Table. Inhibition of fVIII activity in mild hemophilia a patients' plasma.

fVIII Mutation*fVIII Activity (IU/ml)LE2E9 Inhibition (%)
Arg1689Leu  0.04  >70  
Arg1749His  0.39 >70  
Gly1750Arg  0.38  >70  
Ala1824Val  0.16 >70  
Asp1825Gly  0.17  >70  
His1961Tyr  0.24 >70  
Arg1966Gln  0.08  >70  
Met2010Ile  0.04 >70  
Ser2011Asn  0.23  >70  
Val2016Ala  0.05 >70  
Asn2019ser  0.13  >70  
Leu2052Phe  0.23 >70  
Asp2074Gly  0.13  >70  
Thr2086Ile  0.08 >70  
Ile2098Ser  0.20  >70  
Phe2101Leu  0.06 >70  
Asn2129Ser  0.20  >70  
Arg2150His  0.03 0  
Pro2153Gln  0.02  65  
Arg2159Leu  0.16  >70 
Trp2229Cys  0.02  >70  
Gln2246Arg  0.08 >70 
fVIII Mutation*fVIII Activity (IU/ml)LE2E9 Inhibition (%)
Arg1689Leu  0.04  >70  
Arg1749His  0.39 >70  
Gly1750Arg  0.38  >70  
Ala1824Val  0.16 >70  
Asp1825Gly  0.17  >70  
His1961Tyr  0.24 >70  
Arg1966Gln  0.08  >70  
Met2010Ile  0.04 >70  
Ser2011Asn  0.23  >70  
Val2016Ala  0.05 >70  
Asn2019ser  0.13  >70  
Leu2052Phe  0.23 >70  
Asp2074Gly  0.13  >70  
Thr2086Ile  0.08 >70  
Ile2098Ser  0.20  >70  
Phe2101Leu  0.06 >70  
Asn2129Ser  0.20  >70  
Arg2150His  0.03 0  
Pro2153Gln  0.02  65  
Arg2159Leu  0.16  >70 
Trp2229Cys  0.02  >70  
Gln2246Arg  0.08 >70 
*

Mutations in the fVIII gene were detected according to reference 9.

fVIII levels in the patient's plasma were measured in a fVIII chromogenic assay.

Equal volumes of LE2E9 (20 μg/ml) and of plasma were incubated for 1 hour at 37°C. The residual fVIII activity was measured in a fVIII chromogenic assay and expressed in percentage of the activity obtained in the absence of antibody. Experiments were repeated at least twice with similar results.

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