RANK signals from CD4(+)3(-) inducer cells regulate development of Aire-expressing epithelial cells in the thymic medulla

J Exp Med. 2007 Jun 11;204(6):1267-72. doi: 10.1084/jem.20062497. Epub 2007 May 14.

Abstract

Aire-expressing medullary thymic epithelial cells (mTECs) play a key role in preventing autoimmunity by expressing tissue-restricted antigens to help purge the emerging T cell receptor repertoire of self-reactive specificities. Here we demonstrate a novel role for a CD4(+)3(-) inducer cell population, previously linked to development of organized secondary lymphoid structures and maintenance of T cell memory in the functional regulation of Aire-mediated promiscuous gene expression in the thymus. CD4(+)3(-) cells are closely associated with mTECs in adult thymus, and in fetal thymus their appearance is temporally linked with the appearance of Aire(+) mTECs. We show that RANKL signals from this cell promote the maturation of RANK-expressing CD80(-)Aire(-) mTEC progenitors into CD80(+)Aire(+) mTECs, and that transplantation of RANK-deficient thymic stroma into immunodeficient hosts induces autoimmunity. Collectively, our data reveal cellular and molecular mechanisms leading to the generation of Aire(+) mTECs and highlight a previously unrecognized role for CD4(+)3(-)RANKL(+) inducer cells in intrathymic self-tolerance.

Publication types

  • Comparative Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • AIRE Protein
  • Animals
  • Autoimmunity / immunology*
  • CD4-Positive T-Lymphocytes / immunology
  • CD4-Positive T-Lymphocytes / metabolism*
  • Cell Differentiation / immunology
  • DNA Primers
  • Epithelial Cells / cytology
  • Epithelial Cells / metabolism
  • Mice
  • Mice, Transgenic
  • Microscopy, Confocal
  • Polymerase Chain Reaction
  • Receptor Activator of Nuclear Factor-kappa B / immunology
  • Receptor Activator of Nuclear Factor-kappa B / metabolism*
  • Signal Transduction / immunology*
  • Thymus Gland / immunology
  • Thymus Gland / metabolism*
  • Transcription Factors / immunology
  • Transcription Factors / metabolism*

Substances

  • DNA Primers
  • Receptor Activator of Nuclear Factor-kappa B
  • Tnfrsf11a protein, mouse
  • Transcription Factors