Critical role of B cell lymphoma 10 in BAFF-regulated NF-κB activation and survival of anergic B cells

J Immunol. 2012 Dec 1;189(11):5185-93. doi: 10.4049/jimmunol.1102952. Epub 2012 Oct 19.

Abstract

Anergy is a key physiological mechanism for restraining self-reactive B cells. A marked portion of peripheral B cells are anergic B cells that largely depend on BAFF for survival. BAFF activates the canonical and noncanonical NF-κB pathways, both of which are required for B cell survival. In this study we report that deficiency of the adaptor protein B cell lymphoma 10 (Bcl10) impaired the ability of BAFF to support B cell survival in vitro, and it specifically increased apoptosis in anergic B cells in vivo, dramatically reducing anergic B cells in mice. Bcl10-dependent survival of self-reactive anergic B cells was confirmed in the Ig hen egg lysozyme/soluble hen egg lysozyme double-transgenic mouse model of B cell anergy. Furthermore, we found that BAFF stimulation induced Bcl10 association with IκB kinase β, a key component of the canonical NF-κB pathway. Consistently, Bcl10-deficient B cells were impaired in BAFF-induced IκBα phosphorylation and formation of nuclear p50/c-Rel complexes. Bcl10-deficient B cells also displayed reduced expression of NF-κB2/p100, severely reducing BAFF-induced nuclear accumulation of noncanonical p52/RelB complexes. Consequently, Bcl10-deficient B cells failed to express Bcl-x(L), a BAFF-induced NF-κB target gene. Taken together, these data demonstrate that Bcl10 controls BAFF-induced canonical NF-κB activation directly and noncanonical NF-κB activation indirectly. The BAFF-R/Bcl10/NF-κB signaling axis plays a critical role in peripheral B cell tolerance by regulating the survival of self-reactive anergic B cells.

Publication types

  • Research Support, American Recovery and Reinvestment Act
  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adaptor Proteins, Signal Transducing / deficiency
  • Adaptor Proteins, Signal Transducing / genetics
  • Adaptor Proteins, Signal Transducing / immunology*
  • Animals
  • Apoptosis
  • B-Cell Activating Factor / genetics
  • B-Cell Activating Factor / immunology*
  • B-Cell CLL-Lymphoma 10 Protein
  • B-Lymphocytes / cytology
  • B-Lymphocytes / immunology
  • B-Lymphocytes / metabolism
  • Cell Survival / genetics
  • Cell Survival / immunology*
  • Clonal Anergy
  • Gene Expression Regulation / immunology
  • I-kappa B Kinase / genetics
  • I-kappa B Kinase / immunology
  • Mice
  • Mice, Transgenic
  • Muramidase / immunology
  • NF-kappa B / genetics
  • NF-kappa B / immunology*
  • NF-kappa B p52 Subunit / genetics
  • NF-kappa B p52 Subunit / immunology
  • Phosphorylation
  • Proto-Oncogene Proteins c-rel / genetics
  • Proto-Oncogene Proteins c-rel / immunology
  • Signal Transduction*
  • Spleen / cytology
  • Spleen / immunology
  • Spleen / metabolism
  • Transcription Factor RelB / genetics
  • Transcription Factor RelB / immunology
  • bcl-X Protein / genetics
  • bcl-X Protein / immunology

Substances

  • Adaptor Proteins, Signal Transducing
  • B-Cell Activating Factor
  • B-Cell CLL-Lymphoma 10 Protein
  • Bcl10 protein, mouse
  • Bcl2l1 protein, mouse
  • NF-kappa B
  • NF-kappa B p52 Subunit
  • Proto-Oncogene Proteins c-rel
  • Relb protein, mouse
  • Tnfsf13b protein, mouse
  • bcl-X Protein
  • Transcription Factor RelB
  • I-kappa B Kinase
  • hen egg lysozyme
  • Muramidase