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Glycoprotein Ib

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Glycoprotein Ib (GPIb), also known as CD42,[1] is a component of the GPIb-V-IX complex on platelets. The GPIb-V-IX complex binds von Willebrand factor, allowing platelet adhesion and platelet plug formation at sites of vascular injury.

It is deficient in the Bernard–Soulier syndrome. A gain-of-function mutation causes platelet-type von Willebrand disease.[2]

Autoantibodies against Ib/IX can be produced in immune thrombocytopenic purpura.[3]

Components include GP1BA and GP1BB.

It complexes with Glycoprotein IX.

References

  1. ^ Bode AP, Read MS, Reddick RL (February 1999). "Activation and adherence of lyophilized human platelets on canine vessel strips in the Baumgartner perfusion chamber". J. Lab. Clin. Med. 133 (2): 200–11. doi:10.1016/S0022-2143(99)90013-6. PMID 9989772.
  2. ^ McPherson & Pincus: Henry's Clinical Diagnosis and Management by Laboratory Methods, 21st ed., pp. 760–2 (W. B. Saunders, 2006).
  3. ^ McMillan R (October 2007). "The pathogenesis of chronic immune thrombocytopenic purpura". Semin. Hematol. 44 (4 Suppl 5): S3–S11. doi:10.1053/j.seminhematol.2007.11.002. PMID 18096470.