Eosinophilia and reduced STAT3 signaling affect neutrophil cell death in autosomal‐dominant Hyper‐IgE syndrome
Abstract: The autosomal‐dominant hyper‐IgE syndrome (HIES), caused by mutations in STAT3, is a rare primary immunodeficiency that predisposes to mucocutaneous candidiasis and staphylococcal skin and lung infections. This infection phenotype is suggestive of defects in neutrophils, but data on neutrophil functions in HIES are inconsistent. This study was undertaken to functionally characterize neutrophils in STAT3‐deficient HIES patients and to analyze whether the patients` eosinophilia affects the neutrophil phenotype in S. aureus infection. Neutrophil functions and cell death kinetics were studied in eight STAT3‐deficient patients. Moreover, the response of STAT3‐deficient neutrophils to S. aureus and the impact of autologous eosinophils on pathogen‐induced cell death were analyzed. No specific aberrations in neutrophil functions were detected within this cohort. However, the half‐life of STAT3‐deficient neutrophils ex vivo was reduced, which was partially attributable to the presence of eosinophils. Increased S. aureus‐induced cell lysis, dependent on the staphylococcal virulence controlling accessory gene regulator (agr)‐locus, was observed in STAT3‐deficient neutrophils and upon addition of eosinophils. Accelerated neutrophil cell death kinetics may underlie the reported variability in neutrophil function testing in HIES. Increased S. aureus‐induced lysis of STAT3‐deficient neutrophils might affect pathogen control and contribute to tissue destruction during staphylococcal infections in HIES.
- Location
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Deutsche Nationalbibliothek Frankfurt am Main
- Extent
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Online-Ressource
- Language
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Englisch
- Bibliographic citation
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Eosinophilia and reduced STAT3 signaling affect neutrophil cell death in autosomal‐dominant Hyper‐IgE syndrome ; volume:48 ; number:12 ; year:2018 ; pages:1975-1988 ; extent:14
European journal of immunology ; 48, Heft 12 (2018), 1975-1988 (gesamt 14)
- Creator
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Farmand, Susan
Kremer, Bernhard
Häffner, Monika
Pütsep, Katrin
Bergman, Peter
Sundin, Mikael
Ritterbusch, Henrike
Seidl, Maximilian
Follo, Marie
Henneke, Philipp
Henriques‐Normark, Birgitta
- DOI
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10.1002/eji.201847650
- URN
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urn:nbn:de:101:1-2022090507510793799185
- Rights
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Open Access; Der Zugriff auf das Objekt ist unbeschränkt möglich.
- Last update
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15.08.2025, 7:22 AM CEST
Data provider
Deutsche Nationalbibliothek. If you have any questions about the object, please contact the data provider.
Associated
- Farmand, Susan
- Kremer, Bernhard
- Häffner, Monika
- Pütsep, Katrin
- Bergman, Peter
- Sundin, Mikael
- Ritterbusch, Henrike
- Seidl, Maximilian
- Follo, Marie
- Henneke, Philipp
- Henriques‐Normark, Birgitta
Other Objects (12)
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Gene therapy for STAT3-Associated Autosomal-Dominant Hyper-Ige-Syndrome (AD-HIES) using RNA-guided nucleases
Can eosinophilia and neutrophil–lymphocyte ratio predict hospitalization in asthma exacerbation?
Gene Editing in the STAT3 Hyper-IgE Syndrome
STAT3 Hyper-IgE Syndrome—an Update and Unanswered Questions
Autosomal-rezessives Hyper-IgE-Syndrom (DOCK8-Defizienz) : klinische und immunologische Schlüsselbefunde
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Molecular Assessment of Staphylococcus Aureus Strains in STAT3 Hyper-IgE Syndrome Patients
Molecular assessment of staphylococcus aureus strains in STAT3 hyper-IgE syndrome patients
Pulmonary eosinophilia : [mit] 27 tables
Primary immunodeficiencies associated with eosinophilia
Intrazelluläres Zytokinprofil und Toll-like Rezeptor-Signalwege beim autosomal-dominanten Hyper-IgE-Syndrom
Eosinophilia and reduced STAT3 signaling affect neutrophil cell death in autosomal-dominant Hyper-IgE syndrome
Gene therapy for STAT3-Associated Autosomal-Dominant Hyper-Ige-Syndrome (AD-HIES) using RNA-guided nucleases
Can eosinophilia and neutrophil–lymphocyte ratio predict hospitalization in asthma exacerbation?
Gene Editing in the STAT3 Hyper-IgE Syndrome
STAT3 Hyper-IgE Syndrome—an Update and Unanswered Questions
Autosomal-rezessives Hyper-IgE-Syndrom (DOCK8-Defizienz) : klinische und immunologische Schlüsselbefunde
X-linked hyper IgM syndrome with severe eosinophilia: a case report and review of the literature
Molecular Assessment of Staphylococcus Aureus Strains in STAT3 Hyper-IgE Syndrome Patients
Molecular assessment of staphylococcus aureus strains in STAT3 hyper-IgE syndrome patients
Pulmonary eosinophilia : [mit] 27 tables
Primary immunodeficiencies associated with eosinophilia