Age-Dependent Defects of Regulatory B Cells in Wiskott-Aldrich Syndrome Gene Knockout MiceReportar como inadecuado




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The Wiskott-Aldrich syndrome WAS is a rare X-linked primary immunodeficiency characterized by recurrent infections, thrombocytopenia, eczema, and high incidence of malignancy and autoimmunity. The cellular mechanisms underlying autoimmune complications in WAS have been extensively studied; however, they remain incompletely defined. We investigated the characteristics of IL-10-producing CD19+CD1dhighCD5+ B cells CD1dhighCD5+ Breg obtained from Was gene knockout WKO mice and found that their numbers were significantly lower in these mice compared to wild type WT controls. Moreover, we found a significant age-dependent reduction of the percentage of IL-10-expressing cells in WKO CD1dhighCD5+ Breg cells as compared to age-matched WT control mice. CD1dhighCD5+ Breg cells from older WKO mice did not suppress the in vitro production of inflammatory cytokines from activated CD4+ T cells. Interestingly, CD1dhighCD5+ Breg cells from older WKO mice displayed a basal activated phenotype which may prevent normal cellular responses, among which is the expression of IL-10. These defects may contribute to the susceptibility to autoimmunity with age in patients with WAS.



Autor: Tadafumi Yokoyama, Ayumi Yoshizaki, Karen L. Simon, Martha R. Kirby, Stacie M. Anderson, Fabio Candotti

Fuente: http://plos.srce.hr/



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