RGD Reference Report - Fibrin polymerization is crucial for thrombin generation in platelet-rich plasma in a VWF-GPIb-dependent process, defective in Bernard-Soulier syndrome. - Rat Genome Database

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Fibrin polymerization is crucial for thrombin generation in platelet-rich plasma in a VWF-GPIb-dependent process, defective in Bernard-Soulier syndrome.

Authors: Beguin, S  Keularts, I  Al Dieri, R  Bellucci, S  Caen, J  Hemker, HC 
Citation: Beguin S, etal., J Thromb Haemost. 2004 Jan;2(1):170-6.
RGD ID: 1580643
Pubmed: PMID:14717981   (View Abstract at PubMed)

Defective prothrombin consumption has been reported in the proband case of Bernard-Soulier syndrome (BSS). There is no consensus, however, on whether the formation of platelet procoagulant activity (PPA) is impaired in BSS and, if so, whether this is due to the lack of GPIb-V-IX-dependent binding of thrombin or of von Willebrand factor (VWF). We show thrombin generation (TG) in platelet-rich plasma of BSS (BSS-PRP) to be defective provided that fibrin remains present in the reaction mixture and that the giant platelets are not damaged by frequent subsampling. In BSS-PRP addition of (thrombin-free) fibrin did not increase TG as in normal PRP, supporting our previous hypothesis that the interaction of fibrin, VWF and GPIb triggers PPA development. Fibrin formed during the lag phase of TG by a snake venom enzyme which only removed fibrinopeptide A induced an immediate burst of TG, that was inhibited by a monoclonal antibody against GPIb (6D1) that abolishes ristocetin-induced binding of VWF to platelets. Inversely, inhibition of polymerization decreased TG and the residual activity was insensitive to 6D1. We conclude that polymerizing fibrin interacts with VWF so as to activate GPIb.

RGD Manual Disease Annotations    Click to see Annotation Detail View
TermQualifierEvidenceWithReferenceNotesSourceOriginal Reference(s)
Bernard-Soulier syndrome  IDA 1580643 RGD 
Bernard-Soulier syndrome  ISOVWF (Homo sapiens)1580643; 1580643 RGD 

Objects Annotated

Genes (Rattus norvegicus)
Vwf  (von Willebrand factor)

Genes (Mus musculus)
Vwf  (Von Willebrand factor)

Genes (Homo sapiens)
VWF  (von Willebrand factor)


Additional Information