296.x. [DOI] [PubMed] [Google Scholar]
  6. Clemetson K. J., Capitanio A., Pareti F. I., McGregor J. L., Lüscher E. F. Additional platelet membrane glycoprotein abnormalities in Glanzmann's thrombasthenia: A comparison with normals by high resolution two-dimensional polyacrylamide gel electrophoresis. Thromb Res. 1980 Jun 15;18(6):797–806. doi: 10.1016/0049-3848(80)90202-9. [DOI] [PubMed] [Google Scholar]
  7. Frojmovic M. M., Milton J. G., Caen J. P., Tobelem G. Platelets from "giant platelet syndrome (BSS)" are discocytes and normal sized. J Lab Clin Med. 1978 Jan;91(1):109–116. [PubMed] [Google Scholar]
  8. Ganguly P. Binding of thrombin to functionally defective platelets: a hypothesis on the nature of the thrombin receptor. Br J Haematol. 1977 Sep;37(1):47–51. [PubMed] [Google Scholar]
  9. Gröttum K. A., Solum N. O. Congenital thrombocytopenia with giant platelets: a defect in the platelet membrane. Br J Haematol. 1969 Mar;16(3):277–290. doi: 10.1111/j.1365-2141.1969.tb00402.x. [DOI] [PubMed] [Google Scholar]
  10. Hagen I., Nurden A., Bjerrum O. J., Solum N. O., Caen J. Immunochemical evidence for protein abnormalities in platelets from patients with Glanzmann's thrombasthenia and Bernard-Soulier syndrome. J Clin Invest. 1980 Mar;65(3):722–731. doi: 10.1172/JCI109719. [DOI] [PMC free article] [PubMed] [Google Scholar]
  11. Hagen I., Solum N. O. Further studies on the protein composition and surface structure of normal platelets and platelets from patients with Glanzmann's thrombasthenia and Bernard-Soulier syndrome. Thromb Res. 1978 Nov;13(5):845–855. doi: 10.1016/0049-3848(78)90189-5. [DOI] [PubMed] [Google Scholar]
  12. Howard M. A., Hutton R. A., Hardisty R. M. Hereditary giant platelet syndrome: a disorder of a new aspect of platelet function. Br Med J. 1973 Jun 9;2(5866):586–588. doi: 10.1136/bmj.2.5866.586. [DOI] [PMC free article] [PubMed] [Google Scholar]
  13. Jamieson G. A., Okumura T., Fishback B., Johnson M. M., Egan J. J., Weiss H. J. Platelet membrane glycoproteins in thrombasthenia, Bernard-Soulier syndrome, and storage pool disease. J Lab Clin Med. 1979 Apr;93(4):652–660. [PubMed] [Google Scholar]
  14. Jamieson G. A., Okumura T. Reduced thrombin binding and aggregation in Bernard-Soulier platelets. J Clin Invest. 1978 Mar;61(3):861–864. doi: 10.1172/JCI109000. [DOI] [PMC free article] [PubMed] [Google Scholar]
  15. Jenkins C. S., Ali-Briggs E. F., Clemetson K. J. Antibodies against platelet membrane glycoproteins. II. Influence on ADP- and collagen-induced platelet aggregation, crossed immunoelectrophoresis studies and relevance to Glanzmann's thrombasthenia. Br J Haematol. 1981 Nov;49(3):439–447. doi: 10.1111/j.1365-2141.1981.tb07247.x. [DOI] [PubMed] [Google Scholar]
  16. Jenkins C. S., Ali-Briggs E. F., Zonneveld G. T., Sturk A., Clemetson K. J. Human blood platelet membrane glycoproteins. Resolution in different polyacrylamide gel electrophoretic systems. Thromb Haemost. 1980 Feb 29;42(5):1490–1502. [PubMed] [Google Scholar]
  17. Jenkins C. S., Phillips D. R., Clemetson K. J., Meyer D., Larrieu M. J., Lüscher E. F. Platelet membrane glycoproteins implicated in ristocetin-induced aggregation. Studies of the proteins on platelets from patients with Bernard-Soulier syndrome and von Willebrand's disease. J Clin Invest. 1976 Jan;57(1):112–124. doi: 10.1172/JCI108251. [DOI] [PMC free article] [PubMed] [Google Scholar]
  18. Kessler S. W. Rapid isolation of antigens from cells with a staphylococcal protein A-antibody adsorbent: parameters of the interaction of antibody-antigen complexes with protein A. J Immunol. 1975 Dec;115(6):1617–1624. [PubMed] [Google Scholar]
  19. Laskey R. A., Mills A. D. Enhanced autoradiographic detection of 32P and 125I using intensifying screens and hypersensitized film. FEBS Lett. 1977 Oct 15;82(2):314–316. doi: 10.1016/0014-5793(77)80609-1. [DOI] [PubMed] [Google Scholar]
  20. Laskey R. A., Mills A. D. Quantitative film detection of 3H and 14C in polyacrylamide gels by fluorography. Eur J Biochem. 1975 Aug 15;56(2):335–341. doi: 10.1111/j.1432-1033.1975.tb02238.x. [DOI] [PubMed] [Google Scholar]
  21. McGregor J. L., Clemetson K. J., James E., Luscher E. F., Dechavanne M. A comparison of the major platelet membrane glycoproteins from Bernard-Soulier syndrome with normals after radiolabelling of sialic acid or terminal galactose/N-acetylgalactosamine residues. Thromb Res. 1980 Mar 1;17(5):713–718. doi: 10.1016/0049-3848(80)90374-6. [DOI] [PubMed] [Google Scholar]
  22. Mosher D. F., Vaheri A., Choate J. J., Gahmberg C. G. Action of thrombin on surface glycoproteins of human platelets. Blood. 1979 Mar;53(3):437–445. [PubMed] [Google Scholar]
  23. Nurden A. T., Caen J. P. Specific roles for platelet surface glycoproteins in platelet function. Nature. 1975 Jun 26;255(5511):720–722. doi: 10.1038/255720a0. [DOI] [PubMed] [Google Scholar]
  24. Okumura T., Hasitz M., Jamieson G. A. Platelet glycocalicin. Interaction with thrombin and role as thrombin receptor of the platelet surface. J Biol Chem. 1978 May 25;253(10):3435–3443. [PubMed] [Google Scholar]
  25. Phillips D. R., Agin P. P. Platelet plasma membrane glycoproteins. Evidence for the presence of nonequivalent disulfide bonds using nonreduced-reduced two-dimensional gel electrophoresis. J Biol Chem. 1977 Mar 25;252(6):2121–2126. [PubMed] [Google Scholar]
  26. Phillips D. R., Agin P. P. Platelet plasma membrane glycoproteins. Identification of a proteolytic substrate for thrombin. Biochem Biophys Res Commun. 1977 Apr 25;75(4):940–947. doi: 10.1016/0006-291x(77)91473-5. [DOI] [PubMed] [Google Scholar]
  27. Shulman S., Karpatkin S. Crossed immunoelectrophoresis of human platelet membranes. Diminished major antigen in Glanzmann's thrombasthenia and Bernard-Soulier syndrome. J Biol Chem. 1980 May 10;255(9):4320–4327. [PubMed] [Google Scholar]
  28. Sixma J. J., Schiphorst M. E. Identification of ectoproteins of human platelets. Combination of radioactive labelling and two-dimensional electrophoresis. Biochim Biophys Acta. 1980 Dec 2;603(1):70–83. doi: 10.1016/0005-2736(80)90392-2. [DOI] [PubMed] [Google Scholar]
  29. Tam S. W., Fenton J. W., 2nd, Detwiler T. C. Platelet thrombin receptors. Binding of alpha-thrombin is coupled to signal generation by a chymotrypsin-sensitive mechanism. J Biol Chem. 1980 Jul 25;255(14):6626–6632. [PubMed] [Google Scholar]
  30. Tollefsen D. M., Feagler J. R., Majerus P. W. The binding of thrombin to the surface of human platelets. J Biol Chem. 1974 Apr 25;249(8):2646–2651. [PubMed] [Google Scholar]
  31. Weiss H. J., Tschopp T. B., Baumgartner H. R., Sussman I. I., Johnson M. M., Egan J. J. Decreased adhesion of giant (Bernard-Soulier) platelets to subendothelium. Further implications on the role of the von Willebrand factor in hemostasis. Am J Med. 1974 Dec;57(6):920–925. doi: 10.1016/0002-9343(74)90170-3. [DOI] [PubMed] [Google Scholar]

Articles from Journal of Clinical Investigation are provided here courtesy of American Society for Clinical Investigation

RESOURCES