RGD Reference Report - Decreased thrombotic tendency in mouse models of the Bernard-Soulier syndrome. - Rat Genome Database

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Decreased thrombotic tendency in mouse models of the Bernard-Soulier syndrome.

Authors: Strassel, C  Nonne, C  Eckly, A  David, T  Leon, C  Freund, M  Cazenave, JP  Gachet, C  Lanza, F 
Citation: Strassel C, etal., Arterioscler Thromb Vasc Biol. 2007 Jan;27(1):241-7. Epub 2006 Nov 9.
RGD ID: 11040529
Pubmed: PMID:17095718   (View Abstract at PubMed)
DOI: DOI:10.1161/01.ATV.0000251992.47053.75   (Journal Full-text)

OBJECTIVE: The platelet glycoprotein (GP)Ib-V-IX complex is a receptor required for normal hemostasis deficient in the Bernard-Soulier bleeding disorder. To evaluate the consequences of GPIb-V-IX deficiency in thrombosis we generated mouse models of the disease by targeting the GPIb beta subunit. METHODS AND RESULTS: Complete deletion (GPIb beta-/-) or an intracellular truncation (GPIb beta deltaIC-/-) reproduced typical and variant forms of Bernard-Soulier, with absent and partial (20%) expression of the complex on the platelet surface. Both strains exhibited thrombocytopenia and enlarged platelets with abnormal microtubular structures but normal granule composition. They exhibited prolonged tail bleeding times, which were less pronounced in GPIb beta deltaIC-/-. Decreased thrombus formation was observed after blood perfusion over a collagen coated surface at high shear. Resistance to vascular occlusion and an abnormal thrombus composition were observed in a model of FeCl3-induced lesion of carotid arteries. In a model of laser-induced lesion of mesenteric arterioles, thrombosis was strongly reduced in GPIb beta-/- mice, while a more modest effect was observed in GPIb beta deltaIC-/- animals. Finally, the two strains were protected against death in a model of systemic thromboembolism. CONCLUSIONS: This study provides in vivo evidence of a decreased thrombotic tendency linked to defective platelet GPIb-V-IX in mouse models of Bernard-Soulier syndrome.



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Object Symbol
Species
Term
Qualifier
Evidence
With
Notes
Source
Original Reference(s)
GP1BBHumanBernard-Soulier syndrome  ISORGD:732124 RGD 
Gp1bbRatBernard-Soulier syndrome  ISORGD:732124 RGD 
Gp1bbMouseBernard-Soulier syndrome  IMP  RGD 
GP1BBHumancarotid artery thrombosis  ISORGD:732124 RGD 
Gp1bbRatcarotid artery thrombosis  ISORGD:732124 RGD 
Gp1bbMousecarotid artery thrombosis  IMP  RGD 
GP1BBHumanMesenteric Ischemia  ISORGD:732124 RGD 
Gp1bbRatMesenteric Ischemia  ISORGD:732124 RGD 
Gp1bbMouseMesenteric Ischemia  IMP  RGD 
GP1BBHumanThromboembolism  ISORGD:732124 RGD 
Gp1bbRatThromboembolism  ISORGD:732124 RGD 
Gp1bbMouseThromboembolism  IMP  RGD 
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Genes (Rattus norvegicus)
Gp1bb  (glycoprotein Ib platelet subunit beta)

Genes (Mus musculus)
Gp1bb  (glycoprotein Ib, beta polypeptide)

Genes (Homo sapiens)
GP1BB  (glycoprotein Ib platelet subunit beta)