Differential contribution of Wiskott-Aldrich syndrome protein to selective advantage in T- and B-cell lineages

Blood. 2004 Jan 15;103(2):676-8. doi: 10.1182/blood-2003-05-1739. Epub 2003 Sep 22.

Abstract

Somatic mosaicism because of in vivo reversion has been recently reported in a small number of patients affected with Wiskott-Aldrich syndrome (WAS). Flow cytometry analysis of WAS protein (WASP) expression has shown that these patients carried revertant cells only among T lymphocytes. Here, we have used high-resolution capillary electrophoresis to analyze genomic DNA from highly purified cells of one of these patients and detected revertant sequences also within the B-cell fraction. The demonstration of revertant cells among both T and B lymphocytes in this patient is consistent with the reversion event having occurred in a common lymphoid progenitor. However, although WASP-expressing T cells showed selective advantage and were readily detectable in the periphery of the mosaic patient, revertant B lymphocytes remained below the detection threshold of flow cytometry. These findings suggest that, contrary to T cells, differentiation and survival of B lymphocytes is minimally dependent on WASP.

MeSH terms

  • B-Lymphocytes / immunology*
  • Chromosomes, Human, X / genetics
  • DNA / genetics
  • DNA / isolation & purification
  • Female
  • Flow Cytometry
  • Humans
  • Proteins / genetics*
  • Proteins / immunology*
  • T-Lymphocytes / immunology*
  • Wiskott-Aldrich Syndrome / genetics
  • Wiskott-Aldrich Syndrome / immunology*
  • Wiskott-Aldrich Syndrome Protein

Substances

  • Proteins
  • WAS protein, human
  • Wiskott-Aldrich Syndrome Protein
  • DNA