IL-15, gluten and HLA-DQ8 drive tissue destruction in coeliac disease

Nature. 2020 Feb;578(7796):600-604. doi: 10.1038/s41586-020-2003-8. Epub 2020 Feb 12.

Abstract

Coeliac disease is a complex, polygenic inflammatory enteropathy caused by exposure to dietary gluten that occurs in a subset of genetically susceptible individuals who express either the HLA-DQ8 or HLA-DQ2 haplotypes1,2. The need to develop non-dietary treatments is now widely recognized3, but no pathophysiologically relevant gluten- and HLA-dependent preclinical model exists. Furthermore, although studies in humans have led to major advances in our understanding of the pathogenesis of coeliac disease4, the respective roles of disease-predisposing HLA molecules, and of adaptive and innate immunity in the development of tissue damage, have not been directly demonstrated. Here we describe a mouse model that reproduces the overexpression of interleukin-15 (IL-15) in the gut epithelium and lamina propria that is characteristic of active coeliac disease, expresses the predisposing HLA-DQ8 molecule, and develops villous atrophy after ingestion of gluten. Overexpression of IL-15 in both the epithelium and the lamina propria is required for the development of villous atrophy, which demonstrates the location-dependent central role of IL-15 in the pathogenesis of coeliac disease. In addition, CD4+ T cells and HLA-DQ8 have a crucial role in the licensing of cytotoxic T cells to mediate intestinal epithelial cell lysis. We also demonstrate a role for the cytokine interferon-γ (IFNγ) and the enzyme transglutaminase 2 (TG2) in tissue destruction. By reflecting the complex interaction between gluten, genetics and IL-15-driven tissue inflammation, this mouse model provides the opportunity to both increase our understanding of coeliac disease, and develop new therapeutic strategies.

MeSH terms

  • Animals
  • CD4-Positive T-Lymphocytes / immunology
  • Celiac Disease / immunology*
  • Celiac Disease / pathology*
  • Female
  • Glutens / immunology*
  • HLA-DQ Antigens / genetics
  • HLA-DQ Antigens / immunology*
  • Humans
  • Interferon-gamma / immunology
  • Interleukin-15 / genetics
  • Interleukin-15 / immunology*
  • Male
  • Mice
  • Mice, Transgenic
  • Microfilament Proteins / genetics
  • Microfilament Proteins / metabolism

Substances

  • HLA-DQ Antigens
  • HLA-DQ8 antigen
  • IL15 protein, human
  • Interleukin-15
  • Microfilament Proteins
  • villin
  • Glutens
  • Interferon-gamma