Figure 6

rhC2 restores classical pathway complement activity in C2-deficient patient serum. A) Representative titration of rhC2 in serum from a C2-deficient patient and measurement of complement pathway activity by complement activation ELISA. "C' denotes inclusion of an irrelevant protein used to confirm specificity of the assay to rhC2. Data represent the mean ± SD of three independent experiments. B) rhC2 restores classical pathway hemolytic activity in C2-deficient patient sera. When rhC2 (25 μg/ml) was added to the sera from 3 different patients, complement activation was triggered and hemolysis observed. Samples no. 1 Normal human serum, 2 - 4 C2-deficient patient sera, 5-7 the C2-deficient patient sera restituted with rhC2 (25 μg/ml), 8 C2-deficient serum restituted with plasma purified C2 (25 μg/ml). Alternative pathway of hemolysis as a control is also shown.