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CD Markers

CD42c


Reviewers: Charanjeet Singh, M.D., University of Minnesota (see Reviewers page)
Revised: 23 December 2010, last major update December 2010
Copyright: (c) 2002-2010, PathologyOutlines.com, Inc.

General
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● CD42a-d complex is receptor for von Willebrand factor and thrombin

Terminology
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● Also called platelet GPIb beta, glycoprotein Ib-beta

Physiology
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● CD42a-d complex is receptor for von Willebrand factor and thrombin
● GPIb alpha (CD42b) and GPIb beta (CD42c) form main part of VWF receptor
● CD42c also amplifies the platelet response to thrombin during platelet activation where thrombin is involved
● Platelet activation apparently involves disruption of the macromolecular complex of GP-Ib with the platelet glycoprotein IX (GP-IX) and dissociation of GP-Ib from the actin-binding protein

Drawings
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CD42a-d complex

Clinical features
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● Mutations are associated with Bernard-Soulier syndrome (giant platelet syndrome, OMIM 231200, Int J Hematol 2002;76:319), a bleeding disorder with thrombocytopenia, prolonged bleeding time and giant platelets
● Reduced levels are associated with bleeding disorders in AML patients (Saudi Med J 2005;26:1095)

Case reports
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● Mutation in Bernard-Soulier syndrome patient (J Thromb Haemost 2006;4:217)

Uses for pathologists
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● No significant clinical use by pathologists

Positive stains - normal
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● Platelets and megakaryocytes; also endothelium, heart and brain (J Clin Invest 1994;93:2417)

Additional references
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MIM 138720, Thromb Haemost 2001;86:1238

End of CD Markers > CD42c


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