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).