Article
Bernard-Soulier syndrome: an update.
Seminars in thrombosis and hemostasis - 1 Sept 2013
Andrews Robert K, Berndt Michael C
Abstract excerpt
Bernard-Soulier syndrome (BSS) is a rare inherited platelet bleeding disorder characterized by low platelet count and abnormally large platelets (macrothrombocytopenia). Platelets from BSS patients are typically defective in surface expression of glycoprotein (GP)Ib-IX-V, a platelet-specific adhesion-signaling complex, composed of GPIbα disulfide linked to GPIbβ, and noncovalently associated with GPIX and GPV....
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