Figure 7.

Neutrophil functional capacity in G6PC3 deficiency. (A) Defective microbicidal activity in neutrophils from patients with G6PC3 deficiency resulting from homozygous inheritance of G6PC3 G260R. Staphylococcus aureus was incubated with polymorphonuclear leukocytes from a healthy donor (HD) and the 2 patients in an 8:1 target/effector ratio. Bacterial killing kinetics is plotted for each donor as a percentage of colony-forming units observed for control S aureus incubated in the absence of neutrophils. Data were obtained after the patients were treated for 5 months with G-CSF and are from a single experiment in which each condition was tested in duplicate. (B) Purified mouse bone marrow neutrophils from G6pc3 knockout and wild-type littermate control mice chemotax to CXCL12 equally well (P > .05 at all time points). Data are summarized from 2 experiments with 2 mice in each group.