Jordan S. Orange

Deficient natural killer cell cytotoxicity in patients with IKK-gamma/NEMO mutations.

Wiskott-Aldrich syndrome protein is required for NK cell cytotoxicity and colocalizes with actin to NK cell-activating immunologic synapses.

Viral evasion of natural killer cells.

Human natural killer cell deficiencies and susceptibility to infection.

Acute chylothorax in children: selective retention of memory T cells and natural killer cells.

Disseminated varicella infection due to the vaccine strain of varicella-zoster virus, in a patient with a novel deficiency in natural killer T cells.

Finding NEMO: genetic disorders of NF-[kappa]B activation.

Human decidual natural killer cells are a unique NK cell subset with immunomodulatory potential.

The mature activating natural killer cell immunologic synapse is formed in distinct stages.

Management of an anaphylactoid reaction to methotrexate with a stepwise graded challenge.

A patient with severe black fly (Simuliidae) hypersensitivity referred for evaluation of suspected immunodeficiency.

The presentation and natural history of immunodeficiency caused by nuclear factor kappaB essential modulator mutation.

Natural killer cells inhibit hepatitis C virus expression.

Human nuclear factor kappa B essential modulator mutation can result in immunodeficiency without ectodermal dysplasia.

Characteristics of mycobacterial infection in patients with immunodeficiency and nuclear factor-kappaB essential modulator mutation, with or without ectodermal dysplasia.

Specific NEMO mutations impair CD40-mediated c-Rel activation and B cell terminal differentiation.

Human disease resulting from gene mutations that interfere with appropriate nuclear factor-kappaB activation.

Congenital immunodeficiencies and sepsis.

Practice parameter for the diagnosis and management of primary immunodeficiency.

Effector and memory CD8+ T cell fate coupled by T-bet and eomesodermin.

Natural killer cells in human health and disease.

Development of a clinical assay to evaluate toll-like receptor function.

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.

Formation of a WIP-, WASp-, actin-, and myosin IIA-containing multiprotein complex in activated NK cells and its alteration by KIR inhibitory signaling.

Human natural killer cell deficiencies.

Prevalence of Toll-like receptor signalling defects in apparently healthy children who developed invasive pneumococcal infection.

Impaired specific antibody response and increased B-cell population in transient hypogammaglobulinemia of infancy.

Interleukin-1-induced NF-kappaB activation is NEMO-dependent but does not require IKKbeta.

Asymmetric T lymphocyte division in the initiation of adaptive immune responses.

Superantigen presentation by airway smooth muscle to CD4+ T lymphocytes elicits reciprocal proasthmatic changes in airway function.

Hypogammaglobulinemia in a pediatric tertiary care setting.

A genome-wide association study identifies KIAA0350 as a type 1 diabetes gene.

The lytic NK cell immunological synapse and sequential steps in its formation.

Cdc42-interacting protein-4 functionally links actin and microtubule networks at the cytolytic NK cell immunological synapse.

Myosin IIA is required for cytolytic granule exocytosis in human NK cells.

NKp30 ligation induces rapid activation of the canonical NF-kappaB pathway in NK cells.

IKBKG (nuclear factor-kappa B essential modulator) mutation can be associated with opportunistic infection without impairing Toll-like receptor function.

1. Lymphocytes.

Lymphotoxin-alpha 1 beta 2 and LIGHT induce classical and noncanonical NF-kappa B-dependent proinflammatory gene expression in vascular endothelial cells.

Immunodeficiency disorders. Preface.

Evaluation and clinical interpretation of hypergammaglobulinemia E: differentiating atopy from immunodeficiency.

Establishment of HIV-1 resistance in CD4+ T cells by genome editing using zinc-finger nucleases.

Hypomorphic nuclear factor-kappaB essential modulator mutation database and reconstitution system identifies phenotypic and immunologic diversity.

Allogeneic transplantation successfully corrects immune defects, but not susceptibility to colitis, in a patient with nuclear factor-kappaB essential modulator deficiency.

Formation and function of the lytic NK-cell immunological synapse.

Immune dysregulation in severe influenza.

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.

Capsid antigen presentation flags human hepatocytes for destruction after transduction by adeno-associated viral vectors.

Use of cytokine therapy in primary immunodeficiency.

Myosin IIA associates with NK cell lytic granules to enable their interaction with F-actin and function at the immunological synapse.

Comèl-Netherton syndrome defined as primary immunodeficiency.

Chipping away at gamma-H2AX foci.

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.

Variants of DENND1B associated with asthma in children.

Analysis of the NK cell immunological synapse.

Quantitative measurement of F-actin accumulation at the NK cell immunological synapse.

Navigating barriers: the challenge of directed secretion at the natural killer cell lytic immunological synapse.

Pediatric common variable immunodeficiency: immunologic and phenotypic associations with switched memory B cells.

Rapid lytic granule convergence to the MTOC in natural killer cells is dependent on dynein but not cytolytic commitment.

Efficacy and safety of a new 20% immunoglobulin preparation for subcutaneous administration, IgPro20, in patients with primary immunodeficiency.

Latent herpesviruses: aligning human and murine NK cells.

A novel missense mutation in the nuclear factor-κB essential modulator (NEMO) gene resulting in impaired activation of the NF-κB pathway and a unique clinical phenotype presenting as MRSA subdural empyema.

A custom 148 gene-based resequencing chip and the SNP explorer software: new tools to study antibody deficiency.

Impact of trough IgG on pneumonia incidence in primary immunodeficiency: A meta-analysis of clinical studies.

miR-451 protects against erythroid oxidant stress by repressing 14-3-3zeta.

Cutting edge: association with I kappa B kinase beta regulates the subcellular localization of Homer3.

Substance P inhibits natural killer cell cytotoxicity through the neurokinin-1 receptor.

Therapeutic use of immunoglobulins.

Complications of tumor necrosis factor-α blockade in chronic granulomatous disease-related colitis.

Stem-cell gene therapy for the Wiskott-Aldrich syndrome.

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 17.

IL-2 induces a WAVE2-dependent pathway for actin reorganization that enables WASp-independent human NK cell function.

Invariant natural killer T cells from children with versus without food allergy exhibit differential responsiveness to milk-derived sphingomyelin.

Genome-wide association identifies diverse causes of common variable immunodeficiency.

Eosinophilic fasciitis mimicking angioedema and treatment response to infliximab in a pediatric patient.

Mutations in GATA2 are associated with the autosomal dominant and sporadic monocytopenia and mycobacterial infection (MonoMAC) syndrome.

Congenital alterations of NEMO glutamic acid 223 result in hypohidrotic ectodermal dysplasia and immunodeficiency with normal serum IgG levels.

Novel inborn error of folate metabolism: identification by exome capture and sequencing of mutations in the MTHFD1 gene in a single proband.

Natural killer cell lytic granule secretion occurs through a pervasive actin network at the immune synapse.

Global study of primary immunodeficiency diseases (PI)--diagnosis, treatment, and economic impact: an updated report from the Jeffrey Modell Foundation.

Autoimmune regulator (AIRE) contributes to Dectin-1-induced TNF-α production and complexes with caspase recruitment domain-containing protein 9 (CARD9), spleen tyrosine kinase (Syk), and Dectin-1.

Phosphorylation of the myosin IIA tailpiece regulates single myosin IIA molecule association with lytic granules to promote NK-cell cytotoxicity.

Multiple distinct NK-cell synapses.

CD27 deficiency is associated with combined immunodeficiency and persistent symptomatic EBV viremia.

Novel mutation in STXBP2 prevents IL-2-induced natural killer cell cytotoxicity.

Unraveling human natural killer cell deficiency.

Bilateral adrenal EBV-associated smooth muscle tumors in a child with a natural killer cell deficiency.

Tumorigenic adenovirus 12 cells evade NK cell lysis by reducing the expression of NKG2D ligands.

Commensal bacteria-derived signals regulate basophil hematopoiesis and allergic inflammation.

Presentation of hemophagocytic lymphohistiocytosis due to a novel MUNC 13-4 mutation masked by partial therapeutic immunosuppression.

Hypohidrotic ectodermal dysplasia and immunodeficiency with coincident NEMO and EDA mutations.

Dual channel STED nanoscopy of lytic granules on actin filaments in natural killer cells.

Use and interpretation of diagnostic vaccination in primary immunodeficiency: a working group report of the Basic and Clinical Immunology Interest Section of the American Academy of Allergy, Asthma & Immunology.

Human immunodeficiency-causing mutation defines CD16 in spontaneous NK cell cytotoxicity.

NK cell lytic granules are highly motile at the immunological synapse and require F-actin for post-degranulation persistence.

CD2 promotes human natural killer cell membrane nanotube formation.

Reply: To PMID 22935624.

Severe combined immunodeficiency resulting from mutations in MTHFD1.

New views of the human NK cell immunological synapse: recent advances enabled by super- and high-resolution imaging techniques.

Immunodeficiency in patients with 49,XXXXY chromosomal variation.

Mutations in GATA2 cause human NK cell deficiency with specific loss of the CD56(bright) subset.

Defective actin accumulation impairs human natural killer cell function in patients with dedicator of cytokinesis 8 deficiency.

Rapid activation receptor- or IL-2-induced lytic granule convergence in human natural killer cells requires Src, but not downstream signaling.

Murine natural killer immunoreceptors use distinct proximal signaling complexes to direct cell function.

The adaptor molecule SAP plays essential roles during invariant NKT cell cytotoxicity and lytic synapse formation.

Differential localization of T-bet and Eomes in CD8 T cell memory populations.

Bioavailability of IgG administered by the subcutaneous route.

Pro-asthmatic cytokines regulate unliganded and ligand-dependent glucocorticoid receptor signaling in airway smooth muscle.

Prioritization of evidence-based indications for intravenous immunoglobulin.

Lentiviral hematopoietic stem cell gene therapy in patients with Wiskott-Aldrich syndrome.

Natural killer cell deficiency.

Arf-like GTPase Arl8b regulates lytic granule polarization and natural killer cell-mediated cytotoxicity.

GATA2 deficiency: a protean disorder of hematopoiesis, lymphatics, and immunity.

Burden of Copy Number Variation in Common Variable Immunodeficiency.

Modeling primary immunodeficiency disease epidemiology and its treatment to estimate latent therapeutic demand for immunoglobulin.

Cell biological steps and checkpoints in accessing NK cell cytotoxicity.

Noncanonical NF-κB Signaling Is Limited by Classical NF-κB Activity.

iNKT cell cytotoxic responses control T-lymphoma growth in vitro and in vivo.

Recommendations for live viral and bacterial vaccines in immunodeficient patients and their close contacts.