The role of HLA-DQ8 beta57 polymorphism in the anti-gluten T-cell response in coeliac disease

Nature. 2008 Nov 27;456(7221):534-8. doi: 10.1038/nature07524.

Abstract

Major histocompatibility complex (MHC) class II alleles HLA-DQ8 and the mouse homologue I-A(g7) lacking a canonical aspartic acid residue at position beta57 are associated with coeliac disease and type I diabetes. However, the role of this single polymorphism in disease initiation and progression remains poorly understood. The lack of Asp 57 creates a positively charged P9 pocket, which confers a preference for negatively charged peptides. Gluten lacks such peptides, but tissue transglutaminase (TG2) introduces negatively charged residues at defined positions into gluten T-cell epitopes by deamidating specific glutamine residues on the basis of their spacing to proline residues. The commonly accepted model, proposing that HLA-DQ8 simply favours binding of negatively charged peptides, does not take into account the fact that TG2 requires inflammation for activation and that T-cell responses against native gluten peptides are found, particularly in children. Here we show that beta57 polymorphism promotes the recruitment of T-cell receptors bearing a negative signature charge in the complementary determining region 3beta (CDR3beta) during the response against native gluten peptides presented by HLA-DQ8 in coeliac disease. These T cells showed a crossreactive and heteroclitic (stronger) response to deamidated gluten peptides. Furthermore, gluten peptide deamidation extended the T-cell-receptor repertoire by relieving the requirement for a charged residue in CDR3beta. Thus, the lack of a negative charge at position beta57 in MHC class II was met by negatively charged residues in the T-cell receptor or in the peptide, the combination of which might explain the role of HLA-DQ8 in amplifying the T-cell response against dietary gluten.

Publication types

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

MeSH terms

  • Amides / chemistry
  • Animals
  • CD4-Positive T-Lymphocytes / immunology*
  • Celiac Disease / genetics*
  • Celiac Disease / immunology*
  • Complementarity Determining Regions / chemistry
  • Complementarity Determining Regions / immunology
  • Cross Reactions
  • Epitopes, T-Lymphocyte / chemistry
  • Epitopes, T-Lymphocyte / immunology
  • Gliadin / chemistry
  • Gliadin / immunology
  • Glutens / chemistry
  • Glutens / immunology*
  • HLA-DQ Antigens / chemistry
  • HLA-DQ Antigens / genetics*
  • HLA-DQ Antigens / immunology
  • Humans
  • Hybridomas / immunology
  • Mice
  • Mice, Transgenic
  • Polymorphism, Genetic / genetics*
  • Receptors, Antigen, T-Cell / chemistry
  • Receptors, Antigen, T-Cell / immunology
  • Static Electricity

Substances

  • Amides
  • Complementarity Determining Regions
  • Epitopes, T-Lymphocyte
  • HLA-DQ Antigens
  • HLA-DQ8 antigen
  • Receptors, Antigen, T-Cell
  • Glutens
  • Gliadin