GP-IX/CD42a

Glycoprotein IX (CD42a) is the smallest subunit of the GPIb-IX-V receptor complex, the platelet surface machinery that captures von Willebrand factor under high shear stress. Encoded by GP9, the chain associates non-covalently with GPIb alpha, GPIb beta and GPV, stabilizing the complex and contributing to its surface expression. Although GP-IX lacks a direct ligand-binding site, mutations in GP9 produce Bernard-Soulier syndrome, the macrothrombocytopenia caused by failure of the whole complex to reach the membrane. Anti-GP-IX antibodies have also been reported in immune-mediated thrombocytopenia. Recombinant GP-IX serves mainly as a component for reconstituting functional vWF-binding complexes.