Comparison of normal and Bernard-Soulier platelet membrane glycoproteins: isoelectric characteristics and surface radiolabel.
The protein and glycoprotein compositions of platelets from normal individuals, patients with B-S disease, and subjects heterozygous for B-S were determined by two-dimensional gel electrophoretic analysis of platelets that had been radiolabeled on the surface by lactoperoxidase-catalyzed iodination or by the periodate-borohydride reaction. In normal platelets, five PAS-stained glycoproteins were found in the GP I region of the two-dimensional gels. B-S platelets contained four of the glycoproteins of the GP I complex but were deficient in one glycoprotein (Mr = 139,000 and pI 6.36) that, in normal platelets, was not stained by Coomassie blue but had a high affinity for both PAS stain and alcian blue stain and was heavily labeled on the surface of platelets by the periodate-borohydride reaction but only faintly with radioiodine. Platelets from three heterozygous B-S individuals did not differ from normal controls by these techniques. These results further define the glycoprotein composition of normal platelets and show a specific deficiency of a portion of the GP I complex in B-S platelets.
D001345:Autoradiography; D001778:Blood Coagulation Disorders / Q000097:blood* / Q000235:genetics; D001792:Blood Platelets / Q000032:analysis*; D002462:Cell Membrane / Q000032:analysis; D004591:Electrophoresis, Polyacrylamide Gel / Q000379:methods; D006023:Glycoproteins / Q000097:blood* / Q000302:isolation & purification; D006579:Heterozygote; D006801:Humans; D007525:Isoelectric Focusing; D007526:Isoelectric Point; D008970:Molecular Weight; D013194:Staining and Labeling
D006023:Glycoproteins