Clinical and molecular profile of a new series of patients with immune dysregulation, polyendocrinopathy, enteropathy, X-linked syndrome: Inconsistent.

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Presentation transcript:

Clinical and molecular profile of a new series of patients with immune dysregulation, polyendocrinopathy, enteropathy, X-linked syndrome: Inconsistent correlation between forkhead box protein 3 expression and disease severity Eleonora Gambineri, MD, Lucia Perroni, PhD, Laura Passerini, PhD, Lucia Bianchi, PhD, Claudio Doglioni, MD, Franco Meschi, MD, Riccardo Bonfanti, MD, Yves Sznajer, MD, Alberto Tommasini, MD, Anita Lawitschka, MD, Anne Junker, MD, Desiree Dunstheimer, MD, Peter H. Heidemann, MD, Giantonio Cazzola, MD, Marco Cipolli, MD, Wilhelm Friedrich, MD, Dragana Janic, MD, Nadira Azzi, MD, Erick Richmond, MD, Silvia Vignola, MD, Arrigo Barabino, MD, Giuseppe Chiumello, MD, Chiara Azzari, MD, PhD, Maria-Grazia Roncarolo, MD, PhD, Rosa Bacchetta, MD Journal of Allergy and Clinical Immunology Volume 122, Issue 6, Pages 1105-1112.e1 (December 2008) DOI: 10.1016/j.jaci.2008.09.027 Copyright © 2008 American Academy of Allergy, Asthma & Immunology Terms and Conditions

Fig 1 FOXP3 expression by PBMCs of patients with IPEX syndrome. Percentages of CD4+CD25+ T cells (lymphocyte gate) and CD25+FOXP3+ T cells (CD4+ gate) are reported. Stainings are representative of at least 2 determinations, with the exception of patients 8, 12, and 14. p, Patient. ∗Patients undergoing immunosuppression; †3 years after BMT. Journal of Allergy and Clinical Immunology 2008 122, 1105-1112.e1DOI: (10.1016/j.jaci.2008.09.027) Copyright © 2008 American Academy of Allergy, Asthma & Immunology Terms and Conditions

Fig 2 FOXP3 expression on T-cell activation and in the intestinal mucosa. FOXP3 expression with (thick line) or without (thin line) anti-CD3 (aCD3)/anti-CD28 (aCD28) mAb activation of PBMCs (A) or CD4+ T-cell lines (B). Numbers in the plots represent percentages of FOXP3+ T cells (CD4+ T-cell gate). C, FOXP3+ T cells (red) were detected by means of immunohistochemistry in the intestinal mucosa of patient 12 (F373A). Journal of Allergy and Clinical Immunology 2008 122, 1105-1112.e1DOI: (10.1016/j.jaci.2008.09.027) Copyright © 2008 American Academy of Allergy, Asthma & Immunology Terms and Conditions