Glycoprotein Ib
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. Glycoprotein Ibα (GPIbα) is the major ligand-binding subunit of the GPIb-V-IX complex. GPIbα is heavily glycosylated.[2]
It is deficient in the Bernard–Soulier syndrome. A gain-of-function mutation causes platelet-type von Willebrand disease.[3]
Autoantibodies against Ib/IX can be produced in immune thrombocytopenic purpura.[4]
Components include GP1BA and GP1BB.
It complexes with Glycoprotein IX.
References
- ^ Bode AP, Read MS, Reddick RL (February 1999). "Activation and adherence of lyophilized human platelets on canine vessel strips in the Baumgartner perfusion chamber". The Journal of Laboratory and Clinical Medicine. 133 (2): 200–211. doi:10.1016/S0022-2143(99)90013-6. PMID 9989772.
- ^ Hollenhorst MA, Tiemeyer KH, Mahoney KE, Aoki K, Ishihara M, Lowery SC, et al. (April 2023). "Comprehensive analysis of platelet glycoprotein Ibα ectodomain glycosylation". Journal of Thrombosis and Haemostasis. 21 (4): 995–1009. doi:10.1016/j.jtha.2023.01.009. PMC 10065957. PMID 36740532.
- ^ McPherson RA, Pincus MR (2007). Henry's Clinical Diagnosis and Management by Laboratory Methods (21st ed.). Philadelphia, Pa: Saunders Elsevier. pp. 760–762. ISBN 978-1-4160-0287-1.
- ^ McMillan R (October 2007). "The pathogenesis of chronic immune thrombocytopenic purpura". Seminars in Hematology. 44 (4 Suppl 5): S3 – S11. doi:10.1053/j.seminhematol.2007.11.002. PMID 18096470.
External links
- Glycoprotein+Ib at the U.S. National Library of Medicine Medical Subject Headings (MeSH)