Skewed inhibitory receptors expression in a TAP2-deficient patient

Immunol Lett. 2003 Apr 3;86(2):149-53. doi: 10.1016/s0165-2478(03)00005-1.

Abstract

Most of patients suffering from HLA class I deficiency due to mutations in TAP genes show a significative increase of the peripheral minor Vdelta1+ subpopulation of gammadelta T cells. Surface expression of inhibitory receptors (IR) for HLA class I molecules have been mainly attributed to Vdelta2+ gammadelta T clones. In this study we have analysed the expression of these receptors in both subsets of gammadelta T peripheral lymphocytes. We studied 16 healthy controls and a reported case of homozygous TAP2 mutation with a marked increase of Vdelta1+ gammadelta T cells. MICA/B presence in monocytes was also evaluated. In healthy subjects, the expression of CD94 and CD94/NKG2A was higher in the Vdelta2+ subset but cells bearing the IR ILT2 were found increased in the Vdelta1+. The patient Vdelta2+ gammadelta T cells showed the same IR expression than normal controls, in contrast the Vdelta1+ subset presented a special pattern of very high expression of CD94 and ILT2 and low of CD94/NKG2A. The presence of a new IR poorly represented in healthy individuals could account for the selective increase of Vdelta1+ gammadelta T in TAP-deficient patients. MICA/B surface expression in monocytes was not shifted in our patient.

MeSH terms

  • ATP Binding Cassette Transporter, Subfamily B, Member 3
  • ATP-Binding Cassette Transporters / genetics*
  • Adult
  • Antigens, CD / metabolism
  • Female
  • Granuloma / genetics
  • Granuloma / immunology*
  • Histocompatibility Antigens Class I / analysis
  • Histocompatibility Antigens Class I / metabolism
  • Humans
  • Male
  • Monocytes / immunology
  • Mutation
  • NK Cell Lectin-Like Receptor Subfamily C
  • Receptors, Antigen, T-Cell, gamma-delta / analysis
  • Receptors, Immunologic / analysis
  • Receptors, Immunologic / metabolism*
  • Receptors, Natural Killer Cell
  • Skin Diseases / genetics
  • Skin Diseases / immunology*
  • T-Lymphocyte Subsets / immunology*

Substances

  • ATP Binding Cassette Transporter, Subfamily B, Member 3
  • ATP-Binding Cassette Transporters
  • Antigens, CD
  • Histocompatibility Antigens Class I
  • KLRC1 protein, human
  • MHC class I-related chain A
  • NK Cell Lectin-Like Receptor Subfamily C
  • Receptors, Antigen, T-Cell, gamma-delta
  • Receptors, Immunologic
  • Receptors, Natural Killer Cell
  • TAP2 protein, human