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Transitional B Cells and TLR9 Responses Are Defective in Selective IgA Deficiency

Transitional B Cells and TLR9 Responses Are Defective in Selective IgA Deficiency


Title: Transitional B Cells and TLR9 Responses Are Defective in Selective IgA Deficiency
Author: Lemarquis, Andri Leo   orcid.org/0000-0001-5165-0247
Einarsdóttir, Helga K.
Kristjansdóttir, Rakel N.
Jonsdottir, Ingileif   orcid.org/0000-0001-8339-150X
Lúðvíksson, Björn R.
Date: 2018-04-27
Language: English
Scope: 909
University/Institute: Háskóli Íslands
University of Iceland
School: Heilbrigðisvísindasvið (HÍ)
School of Health Sciences (UI)
Department: Læknadeild (HÍ)
Faculty of Medicine (UI)
Series: Frontiers in Immunology;9
ISSN: 1664-3224
DOI: 10.3389/fimmu.2018.00909
Subject: Selective IgA deficiency; B cells; T cells; Ónæmiskerfi; T-frumur; Ónæmisfræði; Mótefni
URI: https://hdl.handle.net/20.500.11815/1001

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Citation:

Lemarquis, A. L., Einarsdottir, H. K., Kristjansdottir, R. N., Jonsdottir, I., & Ludviksson, B. R. (2018). Transitional B Cells and TLR9 Responses Are Defective in Selective IgA Deficiency. Frontiers in Immunology, 9(909). doi:10.3389/fimmu.2018.00909

Abstract:

Selective IgA deficiency (IgAD) is the most common primary antibody deficiency in the western world with affected individuals suffering from an increased burden of autoimmunity, atopic diseases and infections. It has been shown that IgAD B cells can be induced with germinal center mimicking reactions to produce IgA. However, IgA is the most prevalent antibody in mucosal sites, where antigen-independent responses are important. Much interest has recently focused on the role of TLR9 in both naïve and mature B cell differentiation into IgA secreting plasma cells. Here, we analyze the phenotype and function of T and B cells in individuals with IgAD following IgA-inducing CpG-TLR9 stimulations. The IgAD individuals had significantly lower numbers of transitional B cells (CD19+CD24hiCD38hi) and class-switched memory B cells (CD20+CD27+IgD−) ex vivo. However, proportions of T cell populations ex vivo as well as in vitro induced T effector cells and T regulatory cells were comparable to healthy controls. After CpG stimulation, the transitional B cell defect was further enhanced, especially within its B regulatory subset expressing IL-10. Finally, CpG stimulation failed to induce IgA production in IgAD individuals. Collectively, our results demonstrate a defect of the TLR9 responses in IgAD that leads to B cell dysregulation and decreased IgA production.

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