Journal of Clinical Immunology

, Volume 33, Issue 4, pp 857–864

Rapid Detection of Intracellular p47phox and p67phox by Flow Cytometry; Useful Screening Tests for Chronic Granulomatous Disease

  • Taizo Wada
  • Masahiro Muraoka
  • Tomoko Toma
  • Tsuyoshi Imai
  • Tomonari Shigemura
  • Kazunaga Agematsu
  • Kohei Haraguchi
  • Hiroyuki Moriuchi
  • Tsutomu Oh-ishi
  • Toshiyuki Kitoh
  • Osamu Ohara
  • Tomohiro Morio
  • Akihiro Yachie
Original Research

DOI: 10.1007/s10875-012-9859-9

Cite this article as:
Wada, T., Muraoka, M., Toma, T. et al. J Clin Immunol (2013) 33: 857. doi:10.1007/s10875-012-9859-9

Abstract

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.

Keywords

Chronic granulomatous disease p47phox p67phox flow cytometry screening test 

Copyright information

© Springer Science+Business Media New York 2013

Authors and Affiliations

  • Taizo Wada
    • 1
  • Masahiro Muraoka
    • 1
  • Tomoko Toma
    • 1
  • Tsuyoshi Imai
    • 2
  • Tomonari Shigemura
    • 3
  • Kazunaga Agematsu
    • 3
  • Kohei Haraguchi
    • 4
  • Hiroyuki Moriuchi
    • 4
  • Tsutomu Oh-ishi
    • 5
  • Toshiyuki Kitoh
    • 6
  • Osamu Ohara
    • 7
  • Tomohiro Morio
    • 8
  • Akihiro Yachie
    • 1
  1. 1.Department of PediatricsSchool of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa UniversityKanazawaJapan
  2. 2.Department of PediatricsOtsu Red-Cross HospitalOtsuJapan
  3. 3.Department of PediatricsShinshu University School of MedicineMatsumotoJapan
  4. 4.Department of PediatricsNagasaki University HospitalNagasakiJapan
  5. 5.Division of Infectious Diseases, Immunology, and AllergySaitama Children’s Medical CenterSaitamaJapan
  6. 6.Department of PediatricsAichi Medical UniversityNagakuteJapan
  7. 7.Kazusa DNA research InstitutionChibaJapan
  8. 8.Department of PediatricsTokyo Medical and Dental UniversityTokyoJapan

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