| Selective insufficiency of IFN-gamma secretion in patients with hyper-IgE syndrome. | |
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MedLine Citation:
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PMID: 12708982 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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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-gamma (IFN-gamma) 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-gamma remains unknown. To elucidate the pathogenesis of the defective production of IFN-gamma in patients with hyper-IgE syndrome, we assessed the in vitro production and secretion of IFN-gamma 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-gamma, interleukin-2 (IL-2), and IL-4 by PBMCs from three patients with hyper-IgE syndrome were determined. RESULTS: The transcription of IFN-gamma mRNA and the production of its protein molecules progressed normally. However, selective insufficiency in the secretion of IFN-gamma molecules was found in patients with hyper-IgE syndrome. Confocal laser scanning microscopy clearly demonstrated the accumulation of IFN-gamma in patients with hyper-IgE syndrome. CONCLUSION: We demonstrated that there was a selective insufficiency in the secretion of IFN-gamma in patients with hyper-IgE syndrome. We hope that this fact would offer a new paradigm for understanding this disease. |
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Authors:
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R Ito; M Mori; S Katakura; N Kobayashi; T Naruto; Y Osamura; Y Aihara; S Yokota |
Publication Detail:
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Type: Journal Article |
Journal Detail:
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Title: Allergy Volume: 58 ISSN: 0105-4538 ISO Abbreviation: Allergy Publication Date: 2003 Apr |
Date Detail:
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Created Date: 2003-04-23 Completed Date: 2003-09-02 Revised Date: 2009-11-03 |
Medline Journal Info:
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Nlm Unique ID: 7804028 Medline TA: Allergy Country: Denmark |
Other Details:
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Languages: eng Pagination: 329-36 Citation Subset: IM |
Affiliation:
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Department of pediatrics, Yokohama City University School of Medicine, Fukuura kanazawaku Yokohama city, Japan. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adult Case-Control Studies Cells, Cultured Chemotaxis Child Cytokines / metabolism Female Flow Cytometry Fluorescent Antibody Technique Gene Expression Humans Hypergammaglobulinemia / genetics, immunology* Immunoglobulin E / biosynthesis* Interferon-gamma / metabolism* Interferon-gamma, Recombinant / administration & dosage, genetics, metabolism* Interleukin-2 / metabolism Interleukin-4 / metabolism Male Microscopy, Confocal Microscopy, Electron Neutrophils / metabolism RNA, Messenger / analysis Receptors, Interferon / metabolism Syndrome |
| Chemical | |
Reg. No./Substance:
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0/Cytokines; 0/Interferon-gamma, Recombinant; 0/Interleukin-2; 0/RNA, Messenger; 0/Receptors, Interferon; 207137-56-2/Interleukin-4; 37341-29-0/Immunoglobulin E; 82115-62-6/Interferon-gamma |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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