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Decreased content and surface expression of alpha-granule membrane protein GMP-140 in one of two types of platelet alpha delta storage pool deficiency.

Abstract
To determine whether alpha-granule membranes are present in platelets of patients with storage pool deficiencies of both alpha and dense granules (alpha delta-SPD), we examined the content and surface expression of the alpha-granule membrane protein GMP-140 in one patient (J.C.) with a severe alpha-granule deficiency and in three members of a family (family C) with milder alpha-granule deficiencies. Surface expression of GMP-140 in stimulated platelets, assessed by flow cytometric measurements of the binding of two anti-GMP-140 monoclonal antibodies, was 24-38% of normal values in platelets from patient J.C., vs. 60-95% of normal values in family C. Total platelet content of GMP-140, determined in platelet lysates by antigen-capture ELISA, was 49% of normal in patient J.C., but normal in the members of family C. Platelets of patient J.C. were found to be heterogeneous with respect to GMP-140 content and surface expression by both flow cytometry and immunogold electron microscopy. Approximately 80% of her platelets expressed little or no GMP-140 after stimulation, whereas the remaining 20% expressed normal amounts of GMP-140 and showed extensive immunogold labeling of typical alpha-granules and clear vacuoles. No such heterogeneity was found in platelets from family C. These findings in the severe alpha delta-SPD patient are in clear contrast to the observations of normal GMP-140 content in the three other alpha delta-SPD patients, and in patients with the gray platelet syndrome, reported previously by others. These results illustrate the phenotypic heterogeneity of alpha-granule deficiencies in human platelets, and suggest that a defect in granule formation in the megakaryocytes may account for the alpha-granule defect in at least one form of alpha delta-SPD.
AuthorsB Lages, S J Shattil, D F Bainton, H J Weiss
JournalThe Journal of clinical investigation (J Clin Invest) Vol. 87 Issue 3 Pg. 919-29 (Mar 1991) ISSN: 0021-9738 [Print] United States
PMID1705568 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't, Research Support, U.S. Gov't, P.H.S.)
Chemical References
  • Antibodies, Monoclonal
  • Antigens, Surface
  • Immunoglobulin G
  • P-Selectin
  • Platelet Membrane Glycoproteins
Topics
  • Adult
  • Antibodies, Monoclonal
  • Antigens, Surface (analysis)
  • Blood Platelets (metabolism, ultrastructure)
  • Cell Membrane (metabolism)
  • Female
  • Flow Cytometry
  • Humans
  • Immunoglobulin G (metabolism)
  • Immunohistochemistry
  • Middle Aged
  • P-Selectin
  • Platelet Activation
  • Platelet Membrane Glycoproteins (metabolism)
  • Platelet Storage Pool Deficiency (metabolism)

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