B cells from nuclear factor kB essential modulator deficient patients fail to differentiate to antibody secreting cells in response to TLR9 ligand

B cells from nuclear factor kB essential modulator deficient patients fail to differentiate to antibody secreting cells in response to TLR9 ligand

Copyright © 2015 Elsevier Inc. All rights reserved.

Bibliographische Detailangaben
Veröffentlicht in:Clinical immunology (Orlando, Fla.). - 1999. - 161(2015), 2 vom: 05. Dez., Seite 131-5
1. Verfasser: Giardino, Giuliana (VerfasserIn)
Weitere Verfasser: Cirillo, Emilia, Gallo, Vera, Esposito, Tiziana, Fusco, Francesca, Conte, Matilde Immacolata, Quinti, Isabella, Ursini, Matilde Valeria, Carsetti, Rita, Pignata, Claudio
Format: Online-Aufsatz
Sprache:English
Veröffentlicht: 2015
Zugriff auf das übergeordnete Werk:Clinical immunology (Orlando, Fla.)
Schlagworte:Journal Article CpG Encapsulated bacteria IgM memory B cell NEMO Nuclear factor kB essential modulator TLR9 Immunoglobulins Ligands NF-kappa B mehr... TLR9 protein, human Toll-Like Receptor 9
Beschreibung
Zusammenfassung:Copyright © 2015 Elsevier Inc. All rights reserved.
Hypohidrotic ectodermal dysplasia (HED) consists of disorders resulting from molecular alterations of ectodysplasin-A (EDA) pathway. Hypomorphic mutations in NF-kB essential modulator, downstream EDA, result in HED with immunodeficiency (HED-ID), characterized by susceptibility to encapsulated pyogenic bacteria infections. Increased susceptibility to pneumococcal infections and poor response to polysaccharide antigens are associated with defect in T-independent B-cell immunity. We investigated B-cell differentiation and immunoglobulin secretion induced by the TLR9 ligand CpG in two HED-ID and in a HED patient caused by EDA mutations (XLHED). In HED-ID, only few B cells differentiated into plasma cells upon TLR9 stimulation and memory B cells did not produce IgG and IgA, but small amounts of IgM. Unexpectedly, memory B cells from XLHED patient failed to produce normal IgA or IgG amount upon TLR9 stimulation. Our findings expand the knowledge about the pathogenesis of humoral alterations in HED patients and help explain the susceptibility to pneumococcal infections
Beschreibung:Date Completed 29.02.2016
Date Revised 23.11.2015
published: Print-Electronic
Citation Status MEDLINE
ISSN:1521-7035
DOI:10.1016/j.clim.2015.08.008