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. 2013 Jan 23;21(10):1079–1084. doi: 10.1038/ejhg.2012.310

Figure 3.

Figure 3

Phenotypic profiles of neutrophils from patients 1 and 2. (a) The absence of NADPH oxidase activity was evidenced in the neutrophils of patients 1 and 2 by resorufin (Amplex Red) fluorescence kinetics as described in Materials and methods section. Parents of patient 1 exhibited normal activity as it is usually the case in AR CGD. (b) The absence of oxidase activity was confirmed by the absence of immunodetection of p47phox expression in both patients, whereas the other oxidase components were present. The experiment was performed on 1% Triton X100 soluble extract of neutrophils subjected to SDS–PAGE and blotted onto nitrocellulose sheet. Proteins were detected by the ECL Advance Western Blotting Detection Kit (GE Healthcare, formerly Amersham Biosciences) for patient 1 and by infrared-based fluorometric detection by the Odyssey system (LI-COR Biosciences, Ltd,) for patient 2.