CD42

CD42 is a protein that in humans is encoded by the GP1BA gene. It is a platelet membrane glycoprotein that plays a crucial role in the process of blood coagulation and the formation of blood clots. CD42 is also known as Glycoprotein Ib (GPIb), which is a platelet surface membrane glycoprotein composed of two polypeptide chains.

Structure

CD42 is a complex of three proteins, GP1BA, GP1BB, and GPIX, that are all necessary for the formation of a functional receptor. The GP1BA gene encodes the heavy chain of this complex, which contains the von Willebrand factor (vWF) binding site. Mutations in the GP1BA gene are associated with Bernard-Soulier syndrome, Velocardiofacial syndrome, and platelet-type von Willebrand disease.

Function

CD42 functions as a receptor for von Willebrand factor, which initiates platelet adhesion to vascular surfaces in the arterial circulation under high shear stress. The adhesion of platelets to injured vascular surfaces is critical for the initiation of blood clot formation, and thus, CD42 plays a crucial role in hemostasis and thrombosis.

Clinical significance

Defects in CD42 are associated with Bernard-Soulier syndrome, a rare disorder characterized by thrombocytopenia, giant platelets, and prolonged bleeding time. Mutations in the GP1BA gene can also cause platelet-type von Willebrand disease, a bleeding disorder characterized by abnormal platelet function.

See also

• Glycoprotein Ib
• Bernard-Soulier syndrome
• Velocardiofacial syndrome
• Platelet-type von Willebrand disease

References

CD42 Resources
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