Class Switch Recombination Defects impact on B cell maturation and antibody responses

Class Switch Recombination Defects : impact on B cell maturation and antibody responses

Copyright © 2020 Elsevier Inc. All rights reserved.

Bibliographische Detailangaben
Veröffentlicht in:Clinical immunology (Orlando, Fla.). - 1999. - 222(2021) vom: 15. Jan., Seite 108638
1. Verfasser: Renner, Ellen D (VerfasserIn)
Weitere Verfasser: Krätz, Carolin E, Orange, Jordan S, Hagl, Beate, Rylaarsdam, Stacey, Notheis, Gundula, Durandy, Anne, Torgerson, Troy R, Ochs, Hans D
Format: Online-Aufsatz
Sprache:English
Veröffentlicht: 2021
Zugriff auf das übergeordnete Werk:Clinical immunology (Orlando, Fla.)
Schlagworte:Journal Article Research Support, Non-U.S. Gov't B cell development Class switch recombination defect (CSRD) Clinical immunology Hyper-IgM syndromes (HIGM) Immunodeficiencies I-kappa B Proteins Immunoglobulin D Immunoglobulin M mehr... Tumor Necrosis Factor Receptor Superfamily, Member 7 CD40 Ligand 147205-72-9
Beschreibung
Zusammenfassung:Copyright © 2020 Elsevier Inc. All rights reserved.
To assess how B cell phenotype analysis correlates with antigen responses in patients with class switch recombination defects (CSRD) we quantified memory B cells by flow-cytometry and immunized CSRD patients with the neoantigen bacteriophage phiX174 (phage). CSRD patients showed uniformly absent or markedly reduced switched memory B cells (IgM-IgD-CD27+). CD40L patients had reduced CD27+ memory B cells (both non-switched and switched). In NEMO patients, results varied depending on the IKKγ gene variant. Three of four AID patients had normal percentages of CD27+ memory B cells while CD27+IgM-IgD- switched memory B cells were markedly reduced in all AID patients. Antibody response to phage was remarkably decreased with lack of memory amplification and class-switching in immunized CD40L, UNG deficient, and NEMO patients. Distinct B-cell phenotype pattern correlated with abnormal antibody responses to a T-cell dependent neoantigen, representing a powerful tool to identify CSRD patients
Beschreibung:Date Completed 16.06.2021
Date Revised 16.06.2021
published: Print-Electronic
Citation Status MEDLINE
ISSN:1521-7035
DOI:10.1016/j.clim.2020.108638