Animal models of HLA-B27-associated diseases: new outcomes

Joint Bone Spine. 2006 Mar;73(2):132-8. doi: 10.1016/j.jbspin.2005.03.016. Epub 2005 Aug 22.

Abstract

The HLA-B27 molecule is strongly associated with the spondyloarthropathies, a group of chronic inflammatory diseases, affecting the skeleton, the bowel and the skin. This association has been largely studied, but mechanisms of pathology remain unclear. The HLA-B27 transgenic rats develop a spontaneous disease that strikingly resembles human spondyloarthropathies, dependent of bacterial flora and implicating the immune system. The presence of CD4+ T cells is required, and antigen presenting cells (APC) expressing high levels of HLA-B27 likely play an important role. Indeed, APC are defective in naive T lymphocytes stimulation. This default appears to implicate the APC/T cells contact, and may result in a loss of tolerance toward microbial flora. Two models of skeletal inflammation linked to HLA-B27 have been developed in mice. The ANKENT mice develop a spontaneous ossifying enthesitis affecting ankle and tarsal joints, with increased frequency in the presence of an HLA-B27 transgene. The HLA-B27 transgenic mice lacking endogenous beta2 microglobulin develop arthritis of hind-paws. In this model, homodimers of B27 heavy chains could be implicated in the pathogenesis by presenting exogenous peptides to CD4+ T cells.

Publication types

  • Review

MeSH terms

  • Animals
  • Animals, Genetically Modified
  • Disease Models, Animal*
  • HLA-B27 Antigen / genetics*
  • HLA-B27 Antigen / immunology*
  • Mice
  • Mice, Knockout / genetics
  • Rats
  • Rats, Inbred Strains / genetics
  • Spondylarthropathies / genetics*
  • Spondylarthropathies / immunology*

Substances

  • HLA-B27 Antigen