Table 3.

Reactivity of patient sera against wild-type GPIX and GPIX mutants

-Qn*
+Qn P1
+Qn P2
+Qn P3
+Qn P4
+Qn P5
+Qn P6
CHOαβ GPIX
GMF
% Pos
GMF
% Pos
GMF
% Pos
GMF
% Pos
GMF
% Pos
GMF
% Pos
GMF
% Pos
GPIX (wt)   2.5   0.05   73.56   94.61   67.51   94.79   57.22   93.50   31.80   82.05   24.92   77.13   27.08   77.95  
Mut1   2.70   0.17   60.01   92.51   51.88   89.62   40.09   85.73   15.95   53.39   19.63   62.16   18.29   54.38  
Mut2   2.84   0.09   52.43   84.40   43.24   81.44   46.45   83.14   16.05   59.06   23.60   71.86   18.15   63.82  
Mut3   3.1   0.06   61.44   84.41   43.38   79.69   45.05   78.53   19.21   62.61   19.50   61.19   18.06   54.92  
Mut4   3.3   0.05   18.53   53.52   11.29   43.98   10.29   38.11   8.14   25.35   8.11   16.69   6.42   20.55  
    Mut4.1   2.26   0.02   17.76   53.88   9.23   33.12   8.25   26.12   9.42   27.18   10.19   28.75   3.65   3.16  
    Mut4.2   3.15   0.18   43.01   74.98   38.47   69.33   37.29   67.46   16.16   54.45   16.68   45.11   11.03   32.57  
Mut5   2.30   0.02   61.70   83.82   52.7   80.26   41.76   77.26   17.69   62.29   21.76   67.70   22.28   71.83  
Mut6
 		 2.26
 		 0.02
 		 63.94
 		 89.45
 		 54.57
 		 87.26
 		 45.07
 		 83.78
 		 16.95
 		 58.53
 		 21.88
 		 67.12
 		 25.07
 		 76.41
 
-Qn*
+Qn P1
+Qn P2
+Qn P3
+Qn P4
+Qn P5
+Qn P6
CHOαβ GPIX
GMF
% Pos
GMF
% Pos
GMF
% Pos
GMF
% Pos
GMF
% Pos
GMF
% Pos
GMF
% Pos
GPIX (wt)   2.5   0.05   73.56   94.61   67.51   94.79   57.22   93.50   31.80   82.05   24.92   77.13   27.08   77.95  
Mut1   2.70   0.17   60.01   92.51   51.88   89.62   40.09   85.73   15.95   53.39   19.63   62.16   18.29   54.38  
Mut2   2.84   0.09   52.43   84.40   43.24   81.44   46.45   83.14   16.05   59.06   23.60   71.86   18.15   63.82  
Mut3   3.1   0.06   61.44   84.41   43.38   79.69   45.05   78.53   19.21   62.61   19.50   61.19   18.06   54.92  
Mut4   3.3   0.05   18.53   53.52   11.29   43.98   10.29   38.11   8.14   25.35   8.11   16.69   6.42   20.55  
    Mut4.1   2.26   0.02   17.76   53.88   9.23   33.12   8.25   26.12   9.42   27.18   10.19   28.75   3.65   3.16  
    Mut4.2   3.15   0.18   43.01   74.98   38.47   69.33   37.29   67.46   16.16   54.45   16.68   45.11   11.03   32.57  
Mut5   2.30   0.02   61.70   83.82   52.7   80.26   41.76   77.26   17.69   62.29   21.76   67.70   22.28   71.83  
Mut6
 		 2.26
 		 0.02
 		 63.94
 		 89.45
 		 54.57
 		 87.26
 		 45.07
 		 83.78
 		 16.95
 		 58.53
 		 21.88
 		 67.12
 		 25.07
 		 76.41
 

Flow cytometric analysis was performed by incubating patient sera and, with CHO cells, expressing GPIbαβ and the wild- type GPIX or a GPIX mutant, in the presence or absence of quinine as described in “Patients, materials, and methods.” GMF indicates geometric mean of fluorescence; Qn, quinine; P1, patient 1; P2, patient 2, etc; % pos, percentage of positive cells. Reactivity of patient sera against Mut4 and Mut4.1 is substantially reduced.

*

Experiments performed in the absence of quinine gave uniformly low readings and are shown as the average for all six patient sera.

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