Figure 1.
DHR assay and quantitative assessment of superoxide production highlights X-CGD carriers with reduced-frequency functioning phagocytes to produce normal level O2– per cell. (A) FACS DHR assay comparing ROS (blue) production in HD subjects, X-CGD carriers, and X-CGD patients with null mutation, or missense low residual ROS producing, compared with unstimulated controls (red). (B) Quantitative superoxide measurement in pooled cells from HD subjects (n = 1095), X-CGD carriers (n = 187) (pooled cells or normalized to DHR+ cells), and X-CGD patients (n = 128).