Quantitative defects in invariant NKT cells and TLR responses in patients with hyper-IgE syndrome

Allergologia et immunopathologia
S Gutierrez-HincapiéC M Trujillo-Vargas

Abstract

Autosomal dominant hyper-IgE syndrome (AD-HIES) is a primary immunodeficiency mainly caused by mutations in STAT3, a signalling molecule implicated in the development of appropriate immune responses. We aimed to characterise the innate immune response in AD-HIES. The frequency of innate immune cells in peripheral blood (PB) from seven AD-HIES patients and healthy controls were determined. CD80/CD86 surface expression and cytokine levels in supernatants from PBMC after stimulation with TLR-2, -4 and -9 agonists were also measured by flow cytometry. In addition, several SNPs within these TLR genes in genomic DNA samples from patients and controls were examined. A significantly reduced number of PB iNKT cells was observed in the AD-HIES group. CpG-stimulated pDC and mDC from patients exhibited a lower increase in the expression of the costimulatory molecule CD80. We also observed an increase in the secretion of IL-12p70, TNF-alpha and IL-10 in PBMC from HIES patients after LTA or LPS stimuli. No association was found between the different SNPs detected and the HIES phenotype. These findings demonstrate that important mediators of the innate immunity responses are affected in AD-HIES. More studies are necessary to investigate how t...Continue Reading

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Citations

Sep 7, 2017·The Journal of Immunology : Official Journal of the American Association of Immunologists·Stuart G TangyeCindy S Ma
Jun 18, 2018·International Archives of Allergy and Immunology·Bogusław NedoszytkoRoman Nowicki
Dec 29, 2020·Molecular Immunology·Elizabeth A Leadbetter, Mikael C I Karlsson
Jan 14, 2021·Immunological Reviews·Elizabeth A Leadbetter, Mikael C I Karlsson

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