Article (Scientific journals)
Wiskott-Aldrich syndrome protein deficiency in B cells results in impaired peripheral homeostasis
Meyer-Bahlburg, A.; Becker-Herman, S.; Humblet, Stéphanie et al.
2008In Blood, 112 (10), p. 4158-69
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Abstract :
[en] To more precisely identify the B-cell phenotype in Wiskott-Aldrich syndrome (WAS), we used 3 distinct murine in vivo models to define the cell intrinsic requirements for WAS protein (WASp) in central versus peripheral B-cell development. Whereas WASp is dispensable for early bone marrow B-cell development, WASp deficiency results in a marked reduction in each of the major mature peripheral B-cell subsets, exerting the greatest impact on marginal zone and B1a B cells. Using in vivo bromodeoxyuridine labeling and in vitro funtcional assays, we show that these dificits reflect altered peripheral homeostasis, partially resulting from an impairment in integrin function, rather than a developmental defect. Consistent with these observations, we also show that : (1) WASp expression levels increase with cell maturaity, peaking in those subsets exhibiting the greatest sensitivity to WASp deficiency; (2) WASp+ murine B cells exhibit a marked selective advantage beginning at the late transitional B-cell stage; and (3) a similar in vivo selective advantage is manifest by mature WASp+ human B cells. Together, our data provide a better understanding of the clinical phenotype of WAS and suggest that gene therapy might be a useful approach to rescue altered B-cell homeostasis in this disease.
Disciplines :
Hematology
Author, co-author :
Meyer-Bahlburg, A.
Becker-Herman, S.
Humblet, Stéphanie ;  Université de Liège - ULiège > GIGA-R : Hématologie
Khim, S.
Weber, M.
Bouma, G.
Thrasher, A. J.
Batista, F. D.
Rawlings, D. J.
Language :
English
Title :
Wiskott-Aldrich syndrome protein deficiency in B cells results in impaired peripheral homeostasis
Publication date :
2008
Journal title :
Blood
ISSN :
0006-4971
eISSN :
1528-0020
Publisher :
American Society of Hematology, Washington, United States - District of Columbia
Volume :
112
Issue :
10
Pages :
4158-69
Peer reviewed :
Peer Reviewed verified by ORBi
Available on ORBi :
since 27 January 2012

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