GPIb-IX-V

This transmembrane glycoprotein complex is composed of four subunits: GPIbα, GPIbβ, GPV and GPIX. Each of them has a variable number of leucine-rich repeats. GPIbα and GPIbβ are linked by disulfide bridges, while the GPV and GPIX associate non-covalently with the complex. GPIbα subunit bears the binding site for von Willebrand factor (vWF), α-thrombin, leukocyte integrin αMβ2 and P-selectin. The binding between GPIbα and vWF mediates the capture of platelets to the injured vascular wall. The deficiency in glycoprotein Ib-IX-V complex synthesis leads to Bernard-Soulier syndrome.[1]

References

  1. ↑ LEPAGE, A., M. LEBOEUF, J.P. CAZENAVE, C. SALLE, F. LANZA a G. UZAN. The αIIbβ3 integrin and GPIb-V-IX complex identify distinct stages in the maturation of CD34+cord blood cells to megakaryocytes. Blood. [cit. 2012-06-12]. ISSN 1528-0020. http://bloodjournal.hematologylibrary.org/content/96/13/4169.full.pdf


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