title: The Bernard-Soulier platelet: I. Correlation of adhesion defects with abnormalities of surface glycoproteins.
abstract: Bernard-Soulier disease, an inherited human platelet disorder, is characterized by decreased adhesion of platelets to the subendothelium. A review of the clinical features, laboratory diagnostic features and glycoprotein abnormalities is presented. The glycoprotein composition of platelets from patients with Bernard-Soulier disease was compared with normal using 3H-labeling of surface glycoproteins followed by two-dimensional gel electrophoresis. Decreased amounts of 3H-labeling of glycoprotein IIa as well as absence of the specific surface glycoprotein Ib was observed in Bernard-Soulier platelets. Studies of Bernard-Soulier platelets affords an opportunity to understand the mechanisms of platelet adhesion responses in the interactions of platelets with blood vessels.
mesh_terms: D001791:Blood Platelet Disorders / Q000097:blood*; D001792:Blood Platelets / Q000166:cytology*; D002448:Cell Adhesion; D004591:Electrophoresis, Polyacrylamide Gel; D006023:Glycoproteins / Q000097:blood; D006801:Humans; D010974:Platelet Aggregation
chemical_list: D006023:Glycoproteins