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Human phagocyte defect caused by a Rac2 mutation detected by means of neonatal screening for T-cell lymphopenia  Deborah Accetta, MD, Grant Syverson,

Published byLeslie Beasley Modified over 5 years ago

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Presentation on theme: "Human phagocyte defect caused by a Rac2 mutation detected by means of neonatal screening for T-cell lymphopenia  Deborah Accetta, MD, Grant Syverson,"— Presentation transcript:

1 Human phagocyte defect caused by a Rac2 mutation detected by means of neonatal screening for T-cell lymphopenia  Deborah Accetta, MD, Grant Syverson, MD, Benedetta Bonacci, MS, Sreelatha Reddy, PhD, Christine Bengtson, BS, Jill Surfus, BS, Ronald Harbeck, PhD, Anna Huttenlocher, MD, William Grossman, MD, John Routes, MD, James Verbsky, MD, PhD  Journal of Allergy and Clinical Immunology  Volume 127, Issue 2, Pages e2 (February 2011) DOI: /j.jaci Copyright © 2010 American Academy of Allergy, Asthma & Immunology Terms and Conditions

2 Fig 1 A, Neutrophil migration of control (C) or patient (P) neutrophils in response to buffer or zymosan activated serum (ZAS) using a Boyden Chamber. The shaded areas show the range of normal. B, Oxidative burst of control and patient neutrophils in response to PMA or fMLP. DHR, dihydrorhodamine. C, Neutrophil adhesion to fibronectin following TNF-α or fMLP activation. ∗∗∗P < .001, ∗∗P < .01, ∗P < .05. D, Actin staining of neutrophils following stimulation with fMLP. Notice the polarization and actin cap formation in control neutrophils (solid arrows), and disorganized pattern of actin without evidence of polarization in the patient's neutrophils (dashed arrows). Journal of Allergy and Clinical Immunology  , e2DOI: ( /j.jaci ) Copyright © 2010 American Academy of Allergy, Asthma & Immunology Terms and Conditions

3 Fig 2 A, Phase contrast photography of patient or control PBMCs activated with PHA for 24 hours (top). B, Control PBMCs were PHA activated without (left) or with (right) CD18 blocking antibodies for 24 hours. Arrows indicated integrin dependent aggregation of lymphocytes (dark arrows). Journal of Allergy and Clinical Immunology  , e2DOI: ( /j.jaci ) Copyright © 2010 American Academy of Allergy, Asthma & Immunology Terms and Conditions

4 A, Intracellular calcium levels of patient and control lymphocytes following CD3 and CD28 activation. B, Lymphocyte proliferation in response to CD3 antibodies or Concanavalin A (Con A) by carboxyfluorescein succinimidyl ester (CFSE) dilution. Journal of Allergy and Clinical Immunology  , e2DOI: ( /j.jaci ) Copyright © 2010 American Academy of Allergy, Asthma & Immunology Terms and Conditions

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