LRH-1 mitigates intestinal inflammatory disease by maintaining epithelial homeostasis and cell survival

Nat Commun. 2018 Oct 10;9(1):4055. doi: 10.1038/s41467-018-06137-w.

Abstract

Epithelial dysfunction and crypt destruction are defining features of inflammatory bowel disease (IBD). However, current IBD therapies targeting epithelial dysfunction are lacking. The nuclear receptor LRH-1 (NR5A2) is expressed in intestinal epithelium and thought to contribute to epithelial renewal. Here we show that LRH-1 maintains intestinal epithelial health and protects against inflammatory damage. Knocking out LRH-1 in murine intestinal organoids reduces Notch signaling, increases crypt cell death, distorts the cellular composition of the epithelium, and weakens the epithelial barrier. Human LRH-1 (hLRH-1) rescues epithelial integrity and when overexpressed, mitigates inflammatory damage in murine and human intestinal organoids, including those derived from IBD patients. Finally, hLRH-1 greatly reduces disease severity in T-cell-mediated murine colitis. Together with the failure of a ligand-incompetent hLRH-1 mutant to protect against TNFα-damage, these findings provide compelling evidence that hLRH-1 mediates epithelial homeostasis and is an attractive target for intestinal disease.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Cell Differentiation
  • Cell Survival
  • Colitis / metabolism
  • Colitis / pathology
  • Disease Models, Animal
  • Epithelium / pathology*
  • Homeostasis*
  • Humans
  • Inflammatory Bowel Diseases / metabolism*
  • Inflammatory Bowel Diseases / pathology*
  • Mice
  • Organoids / metabolism
  • Receptors, Cytoplasmic and Nuclear / metabolism*
  • Receptors, Notch / metabolism
  • Tumor Necrosis Factor-alpha / metabolism

Substances

  • NR5A2 protein, human
  • Nr5a2 protein, mouse
  • Receptors, Cytoplasmic and Nuclear
  • Receptors, Notch
  • Tumor Necrosis Factor-alpha