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Chronic granulomatous disease
NADPH oxidase complex
Created by previous contributor, Rebecca A. Marsh, MD; used with permission from Cincinnati Children’s Hospital Medical Center Diagnostic Immunology Laboratory
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Chronic granulomatous disease
Sample DHR histograms. Neutrophils were incubated with dihydrorhodamine 123 (DHR 123) and then activated with phorbol 12-myristate 13-acetate (PMA). On activation, DHR 123 is oxidised to highly fluorescent rhodamine 123 in normal neutrophils. Pre-activation histograms are shown on the left and post-activation histograms on the right. Block A shows normal response, with a large rightward shift in mean fluorescent intensity. Block B shows a patient with X-linked CGD lacking a detectable oxidative burst. Block C shows the mother of an affected patient with 2 populations of neutrophils, one normal and one with mutated gp91phox. Block D shows the typical pattern observed in patients with autosomal recessive CGD. Patients with autosomal recessive CGD can also rarely display an absence of oxidative burst activity, as shown in Block E.
Permission by CCHMC clinical diagnostic immunology lab
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