Signaling impairments in maternal T cells engrafted in an infant with a novel IL-2 receptor γ mutation Aaruni Khanolkar, MBBS, PhD, Jeffrey D. Wilks,

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Signaling impairments in maternal T cells engrafted in an infant with a novel IL-2 receptor γ mutation Aaruni Khanolkar, MBBS, PhD, Jeffrey D. Wilks, BS, Lawrence J. Jennings, MD, PhD, Jessica L. Davies, BS, Joshua A. Zollett, BS, Loren L. Lott, BS, Erin R. Fullmer, BS, Nicolas E. Bensen, BS, Katrin M. Carlson-Leuer, PhD, William T. Tse, MD, PhD, Ramsay L. Fuleihan, MD Journal of Allergy and Clinical Immunology Volume 135, Issue 4, Pages 1093-1096.e8 (April 2015) DOI: 10.1016/j.jaci.2015.02.012 Copyright © 2015 American Academy of Allergy, Asthma & Immunology Terms and Conditions

Fig 1 Phenotypic and signaling aberrations in patient-derived lymphocytes. A, IL-2Rγ (CD132) surface expression and intracellular JAK3 expression were assessed by means of multiparameter flow cytometry. B, The phosphorylation status of STAT5 and STAT6 was determined by means of Phosflow analysis after IL-2 and IL-4 treatment. C, X-linked inhibitor of apoptosis protein (XIAP) expression was examined in the cytosol of permeabilized lymphocyte subsets. Journal of Allergy and Clinical Immunology 2015 135, 1093-1096.e8DOI: (10.1016/j.jaci.2015.02.012) Copyright © 2015 American Academy of Allergy, Asthma & Immunology Terms and Conditions

Fig E1 Immunologic assessment of lymphocytes in the patient's samples. A, The majority of the T cells in the patient before transplantation display a memory phenotype. B, Maternal origin of T cells in the patient's samples was examined by evaluating HLA-A2 expression. FSC, Forward scatter; SSC, side scatter. C, Maternally derived CD4 T cells display suboptimal CD40L and CD69 upregulation after stimulation. PMA, Phorbol 12-myristate 13-acetate. Journal of Allergy and Clinical Immunology 2015 135, 1093-1096.e8DOI: (10.1016/j.jaci.2015.02.012) Copyright © 2015 American Academy of Allergy, Asthma & Immunology Terms and Conditions