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Selective insufficiency of IFN‐γ secretion in patients with hyper‐IgE syndrome
Author(s) -
Ito R.,
Mori M.,
Katakura S.,
Kobayashi N.,
Naruto T.,
Osamura Y.,
Aihara Y.,
Yokota S.
Publication year - 2003
Publication title -
allergy
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.363
H-Index - 173
eISSN - 1398-9995
pISSN - 0105-4538
DOI - 10.1034/j.1398-9995.2003.00099.x
Subject(s) - immunoglobulin e , immunology , peripheral blood mononuclear cell , secretion , pathogenesis , chemotaxis , immune system , medicine , biology , antibody , in vitro , receptor , biochemistry
Background: Hyper‐immunoglobulin E (IgE) syndrome is a complex immune deficiency characterized by chronic eczematous dermatitis, recurrent staphylococcal infections, pneumatoceles, reduced neutrophil chemotaxis, and variably impaired T cell function. Although decreased interferon‐ γ (IFN‐ γ ) production in patients with hyper‐IgE syndrome is pointed out and known as a cause of reduced neutrophil chemotaxis, precise mechanism of their inadequate production of IFN‐ γ remains unknown. To elucidate the pathogenesis of the defective production of IFN‐ γ in patients with hyper‐IgE syndrome, we assessed the in vitro production and secretion of IFN‐ γ by peripheral blood mononuclear cells (PBMCs) from patients with hyper‐IgE syndrome. Methods: Chemotaxis of neutrophils, mRNA levels of several cytokines, intracellular production and extracellular secretion of IFN‐ γ , interleukin‐2 (IL‐2), and IL‐4 by PBMCs from three patients with hyper‐IgE syndrome were determined. Results: The transcription of IFN‐ γ mRNA and the production of its protein molecules progressed normally. However, selective insufficiency in the secretion of IFN‐ γ molecules was found in patients with hyper‐IgE syndrome.Confocal laser scanning microscopy clearly demonstrated the accumulation of IFN‐ γ in patients with hyper‐IgE syndrome. Conclusion: We demonstrated that there was a selective insufficiency in the secretion of IFN‐ γ in patients with hyper‐IgE syndrome. We hope that this fact would offer a new paradigm for understanding this disease.