Regulation and function of the interleukin 13 receptor alpha 2 during a T helper cell type 2-dominant immune response

J Exp Med. 2003 Mar 17;197(6):687-701. doi: 10.1084/jem.20020903.

Abstract

Highly polarized type 2 cytokine responses can be harmful and even lethal to the host if they are too vigorous or persist too long. Therefore, it is important to elucidate the mechanisms that down-regulate these reactions. Interleukin (IL)-13 has emerged as a central mediator of T helper cell (Th)2-dominant immune responses, exhibiting a diverse array of functional activities including regulation of airway hyperreactivity, resistance to nematode parasites, and tissue remodeling and fibrosis. Here, we show that IL-13 receptor (R)alpha2 is a critical down-regulatory factor of IL-13-mediated tissue fibrosis induced by the parasitic helminth Schistosoma mansoni. IL-13Ralpha2 expression was induced after the onset of the fibrotic response, IL-10, IL-13, and Stat6 dependent, and inhibited by the Th1-inducing adjuvant IL-12. Strikingly, schistosome-infected C57BL/6 and BALB/c IL-13Ralpha2-deficient mice showed a marked exacerbation in hepatic fibrosis, despite displaying no change in granuloma size, tissue eosinophilia, or mastocytosis. Fibrosis increased despite the fact that IL-13 levels decreased significantly in the liver and serum. Importantly, pathology was prevented when IL-13Ralpha2-deficient mice were treated with a soluble IL-13Ralpha2-Fc construct, formally demonstrating that their exacerbated fibrotic response was due to heightened IL-13 activity. Together, these studies illustrate the central role played by the IL-13Ralpha2 in the down-regulation of a chronic and pathogenic Th2-mediated immune response.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Animals
  • Down-Regulation
  • Female
  • Humans
  • Interleukin-13 / immunology
  • Interleukin-13 / physiology*
  • Interleukin-13 Receptor alpha1 Subunit
  • Liver / immunology
  • Liver / metabolism
  • Liver / parasitology
  • Liver / pathology
  • Liver Cirrhosis / immunology*
  • Liver Cirrhosis / pathology
  • Mice
  • Mice, Inbred BALB C
  • Mice, Inbred C57BL
  • Mice, Knockout
  • Receptors, Interleukin / genetics
  • Receptors, Interleukin / immunology
  • Receptors, Interleukin / metabolism*
  • Receptors, Interleukin-13
  • Receptors, Interleukin-4 / genetics
  • Receptors, Interleukin-4 / immunology
  • Receptors, Interleukin-4 / metabolism
  • Schistosoma mansoni / immunology
  • Schistosomiasis mansoni / immunology*
  • Schistosomiasis mansoni / pathology
  • Th2 Cells / immunology*

Substances

  • IL13RA1 protein, human
  • Il13ra1 protein, mouse
  • Interleukin-13
  • Interleukin-13 Receptor alpha1 Subunit
  • Receptors, Interleukin
  • Receptors, Interleukin-13
  • Receptors, Interleukin-4