کد مقاله کد نشریه سال انتشار مقاله انگلیسی نسخه تمام متن
3258252 1207445 2007 8 صفحه PDF دانلود رایگان
عنوان انگلیسی مقاله ISI
Impaired in vitro regulatory T cell function associated with Wiskott–Aldrich syndrome
موضوعات مرتبط
علوم زیستی و بیوفناوری ایمنی شناسی و میکروب شناسی ایمونولوژی
پیش نمایش صفحه اول مقاله
Impaired in vitro regulatory T cell function associated with Wiskott–Aldrich syndrome
چکیده انگلیسی

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.

ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Clinical Immunology - Volume 124, Issue 1, July 2007, Pages 41–48
نویسندگان
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