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

Giuliana Giardino, Emilia Cirillo, Vera Gallo, Tiziana Esposito, Francesca Fusco, Matilde Immacolata Conte, Isabella Quinti, Matilde Valeria Ursini, Rita Carsetti, Claudio Pignata

Research output: Contribution to journalArticlepeer-review

Abstract

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.

Original languageEnglish
Pages (from-to)131-135
Number of pages5
JournalClinical Immunology
Volume161
Issue number2
DOIs
Publication statusPublished - Dec 1 2015

Keywords

  • CpG
  • Encapsulated bacteria
  • IgM memory B cell
  • NEMO
  • Nuclear factor kB essential modulator
  • TLR9

ASJC Scopus subject areas

  • Immunology
  • Immunology and Allergy

Fingerprint Dive into the research topics of 'B cells from nuclear factor kB essential modulator deficient patients fail to differentiate to antibody secreting cells in response to TLR9 ligand'. Together they form a unique fingerprint.

Cite this