Table 1

Effect of type 2B mutations on VWF function in vivo

wt-mVWFmVWF/R1306QmVWF/V1316M
Tail-bleeding test (correction tail-bleeding time)    
    Treatment:    
        None 6/6 0/5 0/5 
        Platelets ND ND 0/4 
        VWF-rich plasm ND ND 0/3 
        Platelets + VWF-rich plasma ND ND 2/3 
Thrombosis-model    
    Mesenteric veins    
        Formation of thrombi larger than 50 μm 7/7 5/5 1/5 
        Occlusion 6/7 1/5* 0/5 
    Mesenteric arterioles    
        Formation of thrombi larger than 50 μm 7/7 2/5* 0/5 
        Occlusion 6/7 1/5* 0/5 
wt-mVWFmVWF/R1306QmVWF/V1316M
Tail-bleeding test (correction tail-bleeding time)    
    Treatment:    
        None 6/6 0/5 0/5 
        Platelets ND ND 0/4 
        VWF-rich plasm ND ND 0/3 
        Platelets + VWF-rich plasma ND ND 2/3 
Thrombosis-model    
    Mesenteric veins    
        Formation of thrombi larger than 50 μm 7/7 5/5 1/5 
        Occlusion 6/7 1/5* 0/5 
    Mesenteric arterioles    
        Formation of thrombi larger than 50 μm 7/7 2/5* 0/5 
        Occlusion 6/7 1/5* 0/5 

Mouse tail-bleeding test and ferric chloride–induced thrombosis test were performed as described in “Methods.” Correction of bleeding time was defined as the arrest of bleeding within the observation time of 10 minutes. Formation of thrombi larger than 50 μm was defined as the presence of thrombi larger than 50 μm during the observation period of 40 minutes. Occlusion of vessels was defined as the arrest of flow within the observation period of 40 minutes. mVWF/V1316M-expressing mice were treated with platelet concentrates (0.5 ×109 platelets/mouse), VWF-rich plasma, or a combination of both. VWF-rich plasma was obtained from wt-mice overexpressing wt-mVWF, with wt-VWF levels being 800% of normal. Levels of wt-mVWF after treatment were 190% of normal. Numbers represent the number of mice reaching the criteria per number of mice tested.

VWF indicates von Willebrand factor; and ND, not determined.

*

P < .05 compared with wt-mVWF as determined by 2-tail χ2 test.

P < .01 compared with wt-mVWF as determined by 2-tail χ2 test.

or Create an Account

Close Modal
Close Modal