Thymic epithelial cells co-opt lineage-defining transcription factors to eliminate autoreactive T cells

Cell. 2022 Jul 7;185(14):2542-2558.e18. doi: 10.1016/j.cell.2022.05.018. Epub 2022 Jun 16.

Abstract

Medullary thymic epithelial cells (mTECs) ectopically express thousands of peripheral-tissue antigens (PTAs), which drive deletion or phenotypic diversion of self-reactive immature T cells during thymic differentiation. Failure of PTA expression causes multiorgan autoimmunity. By assaying chromatin accessibility in individual mTECs, we uncovered signatures of lineage-defining transcription factors (TFs) for skin, lung, liver, and intestinal cells-including Grhl, FoxA, FoxJ1, Hnf4, Sox8, and SpiB-in distinct mTEC subtypes. Transcriptomic and histologic analyses showed that these subtypes, which we collectively term mimetic cells, expressed PTAs in a biologically logical fashion, mirroring extra-thymic cell types while maintaining mTEC identity. Lineage-defining TFs bound to mimetic-cell open chromatin regions and were required for mimetic cell accumulation, whereas the tolerogenic factor Aire was partially and variably required. Expression of a model antigen in mimetic cells sufficed to induce cognate T cell tolerance. Thus, mTECs co-opt lineage-defining TFs to drive mimetic cell accumulation, PTA expression, and self-tolerance.

Keywords: Aire; T cell; Treg; autoimmunity; mTEC; microfold; mimetic cell; thymus; tolerance; transcription factor.

Publication types

  • Research Support, N.I.H., Extramural

MeSH terms

  • Animals
  • Antigens
  • Cell Differentiation
  • Chromatin / metabolism
  • Epithelial Cells* / metabolism
  • Mice
  • Mice, Inbred C57BL
  • T-Lymphocytes* / metabolism
  • Thymus Gland / metabolism
  • Transcription Factors / genetics
  • Transcription Factors / metabolism

Substances

  • Antigens
  • Chromatin
  • Transcription Factors