Impaired in vitro regulatory T cell function associated with Wiskott-Aldrich syndrome

Details

Serval ID
serval:BIB_63469190065F
Type
Article: article from journal or magazin.
Collection
Publications
Title
Impaired in vitro regulatory T cell function associated with Wiskott-Aldrich syndrome
Journal
Clin Immunol
Author(s)
Adriani M., Aoki J., Horai R., Thornton A. M., Konno A., Kirby M., Anderson S. M., Siegel R. M., Candotti F., Schwartzberg P. L.
ISSN
1521-6616 (Print)
ISSN-L
1521-6616
Publication state
Published
Issued date
07/2007
Volume
124
Number
1
Pages
41-8
Language
english
Notes
Adriani, Marsilio
Aoki, Joseph
Horai, Reiko
Thornton, Angela M
Konno, Akihiro
Kirby, Martha
Anderson, Stacie M
Siegel, Richard M
Candotti, Fabio
Schwartzberg, Pamela L
eng
Z01 AR041133-06/Intramural NIH HHS/
Z01 HG000122-10/Intramural NIH HHS/
Z01 HG000123-10/Intramural NIH HHS/
Z99 HG999999/Intramural NIH HHS/
Research Support, N.I.H., Intramural
Clin Immunol. 2007 Jul;124(1):41-8. Epub 2007 May 18.
Abstract
Wiskott-Aldrich syndrome (WAS) is a primary immunodeficiency characterized by the contradictory coexistence of impaired T-cell function and exaggerated T-cell-mediated pathology, including autoimmunity and eczema. WAS protein (WASp)-deficient mice are also immunodeficient and can develop autoimmune disease. Since defects in regulatory T-cells (Treg) are associated with autoimmunity, we examined the presence and function of these cells in WAS patients and WASp-deficient mice. We found that CD4(+)CD25(+)FOXP3(+) Treg cells can develop in the absence of WASp expression. However, Treg cells both from WASp-deficient mice and from four out of five WAS patients studied showed impaired in vitro suppressor function. In WASp-deficient mice, this defect could be partially rescued by pre-activation with IL-2, suggesting that inadequate cell activation may play a role in WASp-deficient Treg dysfunction. These findings may provide insights into the complex pathophysiology and paradoxical phenotypes of WAS and suggest new therapeutic modalities for autoimmunity in these patients.
Keywords
Adoptive Transfer, Adult, Animals, Autoimmunity/genetics/immunology, CD4-Positive T-Lymphocytes, Child, Preschool, Flow Cytometry, Forkhead Transcription Factors/immunology, Humans, Interleukin-2/immunology, Lymphocyte Activation, Mice, Mice, Knockout, Receptors, Interleukin-2, T-Lymphocytes, Regulatory/*immunology, Wiskott-Aldrich Syndrome/*genetics/*immunology, Wiskott-Aldrich Syndrome Protein/deficiency/genetics/*immunology
Pubmed
Create date
01/11/2017 10:29
Last modification date
20/08/2019 14:19
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