Reduced frequency of CD4(+)CD25(HIGH)FOXP3(+) cells and diminished FOXP3 expression in patients with Common Variable Immunodeficiency: a link to autoimmunity?

Common Variable Immunodeficiency (CVID) is a primary immunodeficiency disease characterized by defective immunoglobulin production and often associated with autoimmunity. We used flow cytometry to analyze CD4(+)CD25(HIGH)FOXP3(+) T regulatory (Treg) cells and ask whether perturbations in their frequ...

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Bibliographic Details
Published in:Clinical immunology (Orlando, Fla.) Fla.), 2009-08, Vol.132 (2), p.215-221
Main Authors: Genre, J, Errante, P R, Kokron, C M, Toledo-Barros, M, Câmara, N O S, Rizzo, L V
Format: Article
Language:English
Subjects:
Adolescent
Adult
Aged
Autoimmunity - immunology
CD4-Positive T-Lymphocytes - cytology
CD4-Positive T-Lymphocytes - immunology
CD4-Positive T-Lymphocytes - metabolism
CD8-Positive T-Lymphocytes - cytology
CD8-Positive T-Lymphocytes - immunology
CD8-Positive T-Lymphocytes - metabolism
Common Variable Immunodeficiency - blood
Common Variable Immunodeficiency - genetics
Common Variable Immunodeficiency - immunology
Female
Flow Cytometry
Forkhead Transcription Factors - genetics
Forkhead Transcription Factors - immunology
Gene Expression
Humans
Interleukin-2 Receptor alpha Subunit - immunology
Lymphocyte Count
Male
Middle Aged
Reverse Transcriptase Polymerase Chain Reaction
T-Lymphocytes, Regulatory - cytology
T-Lymphocytes, Regulatory - immunology
T-Lymphocytes, Regulatory - metabolism
Young Adult
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Summary:Common Variable Immunodeficiency (CVID) is a primary immunodeficiency disease characterized by defective immunoglobulin production and often associated with autoimmunity. We used flow cytometry to analyze CD4(+)CD25(HIGH)FOXP3(+) T regulatory (Treg) cells and ask whether perturbations in their frequency in peripheral blood could underlie the high incidence of autoimmune disorders in CVID patients. In this study, we report for the first time that CVID patients with autoimmune disease have a significantly reduced frequency of CD4(+)CD25(HIGH)FOXP3(+) cells in their peripheral blood accompanied by a decreased intensity of FOXP3 expression. Notably, although CVID patients in whom autoimmunity was not diagnosed had a reduced frequency of CD4(+)CD25(HIGH)FOXP3(+) cells, FOXP3 expression levels did not differ from those in healthy controls. In conclusion, these data suggest compromised homeostasis of CD4(+)CD25(HIGH)FOXP3(+) cells in a subset of CVID patients with autoimmunity, and may implicate Treg cells in pathological mechanisms of CVID.
ISSN:1521-7035
DOI:10.1016/j.clim.2009.03.519