Normal synthesis and expression of endothelial IIb/IIIa in Glanzmann's thrombasthenia.
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."
D001791:Blood Platelet Disorders / Q000378:metabolism*; D001792:Blood Platelets / Q000378:metabolism; D002478:Cells, Cultured; D004727:Endothelium / Q000378:metabolism*; D005455:Fluorescent Antibody Technique; D006801:Humans; D007123:Immunoelectrophoresis, Two-Dimensional; D007231:Infant, Newborn; D008297:Male; D010980:Platelet Membrane Glycoproteins / Q000096:biosynthesis* / Q000276:immunology / Q000302:isolation & purification; D013915:Thrombasthenia / Q000235:genetics / Q000378:metabolism* / Q000473:pathology; D014471:Umbilical Veins / Q000473:pathology
D010980:Platelet Membrane Glycoproteins