NFκB1 is essential to prevent the development of multiorgan autoimmunity by limiting IL-6 production in follicular B cells

J Exp Med. 2016 Apr 4;213(4):621-41. doi: 10.1084/jem.20151182. Epub 2016 Mar 28.

Abstract

We examined the role of NFκB1 in the homeostasis and function of peripheral follicular (Fo) B cells. Aging mice lacking NFκB1 (Nfκb1(-/-)) develop lymphoproliferative and multiorgan autoimmune disease attributed in large part to the deregulated activity of Nfκb1(-/-)Fo B cells that produce excessive levels of the proinflammatory cytokine interleukin 6 (IL-6). Despite enhanced germinal center (GC) B cell differentiation, the formation of GC structures was severely disrupted in the Nfκb1(-/-)mice. Bone marrow chimeric mice revealed that the Fo B cell-intrinsic loss of NFκB1 led to the spontaneous generation of GC B cells. This was primarily the result of an increase in IL-6 levels, which promotes the differentiation of Fo helper CD4(+)T cells and acts in an autocrine manner to reduce antigen receptor and toll-like receptor activation thresholds in a population of proliferating IgM(+)Nfκb1(-/-)Fo B cells. We demonstrate that p50-NFκB1 represses Il-6 transcription in Fo B cells, with the loss of NFκB1 also resulting in the uncontrolled RELA-driven transcription of Il-6.Collectively, our findings identify a previously unrecognized role for NFκB1 in preventing multiorgan autoimmunity through its negative regulation of Il-6 gene expression in Fo B cells.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Autoimmune Diseases / genetics
  • Autoimmune Diseases / immunology*
  • Autoimmune Diseases / pathology
  • B-Lymphocytes / immunology*
  • B-Lymphocytes / pathology
  • Germinal Center / immunology*
  • Germinal Center / pathology
  • Immunoglobulin M / genetics
  • Immunoglobulin M / immunology
  • Interleukin-6 / genetics
  • Interleukin-6 / immunology*
  • Mice
  • Mice, Knockout
  • NF-kappa B p50 Subunit / genetics
  • NF-kappa B p50 Subunit / immunology*
  • T-Lymphocytes, Helper-Inducer / immunology
  • T-Lymphocytes, Helper-Inducer / pathology
  • Transcription, Genetic / genetics
  • Transcription, Genetic / immunology*

Substances

  • Immunoglobulin M
  • Interleukin-6
  • NF-kappa B p50 Subunit
  • Nfkb1 protein, mouse