GSD-Ib neutrophils exhibit oxidative stress. Peritoneal neutrophils were isolated from 6- to 7-week-old unaffected (+/+) and GSD-Ib (−/−) mice during thioglycollate-elicited peritonitis. (A) Representative flow cytometric analysis of neutrophil carboxy-DCF staining. (B) Quantification of carboxy-DCF–positive neutrophils determined by immunofluorescence staining. Data represent the means (± SEM) of 3 independent experiments. ***P < .001. (C) Oxyblot analysis of carbonyl groups in oxidative modified proteins. Each lane contains 50 μg protein. The vertical line has been inserted to indicate a repositioned gel lane. (D) Representative flow cytometric analysis of neutrophil R-123 staining. (E) Quantification of R-123–positive neutrophils determined by immunofluorescence staining. Data represent the means (± SEM) of 3 independent experiments. **P < .001. (F) Western blot analysis of protein extracts of neutrophils using antibodies against Mn-SOD or β-actin. Each lane contains 50 μg protein.