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Prepublished online as a Blood First Edition Paper on September 22, 2003; DOI 10.1182/blood-2003-05-1739.
Submitted May 30, 2003
Accepted September 9, 2003
Differential contribution of Wiskott-Aldrich syndrome protein to selective advantage In T- and B-cell lineages
Akihiro Konno, Taizo Wada, Shepherd H Schurman, Elizabeth K Garabedian, Martha Kirby, Stacie M Anderson, and Fabio Candotti*
Genetics and Molecular Biology Branch, National Human Genome Reseach Institute, National Institutes of Health, Bethesda, MD, USA
Medical Genetics Branch, National Human Genome Research Institute, National institutes of Health, Bethesda, MD, USA
* Corresponding author; email: fabio{at}mail.nih.gov.
Somatic mosaicism due to 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 such 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, while 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.
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