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

Details

Serval ID
serval:BIB_9D2580D8653A
Type
Article: article from journal or magazin.
Publication sub-type
Case report (case report): feedback on an observation with a short commentary.
Collection
Publications
Title
Differential contribution of Wiskott-Aldrich syndrome protein to selective advantage in T- and B-cell lineages
Journal
Blood
Author(s)
Konno A., Wada T., Schurman S. H., Garabedian E. K., Kirby M., Anderson S. M., Candotti F.
ISSN
0006-4971 (Print)
ISSN-L
0006-4971
Publication state
Published
Issued date
2004
Volume
103
Number
2
Pages
676-8
Language
english
Notes
Konno, Akihiro
Wada, Taizo
Schurman, Shepherd H
Garabedian, Elizabeth K
Kirby, Martha
Anderson, Stacie M
Candotti, Fabio
eng
Blood. 2004 Jan 15;103(2):676-8. 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.
Keywords
B-Lymphocytes/*immunology, Chromosomes, Human, X/genetics, DNA/genetics/isolation & purification, Female, Flow Cytometry, Humans, Proteins/*genetics/*immunology, T-Lymphocytes/*immunology, Wiskott-Aldrich Syndrome/genetics/*immunology, Wiskott-Aldrich Syndrome Protein
Pubmed
Open Access
Yes
Create date
01/11/2017 11:29
Last modification date
20/08/2019 16:03
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