Table 1.

Superoxide production in the cell-free system

Membranes From Patient With Control Cytosol Cytosol From Patient With Control Membranes Membranes and Cytosol From Control
JW (T341K)  0.04 (±0.03)  6.64 (±0.12) 6.67 (±0.47)  
CE (C369R)  1.35 (±0.18) 7.65 (±0.34)  7.22 (±1.48)  
MK (G408E) 0.05 (±0.02)  5.95 (±0.19)  4.83 (±0.19)  
DG (E568K)  0.07 (±0.06)  5.86 (±0.25) 5.91 (±0.27) 
Membranes From Patient With Control Cytosol Cytosol From Patient With Control Membranes Membranes and Cytosol From Control
JW (T341K)  0.04 (±0.03)  6.64 (±0.12) 6.67 (±0.47)  
CE (C369R)  1.35 (±0.18) 7.65 (±0.34)  7.22 (±1.48)  
MK (G408E) 0.05 (±0.02)  5.95 (±0.19)  4.83 (±0.19)  
DG (E568K)  0.07 (±0.06)  5.86 (±0.25) 5.91 (±0.27) 

Values are given in μmol O2/min (mean ± SD; n = 3). Neutrophil membranes (10 μg) and cytosol (200 μg) were incubated at 28°C in oxidase buffer, as previously described, in the presence of 60 μmol/L cytochrome c. After 2 minutes of incubation, SDS (100 μmol/L) and GTPγS (10 μmol/L) were added. After another 5 minutes, NADPH (250 μmol/L) was added, and the rate of cytochrome c reduction was determined by the change in absorbance at 550 nm.

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