Journal
JOURNAL OF CLINICAL IMMUNOLOGY
Volume 33, Issue 4, Pages 857-864Publisher
SPRINGER/PLENUM PUBLISHERS
DOI: 10.1007/s10875-012-9859-9
Keywords
Chronic granulomatous disease; p47phox; p67phox; flow cytometry; screening test
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Funding
- Morinaga Houshikai, Tokyo
- Ministry of Education, Culture, Sports, Science and Technology of Japan
- Ministry of Health, Labour, and Welfare of Japan, Tokyo
- Grants-in-Aid for Scientific Research [24591542, 22591155, 24590713] Funding Source: KAKEN
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Chronic granulomatous disease (CGD) is caused by defects of NADPH oxidase. The diagnosis of CGD can be made by analysis of NADPH oxidase activity, however, identification of the CGD subgroups is required before performing mutation analysis. The membrane-bound subunits, gp91phox and p22phox, can be quickly analyzed by flow cytometry, unlike the cytosolic components, p47phox and p67phox. We evaluated the feasibility of flow cytometric detection of p47phox and p67phox with specific monoclonal antibodies in two patients with p47phox deficiency and 7 patients with p67phox deficiency. Consistent with previous observations, p47phox and p67phox were expressed in phagocytes and B cells, but not in T or natural killer cells, from normal controls. In contrast, patients with p47phox and p67phox deficiency showed markedly reduced levels of p47phox and p67phox, respectively. These techniques will be useful to rapidly assess the expression of the cytosolic components, p47phox and p67phox, and represents important secondary screening tests for CGD.
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