Defective inhibition of B-cell proliferation by Wiskott-Aldrich syndrome protein-deficient regulatory T cells

Details

Serval ID
serval:BIB_B08045F8F84C
Type
Article: article from journal or magazin.
Collection
Publications
Title
Defective inhibition of B-cell proliferation by Wiskott-Aldrich syndrome protein-deficient regulatory T cells
Journal
Blood
Author(s)
Adriani M., Jones K. A., Uchiyama T., Kirby M. R., Silvin C., Anderson S. M., Candotti F.
ISSN
1528-0020 (Electronic)
ISSN-L
0006-4971
Publication state
Published
Issued date
2011
Volume
117
Number
24
Pages
6608-11
Language
english
Notes
Adriani, Marsilio
Jones, Krysten A
Uchiyama, Toru
Kirby, Martha R
Silvin, Christopher
Anderson, Stacie M
Candotti, Fabio
eng
Blood. 2011 Jun 16;117(24):6608-11. doi: 10.1182/blood-2010-12-322834. Epub 2011 Apr 22.
Abstract
Wiskott-Aldrich syndrome (WAS) is an inherited immunodeficiency characterized by high incidence of autoantibody-mediated autoimmune complications. Such a feature has been associated with defective suppressor activity of WAS protein-deficient, naturally occurring CD4(+)CD25(+)Foxp3(+) regulatory T cells on responder T cells. However, it remains to be established whether the altered B-cell tolerance reported in WAS patients and Was knockout (WKO) mice is secondary to abnormalities in the direct suppression of B-cell function by nTreg cells or to impaired regulation of T-helper function. Because activated nTreg cells are known to induce granzyme B-mediated B-cell killing, we decided to evaluate the regulatory capabilities of WKO nTregs on B lymphocytes. We found that preactivated WKO nTreg cells failed to effectively suppress B-cell proliferation and that such a defect was associated with reduced killing of B cells and significantly decreased degranulation of granzyme B. Altogether, these results provide additional mechanistic insights into the loss of immune tolerance in WAS.
Keywords
Animals, B-Lymphocytes/metabolism/*physiology, Cell Death/genetics/immunology, Cell Degranulation/genetics/immunology, *Cell Proliferation, Cells, Cultured, Down-Regulation/genetics/immunology, Granzymes/metabolism, Immune Tolerance/genetics/immunology, Lymphocyte Activation/genetics/immunology, Mice, Mice, Knockout, T-Lymphocytes, Regulatory/metabolism/*physiology, Wiskott-Aldrich Syndrome Protein/deficiency/*genetics/metabolism
Pubmed
Open Access
Yes
Create date
01/11/2017 10:29
Last modification date
20/08/2019 15:19
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