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Blood, 15 January 2004, Vol. 103, No. 2, pp. 676-678. Prepublished online as a Blood First Edition Paper on September 22, 2003; DOI 10.1182/blood-2003-05-1739.
IMMUNOBIOLOGY Differential contribution of Wiskott-Aldrich syndrome protein to selective advantage in T- and B-cell lineagesFrom the Genetics and Molecular Biology Branch, National Human Genome Research Institute, National Institutes of Health (NIH); and the Medical Genetics Branch, National Human Genome Research Institute, NIH, Bethesda, MD.
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.
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