The GPIb-IX complex on platelets functions as a mechanosensor and thrombin receptor that regulates platelet biogenesis, clearance, immune surveillance, and liver cancer development.
The glycoprotein (GP) Ib-IX complex is a platelet receptor that mediates the initial interaction with subendothelial von Willebrand factor (VWF) causing platelet arrest at sites of vascular injury even under conditions of high shear. GPIb-IX dysfunction or deficiency is the reason for the rare but severe Bernard-Soulier syndrome (BSS), a congenital bleeding disorder. Although knowledge on GPIb-IX structure, its basic functions, ligands, and intracellular signaling cascades have been well established, several advances in GPIb-IX biology have been made in the recent years. Thus, two mechanosensitive domains and a trigger sequence in GPIb were characterized and its role as a thrombin receptor was deciphered. Furthermore, it became clear that GPIb-IX is involved in the regulation of platelet production, clearance and thrombopoietin secretion. GPIb is deemed to contribute to liver cancer development and metastasis. This review recapitulates these novel findings highlighting GPIb-IX in its multiple functions as a key for immune regulation, host defense, and liver cancer development.
Bendas et al. (Sat,) conducted a review in Bernard-Soulier syndrome, liver cancer. The GPIb-IX complex on platelets functions as a mechanosensor and thrombin receptor that regulates platelet biogenesis, clearance, immune surveillance, and liver cancer development.