Jordan S Orange

Summary

Affiliation: University of Pennsylvania
Country: USA

Publications

  1. pmc IL-2 induces a WAVE2-dependent pathway for actin reorganization that enables WASp-independent human NK cell function
    Jordan S Orange
    Children s Hospital of Philadelphia Research Institute, Philadelphia, Pennsylvania, USA
    J Clin Invest 121:1535-48. 2011
  2. pmc Unraveling human natural killer cell deficiency
    Jordan S Orange
    University of Pennsylvania School of Medicine, Department of Pediatrics, Children s Hospital of Philadelphia Research Institute, Philadelphia, Pennsylvania 19104, USA
    J Clin Invest 122:798-801. 2012
  3. pmc Phosphorylation of the myosin IIA tailpiece regulates single myosin IIA molecule association with lytic granules to promote NK-cell cytotoxicity
    Keri B Sanborn
    Immunology Graduate Group, University of Pennsylvania School of Medicine, Philadelphia, PA, USA
    Blood 118:5862-71. 2011
  4. pmc Natural killer cell lytic granule secretion occurs through a pervasive actin network at the immune synapse
    Gregory D Rak
    University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania, United States of America
    PLoS Biol 9:e1001151. 2011
  5. pmc Rapid lytic granule convergence to the MTOC in natural killer cells is dependent on dynein but not cytolytic commitment
    Ashley N Mentlik
    Department of Pediatrics and Physiology, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA
    Mol Biol Cell 21:2241-56. 2010
  6. pmc Hypomorphic nuclear factor-kappaB essential modulator mutation database and reconstitution system identifies phenotypic and immunologic diversity
    Eric P Hanson
    Division of Rheumatology, Joseph Stokes Jr Research Institute, Children s Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA
    J Allergy Clin Immunol 122:1169-1177.e16. 2008
  7. pmc Rapid activation receptor- or IL-2-induced lytic granule convergence in human natural killer cells requires Src, but not downstream signaling
    Ashley Mentlik James
    Graduate Program in Cell Biology and Physiology, University of Pennsylvania School of Medicine, Philadelphia, PA, USA
    Blood 121:2627-37. 2013
  8. pmc Myosin IIA associates with NK cell lytic granules to enable their interaction with F-actin and function at the immunological synapse
    Keri B Sanborn
    Immunology Graduate Group, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA
    J Immunol 182:6969-84. 2009
  9. doi request reprint Analysis of the NK cell immunological synapse
    Keri B Sanborn
    Immunology Graduate Group, Division of Immunology, Children s Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, PA, USA
    Methods Mol Biol 612:127-48. 2010
  10. pmc Pro-asthmatic cytokines regulate unliganded and ligand-dependent glucocorticoid receptor signaling in airway smooth muscle
    Aihua Hu
    Division of Pulmonary Medicine, Children s Hospital of Philadelphia Research Institute, University of Pennsylvania Perlman School of Medicine, Philadelphia, Pennsylvania, United States of America
    PLoS ONE 8:e60452. 2013

Detail Information

Publications55

  1. pmc IL-2 induces a WAVE2-dependent pathway for actin reorganization that enables WASp-independent human NK cell function
    Jordan S Orange
    Children s Hospital of Philadelphia Research Institute, Philadelphia, Pennsylvania, USA
    J Clin Invest 121:1535-48. 2011
    ..These results demonstrate how overlapping cytoskeletal activities can utilize immunologically distinct pathways to achieve synonymous immune function...
  2. pmc Unraveling human natural killer cell deficiency
    Jordan S Orange
    University of Pennsylvania School of Medicine, Department of Pediatrics, Children s Hospital of Philadelphia Research Institute, Philadelphia, Pennsylvania 19104, USA
    J Clin Invest 122:798-801. 2012
    ..These reports suggest a critical role for the minichromosome maintenance helicase complex in NK cells and NK cell-mediated host defense...
  3. pmc Phosphorylation of the myosin IIA tailpiece regulates single myosin IIA molecule association with lytic granules to promote NK-cell cytotoxicity
    Keri B Sanborn
    Immunology Graduate Group, University of Pennsylvania School of Medicine, Philadelphia, PA, USA
    Blood 118:5862-71. 2011
    ....
  4. pmc Natural killer cell lytic granule secretion occurs through a pervasive actin network at the immune synapse
    Gregory D Rak
    University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania, United States of America
    PLoS Biol 9:e1001151. 2011
    ....
  5. pmc Rapid lytic granule convergence to the MTOC in natural killer cells is dependent on dynein but not cytolytic commitment
    Ashley N Mentlik
    Department of Pediatrics and Physiology, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA
    Mol Biol Cell 21:2241-56. 2010
    ..This defines a novel paradigm for rapid MTOC-directed transport as a prerequisite for directed secretion, one that may prepare, but not commit cells for precision secretory function...
  6. pmc Hypomorphic nuclear factor-kappaB essential modulator mutation database and reconstitution system identifies phenotypic and immunologic diversity
    Eric P Hanson
    Division of Rheumatology, Joseph Stokes Jr Research Institute, Children s Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA
    J Allergy Clin Immunol 122:1169-1177.e16. 2008
    ..Human hypomorphic nuclear factor-kappaB essential modulator (NEMO) mutations cause diverse clinical and immunologic phenotypes, but understanding of their scope and mechanistic links to immune function and genotype is incomplete...
  7. pmc Rapid activation receptor- or IL-2-induced lytic granule convergence in human natural killer cells requires Src, but not downstream signaling
    Ashley Mentlik James
    Graduate Program in Cell Biology and Physiology, University of Pennsylvania School of Medicine, Philadelphia, PA, USA
    Blood 121:2627-37. 2013
    ....
  8. pmc Myosin IIA associates with NK cell lytic granules to enable their interaction with F-actin and function at the immunological synapse
    Keri B Sanborn
    Immunology Graduate Group, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA
    J Immunol 182:6969-84. 2009
    ....
  9. doi request reprint Analysis of the NK cell immunological synapse
    Keri B Sanborn
    Immunology Graduate Group, Division of Immunology, Children s Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, PA, USA
    Methods Mol Biol 612:127-48. 2010
    ..Here we describe microscopy-based methods for evaluating and quantifying the NK cell immunological synapse that can be useful in enabling experimental studies...
  10. pmc Pro-asthmatic cytokines regulate unliganded and ligand-dependent glucocorticoid receptor signaling in airway smooth muscle
    Aihua Hu
    Division of Pulmonary Medicine, Children s Hospital of Philadelphia Research Institute, University of Pennsylvania Perlman School of Medicine, Philadelphia, Pennsylvania, United States of America
    PLoS ONE 8:e60452. 2013
    ....
  11. pmc Differential localization of T-bet and Eomes in CD8 T cell memory populations
    Laura M McLane
    Department of Microbiology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA 19104, USA
    J Immunol 190:3207-15. 2013
    ..Together, these findings suggest a novel model for CD8 T cell differentiation in humans that is based on the localization of T-bet and Eomes...
  12. pmc Cutting edge: association with I kappa B kinase beta regulates the subcellular localization of Homer3
    Gayatri Yatherajam
    Department of Animal Biology, School of Veterinary Medicine, University of Pennsylvania, Philadelphia, PA 19104, USA
    J Immunol 185:2665-9. 2010
    ..These findings identify a novel interaction between two major signaling proteins and reveal an unexpected NF-kappaB-independent function for the IKK complex in regulating the subcellular localization of Homer3...
  13. pmc Genetic sharing and heritability of paediatric age of onset autoimmune diseases
    Yun R Li
    Center for Applied Genomics, Children s Hospital of Philadelphia, Philadelphia, Pennsylvania 19104, USA
    Nat Commun 6:8442. 2015
    ....
  14. pmc Substance P inhibits natural killer cell cytotoxicity through the neurokinin-1 receptor
    Linda Monaco-Shawver
    The Children s Hospital of Philadelphia Research Institute, University of Pennsylvania, Pediatrics, Philadelphia, PA 19104, USA
    J Leukoc Biol 89:113-25. 2011
    ..Antagonism of this system may present an opportunity to augment NK cell function therapeutically in selected human diseases...
  15. pmc The adaptor molecule SAP plays essential roles during invariant NKT cell cytotoxicity and lytic synapse formation
    Rupali Das
    Division of Oncology, Children s Hospital of Philadelphia, Philadelphia, PA 19104, USA
    Blood 121:3386-95. 2013
    ..Collectively, these studies highlight a novel and essential role for SAP during iNKT cell cytotoxicity and formation of a functional IS...
  16. pmc Navigating barriers: the challenge of directed secretion at the natural killer cell lytic immunological synapse
    Keri B Sanborn
    Immunology Graduate Group, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA
    J Clin Immunol 30:358-63. 2010
    ..This represents a dynamic molecular arrangement that over time progresses through a series of steps to ultimately deliver the contents of specialized organelles known as lytic granules...
  17. pmc Rapid up-regulation and granule-independent transport of perforin to the immunological synapse define a novel mechanism of antigen-specific CD8+ T cell cytotoxic activity
    George Makedonas
    Department of Microbiology, University of Pennsylvania, Philadelphia, PA 19104, USA
    J Immunol 182:5560-9. 2009
    ..Our work suggests a novel mechanism of CTL cytotoxicity and identifies a novel correlate of CD8(+) T cell-mediated immunity...
  18. pmc Arf-like GTPase Arl8b regulates lytic granule polarization and natural killer cell-mediated cytotoxicity
    Amit Tuli
    Division of Rheumatology, Immunology and Allergy, Department of Medicine, Brigham and Women s Hospital, Harvard Medical School, Boston, MA 02115 Division of Cell Biology and Immunology, Institute of Microbial Technology, Chandigarh 160036, India Program in Cellular and Molecular Medicine, Boston Children s Hospital, Harvard Medical School, Boston, MA 02115 Institut National de la Santé et de la Recherche Médicale, Unité 753, Institut Gustave Roussy, Villejuif 75654, France Department of Pediatrics, University of Pennsylvania School of Medicine, Philadelphia, PA 19104 Department of Biological Sciences, Indian Institute of Science Education and Research, Mohali 140306, India Immunology, Allergy and Rheumatology, Texas Children s Hospital, Baylor College of Medicine, Houston, TX 77030
    Mol Biol Cell 24:3721-35. 2013
    ..Silencing of both KIF5B and SKIP in NK cells, similar to Arl8b, led to failure of MTOC-lytic granule polarization to the immune synapse, suggesting that Arl8b and kinesin-1 together control this critical step in NK cell cytotoxicity...
  19. pmc Human immunodeficiency-causing mutation defines CD16 in spontaneous NK cell cytotoxicity
    Jennifer T Grier
    Children s Hospital of Philadelphia Research Institute, Philadelphia, Pennsylvania, USA
    J Clin Invest 122:3769-80. 2012
    ..Thus, CD16 serves a role in NK cell-mediated spontaneous cytotoxicity through a specific association with CD2 and represents a potential mechanism underlying a human congenital immunodeficiency...
  20. pmc Lymphotoxin-alpha 1 beta 2 and LIGHT induce classical and noncanonical NF-kappa B-dependent proinflammatory gene expression in vascular endothelial cells
    Lisa A Madge
    Department of Animal Biology and the Mari Lowe Center for Comparative Oncology, University of Pennsylvania School of Veterinary Medicine, and The Joseph Stokes Jr Research Institute, Children s Hospital of Philadelphia, Philadelphia, PA 19104, USA
    J Immunol 180:3467-77. 2008
    ..Our findings therefore demonstrate that LTbetaR ligation regulates gene expression in endothelial cells via both NF-kappaB pathways and we identify CXCL12 as a bona fide noncanonical NF-kappaB-regulated gene in these cells...
  21. pmc The autoimmune regulator (AIRE), which is defective in autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy patients, is expressed in human epidermal and follicular keratinocytes and associates with the intermediate filament protein cytokeratin
    Vipul Kumar
    Division of Immunology, Department of Pediatrics, Children s Hospital of Philadelphia, Philadelphia, Pennsylvania 19104, USA
    Am J Pathol 178:983-8. 2011
    ..The cytoplasmic association of AIRE with the intermediate filament network in human epidermal and follicular keratinocytes may provide a new path to understanding the ectodermal abnormalities associated with the APECED syndrome...
  22. pmc Quantitative measurement of F-actin accumulation at the NK cell immunological synapse
    Pinaki P Banerjee
    The Children s Hospital of Philadelphia Research Institute, 3615 Civic Center Blvd, Philadelphia, PA 19104, USA
    J Immunol Methods 355:1-13. 2010
    ....
  23. ncbi request reprint The lytic NK cell immunological synapse and sequential steps in its formation
    Jordan S Orange
    University of Pennsylvania School of Medicine, Department of Pediatrics, The Children s Hospital of Philadelphia, Philadelphia, PA, USA
    Adv Exp Med Biol 601:225-33. 2007
    ..These events unfold in a definitive sequence and lead to critical checkpoints that provide regulatory control at specific stages in the formation of the NK cell lytic synapse...
  24. ncbi request reprint Natural killer cells in human health and disease
    Jordan S Orange
    University of Pennsylvania School of Medicine, Department of Pediatrics, Children s Hospital of Philadelphia, Division of Allergy and Immunology, 3615 Civic Center Blvd, Philadelphia, PA 19104, USA
    Clin Immunol 118:1-10. 2006
    ..NK cells play important roles in viral infections, autoimmunity, pregnancy, cancer and bone marrow transplantation. This review highlights recent developments in the understanding of the role of human NK cells in health and disease...
  25. ncbi request reprint Human natural killer cell deficiencies
    Jordan S Orange
    Department of Pediatrics, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA
    Curr Opin Allergy Clin Immunol 6:399-409. 2006
    ..Since previously reviewing these disorders, significant developments warrant their reconsideration...
  26. ncbi request reprint Use of intravenous immunoglobulin in human disease: a review of evidence by members of the Primary Immunodeficiency Committee of the American Academy of Allergy, Asthma and Immunology
    Jordan S Orange
    Department of Pediatrics, University of Pennsylvania School of Medicine, The Children s Hospital of Philadelphia, Philadelphia 19104, USA
    J Allergy Clin Immunol 117:S525-53. 2006
    ..Given the potential risks and inherent scarcity of IGIV, careful consideration of the indications for and administration of IGIV is warranted...
  27. pmc Meta-analysis of shared genetic architecture across ten pediatric autoimmune diseases
    Yun R Li
    The Center for Applied Genomics, The Children s Hospital of Philadelphia, Philadelphia, Pennsylvania, USA
    Nat Med 21:1018-27. 2015
    ..Network and protein-interaction analyses demonstrated converging roles for the signaling pathways of type 1, 2 and 17 helper T cells (TH1, TH2 and TH17), JAK-STAT, interferon and interleukin in multiple autoimmune diseases. ..
  28. pmc Noncanonical NF-κB signaling is limited by classical NF-κB activity
    Carolyn M Gray
    1Department of Animal Biology, University of Pennsylvania School of Veterinary Medicine, 3800 Spruce Street, Philadelphia, PA 19104, USA
    Sci Signal 7:ra13. 2014
    ..Together, these findings identify a crucial role for classical NF-κB activity in the suppression of basal noncanonical NF-κB signaling. ..
  29. pmc CD2 promotes human natural killer cell membrane nanotube formation
    Colin J Comerci
    Department of Pediatrics, Children s Hospital of Philadelphia Research Institute, Philadelphia, Pennsylvania, United States of America
    PLoS ONE 7:e47664. 2012
    ..Thus only specific receptor-ligand pairs promote nanotubes. CD2 also promoted nanotube formation in ex vivo NK cells suggesting that CD2 plays a crucial role in the generation of nanotubes between an NK cell and its target...
  30. pmc Commensal bacteria-derived signals regulate basophil hematopoiesis and allergic inflammation
    David A Hill
    Department of Microbiology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA
    Nat Med 18:538-46. 2012
    ..Collectively, these results identify a previously unrecognized pathway through which commensal-derived signals influence basophil hematopoiesis and susceptibility to T(H)2 cytokine-dependent inflammation and allergic disease...
  31. pmc Chipping away at gamma-H2AX foci
    Velibor Savic
    Department of Pathology and Laboratory Medicine, Children s Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, PA, USA
    Cell Cycle 8:3285-90. 2009
    ..Here, we compare data obtained from parallel gamma-H2AX ChIP and three-dimensional IF analyses and discuss the impact of our findings upon molecular mechanisms that regulate H2AX phosphorylation in chromatin around DNA breakage sites...
  32. pmc IKBKG (nuclear factor-kappa B essential modulator) mutation can be associated with opportunistic infection without impairing Toll-like receptor function
    Bryn H Salt
    Department of Pediatrics, National Jewish Medical and Research Center, Denver, CO 80206, USA
    J Allergy Clin Immunol 121:976-82. 2008
    ..Patients with hypomorphic nuclear factor-kappaB essential modulator (NEMO) mutations have extensive phenotypic variability that can include atypical infectious susceptibility...
  33. doi request reprint Pediatric common variable immunodeficiency: immunologic and phenotypic associations with switched memory B cells
    Pierre L Yong
    Department of Medicine, Philadelphia Veterans Affairs Medical Center, Philadelphia, PA 19104, USA
    Pediatr Allergy Immunol 21:852-8. 2010
    ..Segregation of pediatric patients into high risk (Group I) and average risk (Group II) may assist in targeting surveillance appropriately...
  34. pmc Invariant natural killer T cells from children with versus without food allergy exhibit differential responsiveness to milk-derived sphingomyelin
    Soma Jyonouchi
    Division of Allergy and Immunology, The Children s Hospital of Philadelphia, Philadelphia, PA 19104, USA
    J Allergy Clin Immunol 128:102-109.e13. 2011
    ..Major food allergens (eg, milk, egg) are the richest dietary source of sphingolipids (food-derived sphingolipids [food-SLs]). Nonetheless, the role of iNKTs in FA is unknown...
  35. pmc Genome-wide association identifies diverse causes of common variable immunodeficiency
    Jordan S Orange
    Division of Allergy and Immunology, Children s Hospital of Philadelphia Research Institute, Children s Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, PA, USA
    J Allergy Clin Immunol 127:1360-7.e6. 2011
    ..Common variable immunodeficiency (CVID) is a heterogeneous immune defect characterized by hypogammaglobulinemia, failure of specific antibody production, susceptibility to infections, and an array of comorbidities...
  36. doi request reprint Use of cytokine therapy in primary immunodeficiency
    Sumita Roy-Ghanta
    Division of Allergy and Immunology, Children s Hospital of Philadelphia, Philadelphia, PA 19104, USA
    Clin Rev Allergy Immunol 38:39-53. 2010
    ..Despite encouraging results with interleukin-2 in common variable immunodeficiency and select other indications, its use in PID is not widespread...
  37. pmc HIV Progression Perturbs the Balance of the Cell-Mediated and Anti-Inflammatory Adaptive and Innate Mycobacterial Immune Response
    Andrew R DiNardo
    The Global Tuberculosis Program, Texas Children s Hospital, Immigrant and Global Health, Department of Pediatrics, Baylor College of Medicine, Houston, TX 77030, USA
    Mediators Inflamm 2016:1478340. 2016
    ..76; p = 0.01). Conclusion. As HIV disease progresses, both the adaptive and innate branches skew away from an inflammatory and towards anti-inflammatory phenotype. ..
  38. pmc Immune dysregulation in severe influenza
    Meredith L Heltzer
    Divisions of Allergy and Immunology, Children s Hospital of Philadelphia, Philadelphia, Pennsylvania, USA
    J Leukoc Biol 85:1036-43. 2009
    ....
  39. doi request reprint Severe combined immunodeficiency resulting from mutations in MTHFD1
    Michael D Keller
    Children s Hospital of Philadelphia, 3550 Market St, 3rd Floor, Philadelphia, PA 19104, USA
    Pediatrics 131:e629-34. 2013
    ..This finding expands the known metabolic causes of SCID and presents an important diagnostic consideration given the positive impact of therapy...
  40. doi request reprint Tumorigenic adenovirus 12 cells evade NK cell lysis by reducing the expression of NKG2D ligands
    Christa Y Heyward
    Abramson Cancer Center, School of Medicine, University of Pennsylvania, Philadelphia, PA 19104, United States
    Immunol Lett 144:16-23. 2012
    ....
  41. doi request reprint 1. Lymphocytes
    David F LaRosa
    University of Pennsylvania School of Medicine, Philadelphia, PA, USA
    J Allergy Clin Immunol 121:S364-9; quiz S412. 2008
    ..This chapter provides an overview of the major lymphocyte populations with emphasis on their development, distinguishing characteristics, and functions...
  42. doi request reprint Impact of trough IgG on pneumonia incidence in primary immunodeficiency: A meta-analysis of clinical studies
    Jordan S Orange
    Division of Allergy and Immunology, Children s Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA
    Clin Immunol 137:21-30. 2010
    ..113 cases per patient-year) was 5-fold that with 1000 mg/dL (0.023 cases per patient-year). This meta-analysis provides evidence that pneumonia risk can be progressively reduced by higher trough IgG levels up to at least 1000 mg/dL...
  43. pmc NKp30 ligation induces rapid activation of the canonical NF-kappaB pathway in NK cells
    Rahul Pandey
    Division of Allergy and Immunology, The Joseph Stokes Jr Research Institute, Children s Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA
    J Immunol 179:7385-96. 2007
    ..The latter was prevented by the Syk inhibitor piceatannol. Thus, NK cell activation signaling specifically induces transcriptional activation and synthesis of new NF-kappaB dependent proteins during the initiation of cytotoxicity...
  44. doi request reprint Use of intravenous immunoglobulin and adjunctive therapies in the treatment of primary immunodeficiencies: A working group report of and study by the Primary Immunodeficiency Committee of the American Academy of Allergy Asthma and Immunology
    Pierre L Yong
    Robert Wood Johnson Clinical Scholars Program, University of Pennsylvania, Philadelphia, PA, USA
    Clin Immunol 135:255-63. 2010
    ..Thus, a need for expanded clinical research in PIDD to optimize management and potentially improve outcomes was defined...
  45. pmc Rare variants at 16p11.2 are associated with common variable immunodeficiency
    S Melkorka Maggadottir
    Division of Allergy and Immunology, Children s Hospital of Philadelphia, Philadelphia, Pa Center for Applied Genomics, Abramson Research Center, Children s Hospital of Philadelphia, Philadelphia, PA
    J Allergy Clin Immunol 135:1569-77. 2015
    ..Only a few genes have been uncovered, and the genetic background of CVID remains elusive to date for the majority of patients...
  46. pmc Cdc42-interacting protein-4 functionally links actin and microtubule networks at the cytolytic NK cell immunological synapse
    Pinaki P Banerjee
    Children s Hospital of Philadelphia, Division of Immunology, Abramson Research Center, Philadelphia, PA 19104, USA
    J Exp Med 204:2305-20. 2007
    ..Thus, CIP4 is an important cytoskeletal adaptor that functions after filamentous actin accumulation and Cdc42 activation to enable MTOC polarization and NK cell cytotoxicity...
  47. pmc Murine natural killer immunoreceptors use distinct proximal signaling complexes to direct cell function
    Rebecca M May
    Division of Transfusion Medicine and Therapeutic Pathology, Department of Pathology and Laboratory Medicine, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, USA
    Blood 121:3135-46. 2013
    ..These results demonstrate that NK cells possess an unexpected bifurcation of proximal ITAM-mediated signaling, each involving SLP-76 and contributing to optimal NK-cell function...
  48. pmc Immunodeficiency in patients with 49,XXXXY chromosomal variation
    Michael D Keller
    Division of Allergy and Immunology at the Children s Hospital, Philadelphia, PA 19104, USA
    Am J Med Genet C Semin Med Genet 163:50-4. 2013
    ..These preliminary results suggest a high incidence of both atopy and antibody deficiency in boys with 49,XXXXY...
  49. pmc Formation and function of the lytic NK-cell immunological synapse
    Jordan S Orange
    University of Pennsylvania School of Medicine, Joseph Stokes Jr Research Institute of The Children s Hospital of Philadelphia, 3615 Civic Center Boulevard, ARC 1016H, Philadelphia, Pennsylvania 19104, USA
    Nat Rev Immunol 8:713-25. 2008
    ....
  50. ncbi request reprint Human disease resulting from gene mutations that interfere with appropriate nuclear factor-kappaB activation
    Jordan S Orange
    Division of Immunology, Children s Hospital of Philadelphia, Department of Pediatrics, University of Pennsylvania School of Medicine, Philadelphia, PA, USA
    Immunol Rev 203:21-37. 2005
    ..A detailed study of these diseases can provide insight into the biochemistry of NF-kappaB activation as well as the role of NF-kappaB in human health...
  51. ncbi request reprint Natural killer cells inhibit hepatitis C virus expression
    Yuan Li
    Division of Allergy and Immunology, Joseph Stokes, Jr Research Institute at The Children s Hospital of Philadelphia, University of Pennsylvania School of Medicine, 34th Street and Civic Center Blvd, Philadelphia, PA 19104, USA
    J Leukoc Biol 76:1171-9. 2004
    ..Thus, NK cells hold the potential to play a vital role in controlling HCV replication in hepatic cells using an IFN-gamma-dependent mechanism...
  52. pmc Capsid antigen presentation flags human hepatocytes for destruction after transduction by adeno-associated viral vectors
    Gary C Pien
    Division of Allergy and Immunology, Children s Hospital of Philadelphia and University of Pennsylvania, Philadelphia, Pennsylvania 19104, USA
    J Clin Invest 119:1688-95. 2009
    ....
  53. doi request reprint Evaluation and clinical interpretation of hypergammaglobulinemia E: differentiating atopy from immunodeficiency
    Gary C Pien
    Division of Allergy and Immunology, The Children s Hospital of Philadelphia, The University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104, USA
    Ann Allergy Asthma Immunol 100:392-5. 2008
    ..However, total IgE level is neither a sensitive nor a specific diagnostic marker for any particular disease and, therefore, should not be relied on to establish a diagnosis of either atopy or primary immunodeficiency...
  54. ncbi request reprint Congenital immunodeficiencies and sepsis
    Jordan S Orange
    Department of Pediatrics, University of Pennsylvania School of Medicine, Division of Allergic and Immunologic diseases, The Children s Hospital of Philadelphia, Philadelphia, PA, USA
    Pediatr Crit Care Med 6:S99-S107. 2005
    ..Because a hallmark of congenital immunodeficiency disorders is susceptibility to recurrent, unusual and/or severe infections, an effort was undertaken to identify a subset of these patients with an increased risk for sepsis...
  55. ncbi request reprint Superantigen presentation by airway smooth muscle to CD4+ T lymphocytes elicits reciprocal proasthmatic changes in airway function
    Haviva Veler
    Division of Pulmonary Medicine, Joseph Stokes Jr Research Institute, Children s Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA
    J Immunol 178:3627-36. 2007
    ....