CD25 deficiency causes an immune dysregulation, polyendocrinopathy, enteropathy, X- linked–like syndrome, and defective IL-10 expression from CD4 lymphocytes

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Presentation transcript:

CD25 deficiency causes an immune dysregulation, polyendocrinopathy, enteropathy, X- linked–like syndrome, and defective IL-10 expression from CD4 lymphocytes Amy A. Caudy, PhD, Sreelatha T. Reddy, PhD, Talal Chatila, MD, John P. Atkinson, MD, James W. Verbsky, MD, PhD Journal of Allergy and Clinical Immunology Volume 119, Issue 2, Pages 482-487 (February 2007) DOI: 10.1016/j.jaci.2006.10.007 Copyright © 2007 American Academy of Allergy, Asthma & Immunology Terms and Conditions

Fig 1 CD25 deficiency results in defective IL-10 expression from CD4 cells, but is not required for Foxp3+ TR generation. A, CD4 lymphocytes from a Foxp3 mutant (Foxp3–) and a Foxp3 normal (Foxp3+) patient were activated with IL-2 (10 U/mL) for 3 days, and IL-10 was assayed by ELISA. B, CD4 lymphocytes from control and the Foxp3+ patient were activated for 3 days and stained with antibodies to CD25 and CD69. C, Sequence trace of maternal allele showing single adenine insertion (∗) after position 692 (top). Sequence trace of genomic DNA showing C to T substitution at position 301 (∗) of paternal allele (bottom). D, PBMCs from the Foxp3+ patient were isolated and stained with antibodies to CD4 and Foxp3. Journal of Allergy and Clinical Immunology 2007 119, 482-487DOI: (10.1016/j.jaci.2006.10.007) Copyright © 2007 American Academy of Allergy, Asthma & Immunology Terms and Conditions

Fig 2 IL-15 and high-dose IL-2 rescue the proliferative defect of a CD25-deficient patient. CD4 lymphocytes were activated for 3 days and (A) proliferation measured by 3H-thymidine incorporation and (B) CD25 expression measured by flow cytometry. C, CD4 lymphocytes were isolated and stimulated as shown, and CD25 expression was measured 3 days later. D, CD4 lymphocytes were activated for 3 days, and RT-PCR products of the CD25 gene were concentrated and digested with BbsI. MFI, Mean fluorescence intensity; 1 KB, 1 KB molecular weight marker. Journal of Allergy and Clinical Immunology 2007 119, 482-487DOI: (10.1016/j.jaci.2006.10.007) Copyright © 2007 American Academy of Allergy, Asthma & Immunology Terms and Conditions