
Description
CD42a, also known as glycoprotein 9 (GPIX), composes together with GPIb alpha, GPIb beta and GPV the GPIb-IX-V receptor complex critical in the process of platelet-rich thrombus formation by tethering the platelet to a thrombogenic surface. CD42b binds to von Willebrand factor (VWF) exposed at a site of vascular injury, as well as to thrombin, coagulation factors XI and XII, high molecular wight kininogen, TSP-1, integrin Mac-1 and P-selectin. Defects in the gene encoding CD42a are a cause of Bernard-Soulier syndrome, also known as giant platelet disease. These patients have unusually large platelets and have a clinical bleeding tendency.
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