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Alpha-granule pool of glycoprotein IIb-IIIa in normal and pathologic
platelets and megakaryocytes
EM Cramer, GF Savidge, W Vainchenker, MC Berndt, D Pidard, JP Caen, JM Masse and J Breton-Gorius
Department d'Angio-hematologie and INSERM U.150, Hopital Lariboisiere,
Paris, France.
Using an immunogold staining technique and electron microscopy, we
investigated the localization of the alpha-granule pool of glycoprotein
(GP) IIb-IIIa in normal platelets and maturing megakaryocytes (MK), in
pathologic platelets from a patient with type I Glanzmann's thrombasthenia
(GT), and from three patients with the gray platelet syndrome (GPS). In
normal resting platelets, GPIIb-IIIa was observed on the plasmatic side of
the plasma membrane, the open canicular system (OCS) membranes, and along
the internal face of the alpha-granule membrane. This location was found
with three monospecific polyclonal antibodies: one anti-GPIIb-IIIa
antibody, the second specific for GPIIb, and the third specific for GPIIIa.
After thrombin stimulation, the alpha-granule labeling disappeared whereas
membrane labeling increased. Platelets from GT did not display labeling on
plasma membranes, OCS membranes, or alpha-granule membranes. Platelets from
the three patients with GPS displayed intense labeling of the plasma
membrane and the OCS membrane, as well as the abnormal small alpha-
granules and along the inside of large vacuoles (which contain the granule
membrane protein [GMP]-140). In cultured immature MK from normal
progenitors, both peptide components of GPIIb-IIIa appeared in the Golgi
saccules and vesicles, and in the small precursors of alpha- granules,
labeling both their membranes and their matrix. It was then observed only
on the membrane of the mature MK alpha-granules, although labeling was less
consistent than on the platelet granules. The MK plasma membrane and
demarcation membrane system also displayed GPIIb- IIIa labeling. In
conclusion, this study demonstrates that GPIIb-IIIa is present on the
internal face of the alpha-granule membranes of platelets (where it appears
early during MK maturation) as well as in the abnormal alpha-granules of
gray platelets; it is absent from GT type I platelets.
Volume 75,
Issue 6,
pp. 1220-1227,
03/15/1990
Copyright © 1990 by The American Society of Hematology
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