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

2.50
Hdl Handle:
http://hdl.handle.net/2336/620590
Title:
Transitional B Cells and TLR9 Responses Are Defective in Selective IgA Deficiency.
Authors:
Lemarquis, Andri L; Einarsdottir, Helga K; Kristjansdottir, Rakel N; Jonsdottir, Ingileif; Ludviksson, Bjorn R
Citation:
Transitional B Cells and TLR9 Responses Are Defective in Selective IgA Deficiency. 2018, 9:909 Front Immunol
Issue Date:
2018
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.
Description:
To access publisher's full text version of this article, please click on the hyperlink in Additional Links field or click on the hyperlink at the top of the page marked Files
Additional Links:
https://www.frontiersin.org/articles/10.3389/fimmu.2018.00909/full
Rights:
Archived with thanks to Frontiers in immunology

Full metadata record

DC FieldValue Language
dc.contributor.authorLemarquis, Andri Len
dc.contributor.authorEinarsdottir, Helga Ken
dc.contributor.authorKristjansdottir, Rakel Nen
dc.contributor.authorJonsdottir, Ingileifen
dc.contributor.authorLudviksson, Bjorn Ren
dc.date.accessioned2018-06-12T14:30:17Z-
dc.date.available2018-06-12T14:30:17Z-
dc.date.issued2018-
dc.date.submitted2018-
dc.identifier.citationTransitional B Cells and TLR9 Responses Are Defective in Selective IgA Deficiency. 2018, 9:909 Front Immunolen
dc.identifier.issn1664-3224-
dc.identifier.pmid29755476-
dc.identifier.doi10.3389/fimmu.2018.00909-
dc.identifier.urihttp://hdl.handle.net/2336/620590-
dc.descriptionTo access publisher's full text version of this article, please click on the hyperlink in Additional Links field or click on the hyperlink at the top of the page marked Filesen
dc.description.abstractSelective 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.en
dc.description.sponsorshipIcelandic Research Fund University hospital of Iceland research funden
dc.language.isoenen
dc.publisherFrontiers Media SAen
dc.relation.urlhttps://www.frontiersin.org/articles/10.3389/fimmu.2018.00909/fullen
dc.rightsArchived with thanks to Frontiers in immunologyen
dc.subjectSjálfsofnæmissjúkdómaren
dc.subjectÓnæmiskerfien
dc.subjectMótefnien
dc.subjectNAF12en
dc.subjectAAI12en
dc.subject.meshIgA Deficiencyen
dc.subject.meshAutoimmune Diseasesen
dc.titleTransitional B Cells and TLR9 Responses Are Defective in Selective IgA Deficiency.en
dc.typeArticleen
dc.contributor.department1 ] Landspitali Univ Hosp, Dept Immunol, Reykjavik, Iceland Show more [ 2 ] Univ Iceland, Fac Med, Reykjavik, Iceland [ 3 ] deCODE Genet, Div Infect & Inflammatory Dis, Reykjavik, Icelanden
dc.identifier.journalFrontiers in immunologyen
dc.rights.accessOpen Access - Opinn aðganguren

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