Expression of the alloantigen Zwa (or P1A1) on human vascular smooth muscle
cells and foreskin fibroblasts: a study on normal individuals and a patient
with Glanzmann's thrombasthenia
JC Giltay, HJ Brinkman, AE von dem Borne and JA van Mourik
Central Laboratory, The Netherlands Red Cross Blood Transfusion Service,
Amsterdam.
The cytoadhesin family consists of platelet glycoprotein (GP) IIb-IIIa and
the endothelial vitronectin receptor. The beta subunit (GP IIIa) of these
complexes expresses the alloantigen Zwa (or PIA1). This alloantigen is not
expressed by members of other integrin subfamilies. By using
immunoprecipitation and immunoblot techniques, we found that the beta
subunit of a heterodimer, expressed by cultured human arterial smooth
muscle cells and cultured foreskin fibroblasts, carries the Zwa antigenic
determinant. Furthermore, the mobilities of the alpha and beta subunits of
these two heterodimers are indistinguishable from those of the alpha and
beta subunits of the endothelial vitronectin receptor. Therefore, we
propose that the smooth muscle cell and fibroblast heterodimer are members
of the cytoadhesin family. In Glanzmann's thrombasthenia, platelet GP
IIb-IIIa is absent or severely reduced. Previously, we showed that
endothelial cells from a thrombasthenic patient normally synthesize and
express a GP IIb-IIIa- related molecule (the vitronectin receptor). Here we
show that arterial smooth muscle cells, obtained from the same patient,
express a surface molecule indistinguishable from the endothelial
vitronectin receptor. We also demonstrate that both the endothelial and the
smooth muscle cell GP IIIa-related molecule in this Glanzmann patient
express Zwa. Our data indicate that (a) GP IIb-IIIa-related molecules on
cell types other than platelets and endothelial cells can express Zwa in
vitro, and (b) patients with Glanzmann's disease can express the Zwa
antigen. This study substantiates our view that the defect in Glanzmann's
disease is restricted to the megakaryocytes/platelets.
Volume 74,
Issue 3,
pp. 965-970,
08/15/1989
Copyright © 1989 by The American Society of Hematology