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The Bernard-Soulier platelet: I. Correlation of adhesion defects with abnormalities of surface glycoproteins
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.
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.