Document Detail


The organizing principle of the platelet glycoprotein Ib-IX-V complex.
MedLine Citation:
PMID:  23336709     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
The glycoprotein (GP)Ib-IX-V complex is the platelet receptor for von Willebrand factor and many other molecules that are critically involved in hemostasis and thrombosis. The lack of functional GPIb-IX-V complexes on the platelet surface is the cause of Bernard-Soulier syndrome, a rare hereditary bleeding disorder that is also associated with macrothrombocytopenia. GPIb-IX-V contains GPIbα, GPIbβ, GPIX and GPV subunits, all of which are type I transmembrane proteins containing leucine-rich repeat domains. Although all of the subunits were identified decades ago, not until recently did the mechanism of complex assembly begin to emerge from a systematic characterization of inter-subunit interactions. This review summarizes the forces driving the assembly of GPIb-IX-V, discusses their implications for the pathogenesis of Bernard-Soulier syndrome, and identifies questions that remain about the structure and organization of GPIb-IX-V.
Authors:
R Li; J Emsley
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Publication Detail:
Type:  Journal Article; Research Support, N.I.H., Extramural; Review    
Journal Detail:
Title:  Journal of thrombosis and haemostasis : JTH     Volume:  11     ISSN:  1538-7836     ISO Abbreviation:  J. Thromb. Haemost.     Publication Date:  2013 Apr 
Date Detail:
Created Date:  2013-04-12     Completed Date:  2013-09-30     Revised Date:  2014-04-02    
Medline Journal Info:
Nlm Unique ID:  101170508     Medline TA:  J Thromb Haemost     Country:  England    
Other Details:
Languages:  eng     Pagination:  605-14     Citation Subset:  IM    
Copyright Information:
© 2013 International Society on Thrombosis and Haemostasis.
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MeSH Terms
Descriptor/Qualifier:
Humans
Models, Molecular
Mutation
Platelet Glycoprotein GPIb-IX Complex / chemistry,  metabolism,  physiology*
Protein Binding
Protein Conformation
Grant Support
ID/Acronym/Agency:
HL082808/HL/NHLBI NIH HHS; HL097226/HL/NHLBI NIH HHS; R01 HL082808/HL/NHLBI NIH HHS; R21 HL097226/HL/NHLBI NIH HHS
Chemical
Reg. No./Substance:
0/Platelet Glycoprotein GPIb-IX Complex
Comments/Corrections

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