Normal synthesis and expression of endothelial IIb/IIIa in Glanzmann's thrombasthenia

Blood. 1987 Mar;69(3):809-12.

Abstract

Glanzmann's thrombasthenia is a bleeding disorder, inherited in an autosomal recessive way and characterized by an absence or deficiency of the platelet glycoprotein (GP) IIb/IIIa complex. Recently, we and others demonstrated that cultured human umbilical vein endothelial cells synthesized a membrane protein complex similar to the platelet GP IIb/IIIa complex. In this article, we demonstrate that endothelial cells isolated from the umbilical vein of a newborn with Glanzmann's thrombasthenia, as compared with normal endothelial cells, show no difference in their ability to synthesize and express this GP IIb/IIIa complex. Our results indicate that Glanzmann's thrombasthenia is not accompanied by an "endotheliopathy."

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Blood Platelet Disorders / metabolism*
  • Blood Platelets / metabolism
  • Cells, Cultured
  • Endothelium / metabolism*
  • Fluorescent Antibody Technique
  • Humans
  • Immunoelectrophoresis, Two-Dimensional
  • Infant, Newborn
  • Male
  • Platelet Membrane Glycoproteins / biosynthesis*
  • Platelet Membrane Glycoproteins / immunology
  • Platelet Membrane Glycoproteins / isolation & purification
  • Thrombasthenia / genetics
  • Thrombasthenia / metabolism*
  • Thrombasthenia / pathology
  • Umbilical Veins / pathology

Substances

  • Platelet Membrane Glycoproteins