Decreased alpha/beta heterodimer among CD8 molecules of peripheral blood T cells in Wiskott-Aldrich syndrome

Clin Immunol Immunopathol. 1996 Nov;81(2):129-35. doi: 10.1006/clin.1996.0168.

Abstract

Cell surface CD8 molecules exist as either alpha/alpha homodimers or alpha/beta heterodimers, and most CD8+ T cells that undergo differentiation in the thymus express alpha/beta heterodimers. CD8 molecules on peripheral blood lymphocytes of patients with Wiskott-Aldrich syndrome (WAS), an X-linked immunodeficiency disease, have now been shown to be composed mostly of alpha/alpha homodimers, while the total number of lymphocytes was maintained. This observation may indicate aberrant differentiation of CD8+ T cells in the thymus of WAS. Decreased CD8alpha/beta heterodimer expression, and the relative increase of CD8+ T cells expressing CD8alpha/alpha homodimer, may explain a part of the defective lymphocyte functions in WAS patients.

MeSH terms

  • Aging / physiology
  • CD3 Complex / analysis
  • CD8-Positive T-Lymphocytes / chemistry
  • CD8-Positive T-Lymphocytes / cytology*
  • Flow Cytometry / methods
  • Humans
  • Immunoglobulins / blood
  • Infant
  • Killer Cells, Natural / cytology
  • Lymphocyte Count
  • Receptors, Antigen, T-Cell, alpha-beta / analysis
  • Wiskott-Aldrich Syndrome / blood
  • Wiskott-Aldrich Syndrome / immunology
  • Wiskott-Aldrich Syndrome / pathology*

Substances

  • CD3 Complex
  • Immunoglobulins
  • Receptors, Antigen, T-Cell, alpha-beta