Presentation is loading. Please wait.

Presentation is loading. Please wait.

Persistence of natural killer cells with expansion of a hypofunctional CD56−CD16+KIR+NKG2C+ subset in a patient with atypical Janus kinase 3–deficient.

Published byAnn-Sofie Hedlund Modified over 5 years ago

Similar presentations

Presentation on theme: "Persistence of natural killer cells with expansion of a hypofunctional CD56−CD16+KIR+NKG2C+ subset in a patient with atypical Janus kinase 3–deficient."— Presentation transcript:

1 Persistence of natural killer cells with expansion of a hypofunctional CD56−CD16+KIR+NKG2C+ subset in a patient with atypical Janus kinase 3–deficient severe combined immunodeficiency  Laure Farnault, MD, Hervé Chambost, MD, Gérard Michel, MD, Isabelle Thuret, MD, Geneviève de Saint Basile, MD, Alain Fischer, MD, PhD, Capucine Picard, MD, PhD, Christophe Picard, MD, Florence Orlanducci, Catherine Farnarier, MD, Alessandro Moretta, MD, PhD, Daniel Olive, MD, PhD  Journal of Allergy and Clinical Immunology  Volume 131, Issue 4, Pages e2 (April 2013) DOI: /j.jaci Copyright © 2012 American Academy of Allergy, Asthma & Immunology Terms and Conditions

2 Fig 1 Phenotypic characteristics of NK cell subsets. A, Dot plots show NK cell subsets as defined by CD56 and CD16 expression in our patient and in representative age-matched control subjects. The represented cells were gated on CD3− lymphocytes. B, Histograms of CD158b, NKG2A, NKG2C, and Siglec-7 expression on the CD56−CD16+ NK cell subset of patient. C, Graphs summarizing the expression of various markers in CD56−CD16+ and CD56dimCD16+ NK cell subsets of the patient (black bars) and control subjects (white bars; n = 4 age-matched healthy volunteers). D, Dot plot showing the expression of CD56 and Siglec-7 in NK cells of our patient and of a representative control subject. Cells were gated on Siglec-7-aminoactinomycin D–negative, CD19−, CD14−, and CD3− lymphocytes. Journal of Allergy and Clinical Immunology  , e2DOI: ( /j.jaci ) Copyright © 2012 American Academy of Allergy, Asthma & Immunology Terms and Conditions

3 Fig 2 Functional capacity of CD56dimCD16+ and CD56−CD16+ NK cell subsets. The degranulation activity (A) and cytokine secretion capacity (B) of CD56dimCD16+ and CD56−CD16+ NK cell subsets of the patient (black bars) and the control group (n = 3, gray bars) are shown. The graph represents the percentage of degranulation measured by using CD107 expression of NK subsets after stimulation with the negative control P815, K562, and P815 coated with anti-CD16 mAb. C, Histograms of IFN-γ secretion and CD107 expression by CD56−CD16+ and CD56dimCD16+ NK cell subsets after stimulation with P815 coated with anti-NKG2C mAb. Journal of Allergy and Clinical Immunology  , e2DOI: ( /j.jaci ) Copyright © 2012 American Academy of Allergy, Asthma & Immunology Terms and Conditions

4 Fig E1 Functionality of JAK3 protein. A, Histogram of JAK3 protein expression on NK cells of the patient and his mother. B, NK cell activation through IL-2 and IL-15 stimulation through the JAK3 and subsequently signal transducer and activator of transcription 5 pathway. Histograms show increased expression of the proliferation-associated molecule Ki67, upregulation of NKp44, and increased cell division evaluated with Cell Trace (Invitrogen, Carlsbad, Calif) in the patient’s mother's NK cells conversely to the patient’s NK cells, which exhibited only weak or no response to IL-2 plus IL-15 stimulation. Journal of Allergy and Clinical Immunology  , e2DOI: ( /j.jaci ) Copyright © 2012 American Academy of Allergy, Asthma & Immunology Terms and Conditions

Download ppt "Persistence of natural killer cells with expansion of a hypofunctional CD56−CD16+KIR+NKG2C+ subset in a patient with atypical Janus kinase 3–deficient."

Similar presentations

Differentiation stage determines pathologic and protective allergen-specific CD4+ T-cell outcomes during specific immunotherapy  Erik Wambre, PhD, Jonathan.

Differentiation stage determines pathologic and protective allergen-specific CD4+ T-cell outcomes during specific immunotherapy  Erik Wambre, PhD, Jonathan.
The loss of IgM memory B cells correlates with clinical disease in common variable immunodeficiency  Rita Carsetti, MD, Maria Manuela Rosado, PhD, Simona.

The loss of IgM memory B cells correlates with clinical disease in common variable immunodeficiency  Rita Carsetti, MD, Maria Manuela Rosado, PhD, Simona.
Defective calcium signaling and disrupted CD20–B-cell receptor dissociation in patients with common variable immunodeficiency disorders  Annick A.J.M.

Defective calcium signaling and disrupted CD20–B-cell receptor dissociation in patients with common variable immunodeficiency disorders  Annick A.J.M.
Defective natural killer–cell cytotoxic activity in NFKB2-mutated CVID-like disease  Vassilios Lougaris, MD, Giovanna Tabellini, PhD, Massimiliano Vitali,

Defective natural killer–cell cytotoxic activity in NFKB2-mutated CVID-like disease  Vassilios Lougaris, MD, Giovanna Tabellini, PhD, Massimiliano Vitali,
Margaretha A. Faber, MD, Vito Sabato, MD, Chris H

Margaretha A. Faber, MD, Vito Sabato, MD, Chris H
Homozygous N-terminal missense mutation in TRNT1 leads to progressive B-cell immunodeficiency in adulthood  Glynis Frans, MPharm, Leen Moens, PhD, Heidi.

Homozygous N-terminal missense mutation in TRNT1 leads to progressive B-cell immunodeficiency in adulthood  Glynis Frans, MPharm, Leen Moens, PhD, Heidi.
Successful allogeneic hematopoietic stem cell transplantation for DOCK8 deficiency  Vincent Barlogis, MD, Claire Galambrun, MD, Hervé Chambost, MD, Sylvie.

Successful allogeneic hematopoietic stem cell transplantation for DOCK8 deficiency  Vincent Barlogis, MD, Claire Galambrun, MD, Hervé Chambost, MD, Sylvie.
IgE cross-linking impairs monocyte antiviral responses and inhibits influenza-driven TH1 differentiation  Regina K. Rowe, MD, PhD, David M. Pyle, MD,

IgE cross-linking impairs monocyte antiviral responses and inhibits influenza-driven TH1 differentiation  Regina K. Rowe, MD, PhD, David M. Pyle, MD,
A novel mutation in CD132 causes X-CID with defective T-cell activation and impaired humoral reactivity  Taco W. Kuijpers, MD, PhD, Paul A. Baars, PhD,

A novel mutation in CD132 causes X-CID with defective T-cell activation and impaired humoral reactivity  Taco W. Kuijpers, MD, PhD, Paul A. Baars, PhD,
Human B cells promote T-cell plasticity to optimize antibody response by inducing coexpression of TH1/TFH signatures  Jelle de Wit, PhD, Tineke Jorritsma,

Human B cells promote T-cell plasticity to optimize antibody response by inducing coexpression of TH1/TFH signatures  Jelle de Wit, PhD, Tineke Jorritsma,
Immunologic defects in 22q11.2 deletion syndrome

Immunologic defects in 22q11.2 deletion syndrome
Toll-like receptor 7–induced naive human B-cell differentiation and immunoglobulin production  Mark C. Glaum, MD, PhD, Shilpi Narula, MD, Decheng Song,

Toll-like receptor 7–induced naive human B-cell differentiation and immunoglobulin production  Mark C. Glaum, MD, PhD, Shilpi Narula, MD, Decheng Song,
Bruton tyrosine kinase mediates TLR9-dependent human dendritic cell activation  Vassilios Lougaris, MD, Manuela Baronio, PhD, Massimiliano Vitali, PhD,

Bruton tyrosine kinase mediates TLR9-dependent human dendritic cell activation  Vassilios Lougaris, MD, Manuela Baronio, PhD, Massimiliano Vitali, PhD,
Taco W. Kuijpers, MD, PhD, Paul A. Baars, PhD, Daan J

Taco W. Kuijpers, MD, PhD, Paul A. Baars, PhD, Daan J
T-cell regulation during viral and nonviral asthma exacerbations

T-cell regulation during viral and nonviral asthma exacerbations
Anti–IL-5 (mepolizumab) therapy reduces eosinophil activation ex vivo and increases IL- 5 and IL-5 receptor levels  Miguel L. Stein, MD, Joyce M. Villanueva,

Anti–IL-5 (mepolizumab) therapy reduces eosinophil activation ex vivo and increases IL- 5 and IL-5 receptor levels  Miguel L. Stein, MD, Joyce M. Villanueva,
Type 2 innate lymphoid cells in induced sputum from children with severe asthma  Prasad Nagakumar, MBBS, Laura Denney, PhD, Louise Fleming, MD, Andrew.

Type 2 innate lymphoid cells in induced sputum from children with severe asthma  Prasad Nagakumar, MBBS, Laura Denney, PhD, Louise Fleming, MD, Andrew.
Reduction of CRKL expression in patients with partial DiGeorge syndrome is associated with impairment of T-cell functions  Mauro Giacomelli, PhD, Rajesh.

Reduction of CRKL expression in patients with partial DiGeorge syndrome is associated with impairment of T-cell functions  Mauro Giacomelli, PhD, Rajesh.
Identification of a subset of human natural killer cells expressing high levels of programmed death 1: A phenotypic and functional characterization  Silvia.

Identification of a subset of human natural killer cells expressing high levels of programmed death 1: A phenotypic and functional characterization  Silvia.
Type 3 innate lymphoid cells induce proliferation of CD94+ natural killer cells  Shuo Li, PhD, Hideaki Morita, MD, PhD, Beate Rückert, Sci Tec, Tadech.

Type 3 innate lymphoid cells induce proliferation of CD94+ natural killer cells  Shuo Li, PhD, Hideaki Morita, MD, PhD, Beate Rückert, Sci Tec, Tadech.

Similar presentations

Ads by Google