Raif Geha's lab pursues the molecular basis of inherited immune deficiencies. The Geha lab has established a mouse model of Atopic Dermatitis and is studying the mechanisms of allergic sensitization through the skin and of recruitment of T cells & eosinophils to the skin. The researchers are pursuing several investigational avenues. One seeks to identify the molecular process by which a B cell switches from producing one class of antibody to another. A second explores inherited immune deficiency disorders, with special emphasis on Wiscott-Aldrich Syndrome. A third investigates the molecular basis of atopic dermatitis (AD) a common, an allergic inflammation of the skin that is common, but poorly understood. The lab has created an animal model of AD that may ultimately be used to develop potential drugs. Isotype switching is the mechanism by which a B cell goes from producing one type of antibody to another while maintaining antigenic specificity. This phenomenon allows the immune system to produce various antibody types against the same antigen, but having different effector functions. Isotype switching is a highly orchestrated process with several cytokines and T and B cell surface molecules participating. The Geha laboratory has shown that B cell surface molecules CD40, BAFF and APRIL are important for the switching process, and that defects in these molecules, or in the signaling cascade emanating from them, could potentially lead to immunodeficiency. Geha and colleagues have also shown that C4BP, a complement regulatory protein, binds to CD40 and induces isotype switching. At present, they are studying the role of BAFF and APRIL in isotype switching and antibody affinity maturation. Wiskott-Aldrich syndrome (WAS) is a primary immunodeficiency caused by mutation in the gene encoding for the WAS protein (WASP). The Geha group identified a novel cellular negative regulator of WASP they have named WIP. WASP and WIP together regulate most cell functions that require the remodeling of the actin-based cytoskeleton--the structural framework of the cell. The Geha lab is now studying the role of WASP and WIP in immune cell functions that require active cytoskeletal remodeling such as migrating in response to chemical signals and homing. They are also mapping the domains of WASP and WIP that are involved in discrete functions of these molecules.
Research Background
Raif Geha is a graduate of American University of Beirut, Lebanon. He completed his internship at the American University Hospital in Lebanon, and his residency and a post-graduate fellowship at Boston Children's Hospital.
Education
Medical School
American University of Beirut
1969
Beirut
Lebanon
Internship
American University of Beirut
1969
Beirut
Lebanon
Residency
Boston Children's Hospital
1971
Boston Children's Hospital
MA
Fellowship
Boston Children's Hospital
1974
Boston
MA
Publications
Multiomics dissection of human RAG deficiency reveals distinctive patterns of immune dysregulation but a common inflammatory signature. Sci Immunol. 2025 Jan 10; 10(103):eadq1697. View Abstract
Germline mutations in a G protein identify signaling cross-talk in T cells. Science. 2024 Sep 20; 385(6715):eadd8947. View Abstract
IL-4 acts on skin-derived dendritic cells to promote the TH2 response to cutaneous sensitization and the development of allergic skin inflammation. J Allergy Clin Immunol. 2024 Dec; 154(6):1462-1471.e3. View Abstract
Reflections on a half century of mentoring. J Allergy Clin Immunol. 2024 Oct; 154(4):917-921. View Abstract
Clinical, immunological features, treatments, and outcomes of autoimmune hemolytic anemia in patients with RAG deficiency. Blood Adv. 2024 02 13; 8(3):603-607. View Abstract
Basophils are important for development of allergic skin inflammation. J Allergy Clin Immunol. 2024 May; 153(5):1344-1354.e5. View Abstract
Basophils Play a Protective Role in the Recovery of Skin Barrier Function from Mechanical Injury in Mice. J Invest Dermatol. 2024 Aug; 144(8):1784-1797.e4. View Abstract
Regulatory T-cell dysfunction and cutaneous exposure to Staphylococcus aureus underlie eczema in DOCK8 deficiency. J Allergy Clin Immunol. 2024 Jul; 154(1):143-156. View Abstract
Ant Venom-Based Ceramide Therapy Is Effective Against Atopic Dermatitis In Vivo. J Drugs Dermatol. 2023 Oct 01; 22(10):1001-1006. View Abstract
DOCK8 is essential for neutrophil mediated clearance of cutaneous S. aureus infection. Clin Immunol. 2023 09; 254:109681. View Abstract
IL-4 receptor alpha blockade dampens allergic inflammation and upregulates IL-17A expression to promote Saureus clearance in antigen sensitized mouse skin. J Allergy Clin Immunol. 2023 10; 152(4):907-915. View Abstract
The IL-4Ra Q576R polymorphism is associated with increased severity of atopic dermatitis and exaggerates allergic skin inflammation in mice. J Allergy Clin Immunol. 2023 05; 151(5):1296-1306.e7. View Abstract
The Notch1/CD22 signaling axis disrupts Treg function in SARS-CoV-2-associated multisystem inflammatory syndrome in children. J Clin Invest. 2023 01 03; 133(1). View Abstract
NFKB2 haploinsufficiency identified via screening for IFN-a2 autoantibodies in children and adolescents hospitalized with SARS-CoV-2-related complications. J Allergy Clin Immunol. 2023 04; 151(4):926-930.e2. View Abstract
Immune dysregulation caused by homozygous mutations in CBLB. J Clin Invest. 2022 10 17; 132(20). View Abstract
The Middle East and North Africa Diagnosis and Management Guidelines for Inborn Errors of Immunity. J Allergy Clin Immunol Pract. 2023 01; 11(1):158-180.e11. View Abstract
A homozygous truncating mutation of FGL2 is associated with immune dysregulation. J Allergy Clin Immunol. 2023 02; 151(2):572-578.e1. View Abstract
Clinical, immunological, molecular and therapeutic findings in monogenic immune dysregulation diseases: Middle East and North Africa registry. Clin Immunol. 2022 11; 244:109131. View Abstract
Author Correction: DOCK8 functions as an adaptor that links TLR-MyD88 signaling to B cell activation. Nat Immunol. 2022 May; 23(5):815. View Abstract
Inborn Errors of the Immune System Associated With Atopy. Front Immunol. 2022; 13:860821. View Abstract
Linker-Improved Chimeric Endolysin Selectively Kills Staphylococcus aureus In Vitro, on Reconstituted Human Epidermis, and in a Murine Model of Skin Infection. Antimicrob Agents Chemother. 2022 05 17; 66(5):e0227321. View Abstract
Notch1-CD22-Dependent Immune Dysregulation in the SARS-CoV2-Associated Multisystem Inflammatory Syndrome in Children. Res Sq. 2022 Apr 11. View Abstract
Multi-kingdom ecological drivers of microbiota assembly in preterm infants. Nature. 2021 03; 591(7851):633-638. View Abstract
Combined immunodeficiency due to a mutation in the ?1 subunit of the coat protein I complex. J Clin Invest. 2021 02 01; 131(3). View Abstract
Author Correction: A regulatory T cell Notch4-GDF15 axis licenses tissue inflammation in asthma. Nat Immunol. 2021 Jan; 22(1):100. View Abstract
Mast cell-derived IL-13 downregulates IL-12 production by skin dendritic cells to inhibit the TH1 cell response to cutaneous antigen exposure. J Allergy Clin Immunol. 2021 06; 147(6):2305-2315.e3. View Abstract
Successful hematopoietic stem cell transplantation in a 4-1BB deficient patient with EBV-induced lymphoproliferation. Clin Immunol. 2021 01; 222:108639. View Abstract
DOCK8 Expression in Regulatory T Cells Maintains their Stability and Limits Contact Hypersensitivity. J Invest Dermatol. 2021 06; 141(6):1503-1511.e3. View Abstract
Transferrin receptor 1 is a cellular receptor for human heme-albumin. Commun Biol. 2020 10 27; 3(1):621. View Abstract
Hematopoietic Stem Cell Transplantation Is a Curative Therapy for Transferrin Receptor 1 (TFRC) Deficiency. J Allergy Clin Immunol Pract. 2021 02; 9(2):753-759.e2. View Abstract
A regulatory T cell Notch4-GDF15 axis licenses tissue inflammation in asthma. Nat Immunol. 2020 11; 21(11):1359-1370. View Abstract
Efficacy and economics of targeted panel versus whole-exome sequencing in 878 patients with suspected primary immunodeficiency. J Allergy Clin Immunol. 2021 02; 147(2):723-726. View Abstract
Immune dysregulation and multisystem inflammatory syndrome in children (MIS-C) in individuals with haploinsufficiency of SOCS1. J Allergy Clin Immunol. 2020 11; 146(5):1194-1200.e1. View Abstract
APRIL expression is upregulated in atopic dermatitis skin lesions and at sites of antigen driven allergic skin inflammation in mice. Clin Immunol. 2020 10; 219:108556. View Abstract
DOCK8 is essential for LFA-1-dependent positioning of T follicular helper cells in germinal centers. JCI Insight. 2020 08 06; 5(15). View Abstract
Acetaminophen Inhibits the Neutrophil Oxidative Burst: Implications for Diagnostic Testing. J Allergy Clin Immunol Pract. 2020 Nov - Dec; 8(10):3543-3548. View Abstract
Immunoglobulins in the treatment of COVID-19 infection: Proceed with caution! Clin Immunol. 2020 07; 216:108459. View Abstract
Cutaneous barrier leakage and gut inflammation drive skin disease in Omenn syndrome. J Allergy Clin Immunol. 2020 11; 146(5):1165-1179.e11. View Abstract
ILC2 activation by keratinocyte-derived IL-25 drives IL-13 production at sites of allergic skin inflammation. J Allergy Clin Immunol. 2020 06; 145(6):1606-1614.e4. View Abstract
Severe combined immunodeficiency caused by inositol-trisphosphate 3-kinase B (ITPKB) deficiency. J Allergy Clin Immunol. 2020 06; 145(6):1696-1699.e6. View Abstract
Genotype and functional correlates of disease phenotype in deficiency of adenosine deaminase 2 (DADA2). J Allergy Clin Immunol. 2020 06; 145(6):1664-1672.e10. View Abstract
Hypomorphic variants in AK2 reveal the contribution of mitochondrial function to B-cell activation. J Allergy Clin Immunol. 2020 07; 146(1):192-202. View Abstract
A novel truncating mutation in MYD88 in a patient with BCG adenitis, neutropenia and delayed umbilical cord separation. Clin Immunol. 2019 10; 207:40-42. View Abstract
Combined immunodeficiency in a patient with c-Rel deficiency. J Allergy Clin Immunol. 2019 08; 144(2):606-608.e4. View Abstract
A novel variant in STAT2 presenting with hemophagocytic lymphohistiocytosis. J Allergy Clin Immunol. 2019 08; 144(2):611-613.e3. View Abstract
Outcomes and Treatment Strategies for Autoimmunity and Hyperinflammation in Patients with RAG Deficiency. J Allergy Clin Immunol Pract. 2019 Jul - Aug; 7(6):1970-1985.e4. View Abstract
Immunodeficiency and EBV-induced lymphoproliferation caused by 4-1BB deficiency. J Allergy Clin Immunol. 2019 08; 144(2):574-583.e5. View Abstract
Rethinking newborn screening for severe combined immunodeficiency: Lessons from an international partnership for patients with primary immunodeficiencies in Pakistan. Clin Immunol. 2019 05; 202:29-32. View Abstract
T-cell mitochondrial dysfunction and lymphopenia in DOCK2-deficient patients. J Allergy Clin Immunol. 2019 07; 144(1):306-309.e2. View Abstract
F-BAR domain only protein 1 (FCHO1) deficiency is a novel cause of combined immune deficiency in human subjects. J Allergy Clin Immunol. 2019 06; 143(6):2317-2321.e12. View Abstract
Comprehensive Genetic Results for Primary Immunodeficiency Disorders in a Highly Consanguineous Population. Front Immunol. 2018; 9:3146. View Abstract
A monoallelic activating mutation in RAC2 resulting in a combined immunodeficiency. J Allergy Clin Immunol. 2019 04; 143(4):1649-1653.e3. View Abstract
Report from the National Institute of Allergy and Infectious Diseases workshop on "Atopic dermatitis and the atopic march: Mechanisms and interventions". J Allergy Clin Immunol. 2019 03; 143(3):894-913. View Abstract
Primary immunodeficiencies caused by mutations in actin regulatory proteins. Immunol Rev. 2019 01; 287(1):121-134. View Abstract
Atypical phenotype of an old disease or typical phenotype of a new disease: deficiency of adenosine deaminase 2. Turk J Pediatr. 2019; 61(3):413-417. View Abstract
Deficient LRRC8A-dependent volume-regulated anion channel activity is associated with male infertility in mice. JCI Insight. 2018 08 23; 3(16). View Abstract
Defective TLR9-driven STAT3 activation in B cells of patients with CVID. Clin Immunol. 2018 12; 197:40-44. View Abstract
MyD88 signaling in T regulatory cells by endogenous ligands dampens skin inflammation in filaggrin deficient mice. Clin Immunol. 2018 10; 195:88-92. View Abstract
Autosomal Recessive Agammaglobulinemia - first case with a novel TCF3 mutation from Pakistan. Clin Immunol. 2019 01; 198:100-101. View Abstract
The Lack of WIP Binding to Actin Results in Impaired B Cell Migration and Altered Humoral Immune Responses. Cell Rep. 2018 07 17; 24(3):619-629. View Abstract
WASP-mediated regulation of anti-inflammatory macrophages is IL-10 dependent and is critical for intestinal homeostasis. Nat Commun. 2018 05 03; 9(1):1779. View Abstract
Novel biallelic TRNT1 mutations resulting in sideroblastic anemia, combined B and T cell defects, hypogammaglobulinemia, recurrent infections, hypertrophic cardiomyopathy and developmental delay. Clin Immunol. 2018 03; 188:20-22. View Abstract
Staphylococcus aureus Epicutaneous Exposure Drives Skin Inflammation via IL-36-Mediated T Cell Responses. Cell Host Microbe. 2017 Nov 08; 22(5):653-666.e5. View Abstract
A digenic human immunodeficiency characterized by IFNAR1 and IFNGR2 mutations. J Clin Invest. 2017 12 01; 127(12):4415-4420. View Abstract
DOCK8 Deficiency Presenting as an IPEX-Like Disorder. J Clin Immunol. 2017 Nov; 37(8):811-819. View Abstract
DNA recombination defects in Kuwait: Clinical, immunologic and genetic profile. Clin Immunol. 2018 02; 187:68-75. View Abstract
DOCK8 enforces immunological tolerance by promoting IL-2 signaling and immune synapse formation in Tregs. JCI Insight. 2017 10 05; 2(19). View Abstract
A novel mutation in the JH4 domain of JAK3 causing severe combined immunodeficiency complicated by vertebral osteomyelitis. Clin Immunol. 2017 10; 183:198-200. View Abstract
Clinical, immunologic, and genetic spectrum of 696 patients with combined immunodeficiency. J Allergy Clin Immunol. 2018 04; 141(4):1450-1458. View Abstract
DOCK8 and STAT3 dependent inhibition of IgE isotype switching by TLR9 ligation in human B cells. Clin Immunol. 2017 10; 183:263-265. View Abstract
Advances in basic and clinical immunology in 2016. J Allergy Clin Immunol. 2017 Oct; 140(4):959-973. View Abstract
Combined immunodeficiency with EBV positive B cell lymphoma and epidermodysplasia verruciformis due to a novel homozygous mutation in RASGRP1. Clin Immunol. 2017 10; 183:142-144. View Abstract
Uses of Next-Generation Sequencing Technologies for the Diagnosis of Primary Immunodeficiencies. Front Immunol. 2017; 8:847. View Abstract
Cernunnos deficiency associated with BCG adenitis and autoimmunity: First case from the national Iranian registry and review of the literature. Clin Immunol. 2017 10; 183:201-206. View Abstract
Mechanisms of genotype-phenotype correlation in autosomal dominant anhidrotic ectodermal dysplasia with immune deficiency. J Allergy Clin Immunol. 2018 03; 141(3):1060-1073.e3. View Abstract
Human RELA haploinsufficiency results in autosomal-dominant chronic mucocutaneous ulceration. J Exp Med. 2017 Jul 03; 214(7):1937-1947. View Abstract
Exaggerated follicular helper T-cell responses in patients with LRBA deficiency caused by failure of CTLA4-mediated regulation. J Allergy Clin Immunol. 2018 03; 141(3):1050-1059.e10. View Abstract
The LRRC8A Mediated "Swell Activated" Chloride Conductance Is Dispensable for Vacuolar Homeostasis in Neutrophils. Front Pharmacol. 2017; 8:262. View Abstract
Disseminated Mycobacterium malmoense and Salmonella Infections Associated with a Novel Variant in NFKBIA. J Clin Immunol. 2017 07; 37(5):415-418. View Abstract
Leucine-rich repeat containing 8A (LRRC8A)-dependent volume-regulated anion channel activity is dispensable for T-cell development and function. J Allergy Clin Immunol. 2017 Dec; 140(6):1651-1659.e1. View Abstract
Janus kinase 3 deficiency caused by a homozygous synonymous exonic mutation that creates a dominant splice site. J Allergy Clin Immunol. 2017 07; 140(1):268-271.e6. View Abstract
Deficiency of base excision repair enzyme NEIL3 drives increased predisposition to autoimmunity. J Clin Invest. 2016 11 01; 126(11):4219-4236. View Abstract
A DOCK8-WIP-WASp complex links T cell receptors to the actin cytoskeleton. J Clin Invest. 2016 10 03; 126(10):3837-3851. View Abstract
Heterozygosity for transmembrane activator and calcium modulator ligand interactor A144E causes haploinsufficiency and pneumococcal susceptibility in mice. J Allergy Clin Immunol. 2017 Apr; 139(4):1293-1301.e4. View Abstract
Epidermodysplasia verruciformis as a manifestation of ARTEMIS deficiency in a young adult. J Allergy Clin Immunol. 2017 01; 139(1):372-375.e4. View Abstract
IL-23 induced in keratinocytes by endogenous TLR4 ligands polarizes dendritic cells to drive IL-22 responses to skin immunization. J Exp Med. 2016 09 19; 213(10):2147-66. View Abstract
Mutations in pyrin masquerading as a primary immunodeficiency. Clin Immunol. 2016 Oct; 171:65-66. View Abstract
IL-22 derived from ?d T cells restricts Staphylococcus aureus infection of mechanically injured skin. J Allergy Clin Immunol. 2016 10; 138(4):1098-1107.e3. View Abstract
Rapid molecular diagnostics of severe primary immunodeficiency determined by using targeted next-generation sequencing. J Allergy Clin Immunol. 2016 10; 138(4):1142-1151.e2. View Abstract
Dedicator of cytokinesis 8 regulates signal transducer and activator of transcription 3 activation and promotes TH17 cell differentiation. J Allergy Clin Immunol. 2016 11; 138(5):1384-1394.e2. View Abstract
Hyper IgM Syndrome: a Report from the USIDNET Registry. J Clin Immunol. 2016 07; 36(5):490-501. View Abstract
Combined immunodeficiency due to a homozygous mutation in ORAI1 that deletes the C-terminus that interacts with STIM 1. Clin Immunol. 2016 05; 166-167:100-2. View Abstract
Hematopoietic stem cell transplantation outcomes for 11 patients with dedicator of cytokinesis 8 deficiency. J Allergy Clin Immunol. 2016 09; 138(3):852-859.e3. View Abstract
Chronic mucocutaneous candidiasis associated with an SH2 domain gain-of-function mutation that enhances STAT1 phosphorylation. J Allergy Clin Immunol. 2016 07; 138(1):297-299. View Abstract
O-008 Aberrant Anti-inflammatory Macrophage Function and Differentiation in Wiskott-Aldrich Syndrome Protein-Deficient Mice and Humans. Inflamm Bowel Dis. 2016 Mar; 22 Suppl 1:S3. View Abstract
Thymic stromal lymphopoietin and IL-33 promote skin inflammation and vaccinia virus replication in a mouse model of atopic dermatitis. J Allergy Clin Immunol. 2016 07; 138(1):283-286. View Abstract
Spectrum of Phenotypes Associated with Mutations in LRBA. J Clin Immunol. 2016 Jan; 36(1):33-45. View Abstract
A missense mutation in TFRC, encoding transferrin receptor 1, causes combined immunodeficiency. Nat Genet. 2016 Jan; 48(1):74-8. View Abstract
Recurrent viral infections associated with a homozygous CORO1A mutation that disrupts oligomerization and cytoskeletal association. J Allergy Clin Immunol. 2016 Mar; 137(3):879-88.e2. View Abstract
Autoimmune lymphoproliferative syndrome caused by a homozygous FasL mutation that disrupts FasL assembly. J Allergy Clin Immunol. 2016 Jan; 137(1):324-327.e2. View Abstract
Wiskott-Aldrich Syndrome Interacting Protein Deficiency Uncovers the Role of the Co-receptor CD19 as a Generic Hub for PI3 Kinase Signaling in B Cells. Immunity. 2015 Oct 20; 43(4):660-73. View Abstract
Broad spectrum of autoantibodies in patients with Wiskott-Aldrich syndrome and X-linked thrombocytopenia. J Allergy Clin Immunol. 2015 Nov; 136(5):1401-4.e1-3. View Abstract
Targeted deep sequencing identifies rare loss-of-function variants in IFNGR1 for risk of atopic dermatitis complicated by eczema herpeticum. J Allergy Clin Immunol. 2015 Dec; 136(6):1591-1600. View Abstract
A novel mutation in NCF2 associated with autoimmune disease and a solitary late-onset infection. Clin Immunol. 2015 Dec; 161(2):128-30. View Abstract
Inherited DOCK2 Deficiency in Patients with Early-Onset Invasive Infections. N Engl J Med. 2015 Jun 18; 372(25):2409-22. View Abstract
A novel mutation in ORAI1 presenting with combined immunodeficiency and residual T-cell function. J Allergy Clin Immunol. 2015 Aug; 136(2):479-482.e1. View Abstract
The Rho GTPase Cdc42 Is Essential for the Activation and Function of Mature B Cells. J Immunol. 2015 May 15; 194(10):4750-8. View Abstract
The extended clinical phenotype of 64 patients with dedicator of cytokinesis 8 deficiency. J Allergy Clin Immunol. 2015 Aug; 136(2):402-12. View Abstract
A novel mutation in ICOS presenting as hypogammaglobulinemia with susceptibility to opportunistic pathogens. J Allergy Clin Immunol. 2015 Sep; 136(3):794-797.e1. View Abstract
Filaggrin deficiency promotes the dissemination of cutaneously inoculated vaccinia virus. J Allergy Clin Immunol. 2015 Jun; 135(6):1511-8.e6. View Abstract
Defective lymphoid organogenesis underlies the immune deficiency caused by a heterozygous S32I mutation in I?Ba. J Exp Med. 2015 Feb 09; 212(2):185-202. View Abstract
The microbiota is important for IL-17A expression and neutrophil infiltration in lesional skin of Flg(ft/ft) mice. Clin Immunol. 2015 Feb; 156(2):128-30. View Abstract
Regulatory T-cell deficiency and immune dysregulation, polyendocrinopathy, enteropathy, X-linked-like disorder caused by loss-of-function mutations in LRBA. J Allergy Clin Immunol. 2015 Jan; 135(1):217-27. View Abstract
Binding of WIP to actin is essential for T cell actin cytoskeleton integrity and tissue homing. Mol Cell Biol. 2014 Dec 01; 34(23):4343-54. View Abstract
Dedicator of cytokinesis 8-deficient patients have a breakdown in peripheral B-cell tolerance and defective regulatory T cells. J Allergy Clin Immunol. 2014 Dec; 134(6):1365-1374. View Abstract
A novel mutation in FOXN1 resulting in SCID: a case report and literature review. Clin Immunol. 2014 Nov; 155(1):30-32. View Abstract
The CARD11-BCL10-MALT1 (CBM) signalosome complex: Stepping into the limelight of human primary immunodeficiency. J Allergy Clin Immunol. 2014 Aug; 134(2):276-84. View Abstract
CCL25/CCR9 interactions are not essential for colitis development but are required for innate immune cell protection from chronic experimental murine colitis. Inflamm Bowel Dis. 2014 Jul; 20(7):1165-76. View Abstract
Lessons in gene hunting: a RAG1 mutation presenting with agammaglobulinemia and absence of B cells. J Allergy Clin Immunol. 2014 Oct; 134(4):983-5.e1. View Abstract
A novel disease-causing CD40L mutation reduces expression of CD40 ligand, but preserves CD40 binding capacity. Clin Immunol. 2014 Aug; 153(2):288-91. View Abstract
Somatic reversion in dedicator of cytokinesis 8 immunodeficiency modulates disease phenotype. J Allergy Clin Immunol. 2014 Jun; 133(6):1667-75. View Abstract
Plasmacytoid dendritic cell depletion in DOCK8 deficiency: rescue of severe herpetic infections with IFN-a 2b therapy. J Allergy Clin Immunol. 2014 Jun; 133(6):1753-5.e3. View Abstract
Leucine-rich repeat containing 8A (LRRC8A) is essential for T lymphocyte development and function. J Exp Med. 2014 May 05; 211(5):929-42. View Abstract
Presence of hypogammaglobulinemia and abnormal antibody responses in GATA2 deficiency. J Allergy Clin Immunol. 2014 Jul; 134(1):223-6. View Abstract
Flow cytometry diagnosis of dedicator of cytokinesis 8 (DOCK8) deficiency. J Allergy Clin Immunol. 2014 Jul; 134(1):221-3. View Abstract
TRIF signaling is essential for TLR4-driven IgE class switching. J Immunol. 2014 Mar 15; 192(6):2651-8. View Abstract
Food allergy: Insights into etiology, prevention, and treatment provided by murine models. J Allergy Clin Immunol. 2014 Feb; 133(2):309-17. View Abstract
Flow cytometry biomarkers distinguish DOCK8 deficiency from severe atopic dermatitis. Clin Immunol. 2014 Feb; 150(2):220-4. View Abstract
A systematic analysis of recombination activity and genotype-phenotype correlation in human recombination-activating gene 1 deficiency. J Allergy Clin Immunol. 2014 Apr; 133(4):1099-108. View Abstract
Gene hunting in the genomic era: approaches to diagnostic dilemmas in patients with primary immunodeficiencies. J Allergy Clin Immunol. 2014 Aug; 134(2):262-8. View Abstract
A homozygous mucosa-associated lymphoid tissue 1 (MALT1) mutation in a family with combined immunodeficiency. J Allergy Clin Immunol. 2013 Jul; 132(1):151-8. View Abstract
C3a receptor promotes viral containment in mice inoculated with vaccinia virus at sites of allergic skin inflammation. J Allergy Clin Immunol. 2013 Sep; 132(3):746-748.e3. View Abstract
A phenotypic approach for IUIS PID classification and diagnosis: guidelines for clinicians at the bedside. J Clin Immunol. 2013 Aug; 33(6):1078-87. View Abstract
Development of skin lesions in filaggrin-deficient mice is dependent on adaptive immunity. J Allergy Clin Immunol. 2013 Apr; 131(4):1247-50, 1250.e1. View Abstract
Wiskott-Aldrich syndrome: a comprehensive review. Ann N Y Acad Sci. 2013 May; 1285:26-43. View Abstract
Defective actin accumulation impairs human natural killer cell function in patients with dedicator of cytokinesis 8 deficiency. J Allergy Clin Immunol. 2013 Mar; 131(3):840-8. View Abstract
Filaggrin-dependent secretion of sphingomyelinase protects against staphylococcal a-toxin-induced keratinocyte death. J Allergy Clin Immunol. 2013 Feb; 131(2):421-7.e1-2. View Abstract
Intronic SH2D1A mutation with impaired SAP expression and agammaglobulinemia. Clin Immunol. 2013 Feb; 146(2):84-9. View Abstract
Use of whole exome and genome sequencing in the identification of genetic causes of primary immunodeficiencies. Curr Opin Allergy Clin Immunol. 2012 Dec; 12(6):623-8. View Abstract
Leukotriene B4-driven neutrophil recruitment to the skin is essential for allergic skin inflammation. Immunity. 2012 Oct 19; 37(4):747-58. View Abstract
Enteropathogenic Escherichia coli and vaccinia virus do not require the family of WASP-interacting proteins for pathogen-induced actin assembly. Infect Immun. 2012 Dec; 80(12):4071-7. View Abstract
SLP-76 is required for high-affinity IgE receptor- and IL-3 receptor-mediated activation of basophils. Int Immunol. 2012 Nov; 24(11):719-27. View Abstract
A novel homozygous mutation in recombination activating gene 2 in 2 relatives with different clinical phenotypes: Omenn syndrome and hyper-IgM syndrome. J Allergy Clin Immunol. 2012 Dec; 130(6):1414-6. View Abstract
LPS-responsive beige-like anchor (LRBA) gene mutation in a family with inflammatory bowel disease and combined immunodeficiency. J Allergy Clin Immunol. 2012 Aug; 130(2):481-8.e2. View Abstract
DOCK8 functions as an adaptor that links TLR-MyD88 signaling to B cell activation. Nat Immunol. 2012 May 13; 13(6):612-20. View Abstract
Clinical, immunologic and genetic profiles of DOCK8-deficient patients in Kuwait. Clin Immunol. 2012 Jun; 143(3):266-72. View Abstract
Eosinophil-derived leukotriene C4 signals via type 2 cysteinyl leukotriene receptor to promote skin fibrosis in a mouse model of atopic dermatitis. Proc Natl Acad Sci U S A. 2012 Mar 27; 109(13):4992-7. View Abstract
B cell-intrinsic deficiency of the Wiskott-Aldrich syndrome protein (WASp) causes severe abnormalities of the peripheral B-cell compartment in mice. Blood. 2012 Mar 22; 119(12):2819-28. View Abstract
A novel primary human immunodeficiency due to deficiency in the WASP-interacting protein WIP. J Exp Med. 2012 Jan 16; 209(1):29-34. View Abstract
Vaccinia Ig ameliorates eczema vaccinatum in a murine model of atopic dermatitis. J Invest Dermatol. 2012 Apr; 132(4):1299-301. View Abstract
Primary immunodeficiency diseases: an update on the classification from the international union of immunological societies expert committee for primary immunodeficiency. Front Immunol. 2011; 2:54. View Abstract
Immunization with modified vaccinia virus Ankara prevents eczema vaccinatum in a murine model of atopic dermatitis. J Allergy Clin Immunol. 2011 Oct; 128(4):890-892.e3. View Abstract
Toll-like receptor 9, transmembrane activator and calcium-modulating cyclophilin ligand interactor, and CD40 synergize in causing B-cell activation. J Allergy Clin Immunol. 2011 Sep; 128(3):601-9.e1-4. View Abstract
Epicutaneous challenge of orally immunized mice redirects antigen-specific gut-homing T cells to the skin. J Clin Invest. 2011 Jun; 121(6):2210-20. View Abstract
Functional analysis of transmembrane activator and calcium-modulating cyclophilin ligand interactor (TACI) mutations associated with common variable immunodeficiency. J Allergy Clin Immunol. 2011 Jul; 128(1):226-228.e1. View Abstract
A peptide derived from the Wiskott-Aldrich syndrome (WAS) protein-interacting protein (WIP) restores WAS protein level and actin cytoskeleton reorganization in lymphocytes from patients with WAS mutations that disrupt WIP binding. J Allergy Clin Immunol. 2011 Apr; 127(4):998-1005.e1-2. View Abstract
Ligation of CD46 to CD40 inhibits CD40 signaling in B cells. Int Immunol. 2011 Mar; 23(3):215-21. View Abstract
Successful engraftment of donor marrow after allogeneic hematopoietic cell transplantation in autosomal-recessive hyper-IgE syndrome caused by dedicator of cytokinesis 8 deficiency. J Allergy Clin Immunol. 2010 Dec; 126(6):1304-5.e3. View Abstract
The C104R mutant impairs the function of transmembrane activator and calcium modulator and cyclophilin ligand interactor (TACI) through haploinsufficiency. J Allergy Clin Immunol. 2010 Dec; 126(6):1234-41.e2. View Abstract
Mechanical injury polarizes skin dendritic cells to elicit a T(H)2 response by inducing cutaneous thymic stromal lymphopoietin expression. J Allergy Clin Immunol. 2010 Nov; 126(5):976-84, 984.e1-5. View Abstract
Clinical features and outcome of patients with IRAK-4 and MyD88 deficiency. Medicine (Baltimore). 2010 Nov; 89(6):403-425. View Abstract
Cdc42 interacting protein 4 (CIP4) is essential for integrin-dependent T-cell trafficking. Proc Natl Acad Sci U S A. 2010 Sep 14; 107(37):16252-6. View Abstract
The prostaglandin D2 receptor CRTH2 is important for allergic skin inflammation after epicutaneous antigen challenge. J Allergy Clin Immunol. 2010 Oct; 126(4):784-90. View Abstract
Parental consanguinity and the risk of primary immunodeficiency disorders: report from the Kuwait National Primary Immunodeficiency Disorders Registry. Int Arch Allergy Immunol. 2011; 154(1):76-80. View Abstract
Immune deficiency caused by impaired expression of nuclear factor-kappaB essential modifier (NEMO) because of a mutation in the 5' untranslated region of the NEMO gene. J Allergy Clin Immunol. 2010 Jul; 126(1):127-32.e7. View Abstract
Thymic stromal lymphopoietin. Ann N Y Acad Sci. 2010 Jan; 1183:13-24. View Abstract
Exaggerated IL-17 response to epicutaneous sensitization mediates airway inflammation in the absence of IL-4 and IL-13. J Allergy Clin Immunol. 2009 Oct; 124(4):761-70.e1. View Abstract
Platelet-associated IgAs and impaired GPVI responses in platelets lacking WIP. Blood. 2009 Nov 19; 114(21):4729-37. View Abstract
Vaccinia virus inoculation in sites of allergic skin inflammation elicits a vigorous cutaneous IL-17 response. Proc Natl Acad Sci U S A. 2009 Sep 01; 106(35):14954-9. View Abstract
Filaggrin-deficient mice exhibit TH17-dominated skin inflammation and permissiveness to epicutaneous sensitization with protein antigen. J Allergy Clin Immunol. 2009 Sep; 124(3):485-93, 493.e1. View Abstract
The murine equivalent of the A181E TACI mutation associated with common variable immunodeficiency severely impairs B-cell function. Blood. 2009 Sep 10; 114(11):2254-62. View Abstract
Reply to Narra. J Allergy Clin Immunol. 2009 Jun 01; 123(6):1419-1420. View Abstract
Transmembrane activator, calcium modulator, and cyclophilin ligand interactor drives plasma cell differentiation in LPS-activated B cells. J Allergy Clin Immunol. 2009 Jun; 123(6):1277-86.e5. View Abstract
IL-21R is essential for epicutaneous sensitization and allergic skin inflammation in humans and mice. J Clin Invest. 2009 Jan; 119(1):47-60. View Abstract
Transmembrane activator and calcium-modulator and cyclophilin ligand interactor mutations in common variable immunodeficiency. Curr Opin Allergy Clin Immunol. 2008 Dec; 8(6):520-6. View Abstract
Allogeneic transplantation successfully corrects immune defects, but not susceptibility to colitis, in a patient with nuclear factor-kappaB essential modulator deficiency. J Allergy Clin Immunol. 2008 Dec; 122(6):1113-1118.e1. View Abstract
A girl with severe hand swelling and abdominal cramps. Clin Chem. 2008 Oct; 54(10):1733-6; discussion 1736-7. View Abstract
TSLP acts on infiltrating effector T cells to drive allergic skin inflammation. Proc Natl Acad Sci U S A. 2008 Aug 19; 105(33):11875-80. View Abstract
Cutting edge: the dependence of plasma cells and independence of memory B cells on BAFF and APRIL. J Immunol. 2008 Mar 15; 180(6):3655-9. View Abstract
Tyrosine kinases Btk and Tec regulate osteoclast differentiation by linking RANK and ITAM signals. Cell. 2008 Mar 07; 132(5):794-806. View Abstract
Distinct regulatory functions of SLP-76 and MIST in NK cell cytotoxicity and IFN-gamma production. Int Immunol. 2008 Mar; 20(3):345-52. View Abstract
Heterozygous N-terminal deletion of IkappaBalpha results in functional nuclear factor kappaB haploinsufficiency, ectodermal dysplasia, and immune deficiency. J Allergy Clin Immunol. 2007 Oct; 120(4):900-7. View Abstract
Primary immunodeficiency diseases: an update from the International Union of Immunological Societies Primary Immunodeficiency Diseases Classification Committee. J Allergy Clin Immunol. 2007 Oct; 120(4):776-94. View Abstract
Impaired immune response to vaccinia virus inoculated at the site of cutaneous allergic inflammation. J Allergy Clin Immunol. 2007 Dec; 120(6):1382-8. View Abstract
Epicutaneous antigen exposure induces a Th17 response that drives airway inflammation after inhalation challenge. Proc Natl Acad Sci U S A. 2007 Oct 02; 104(40):15817-22. View Abstract
A novel anti-WIP monoclonal antibody detects an isoform of WIP that lacks the WASP binding domain. Biochem Biophys Res Commun. 2007 Feb 23; 353(4):875-81. View Abstract
Primary immunodeficiency diseases: an update from the International Union of Immunological Societies Primary Immunodeficiency Diseases Classification Committee Meeting in Budapest, 2005. J Allergy Clin Immunol. 2006 Apr; 117(4):883-96. View Abstract
A second-site mutation in the initiation codon of WAS (WASP) results in expansion of subsets of lymphocytes in an Wiskott-Aldrich syndrome patient. Hum Mutat. 2006 Apr; 27(4):370-5. View Abstract
Molecular basis of common variable immunodeficiency. J Allergy Clin Immunol. 2006 Apr; 117(4):740-6; quiz 747. View Abstract
The complement component C3 plays a critical role in both Th1 and Th2 responses to antigen. J Allergy Clin Immunol. 2006 Jun; 117(6):1455-61. View Abstract
Lessons learned from molecular defects in nuclear factor kappaB dependent signaling. Microbes Infect. 2006 Apr; 8(4):1151-6. View Abstract
A 10-aa-long sequence in SLP-76 upstream of the Gads binding site is essential for T cell development and function. Proc Natl Acad Sci U S A. 2005 Dec 27; 102(52):19063-8. View Abstract
Defective nuclear translocation of nuclear factor of activated T cells and extracellular signal-regulated kinase underlies deficient IL-2 gene expression in Wiskott-Aldrich syndrome. J Allergy Clin Immunol. 2005 Dec; 116(6):1364-71. View Abstract
Charles A. Janeway and Fred S. Rosen: the discovery of gamma globulin therapy and primary immunodeficiency diseases at Boston Children's Hospital. J Allergy Clin Immunol. 2005 Oct; 116(4):937-40. View Abstract
WIP and WASP play complementary roles in T cell homing and chemotaxis to SDF-1alpha. Int Immunol. 2006 Feb; 18(2):221-32. View Abstract
COX-2 inhibition enhances the TH2 immune response to epicutaneous sensitization. J Allergy Clin Immunol. 2005 Aug; 116(2):390-6. View Abstract
Are you immunodeficient? J Allergy Clin Immunol. 2005 Aug; 116(2):423-5. View Abstract
TACI is mutant in common variable immunodeficiency and IgA deficiency. Nat Genet. 2005 Aug; 37(8):829-34. View Abstract
Fc{epsilon}RI-mediated mast cell degranulation requires calcium-independent microtubule-dependent translocation of granules to the plasma membrane. J Cell Biol. 2005 Jul 04; 170(1):115-26. View Abstract
Role of APRIL in the mucosal Iga antibody response. J Pediatr Gastroenterol Nutr. 2005 Apr; 40 Suppl 1:S27. View Abstract
Jun N-terminal kinase is essential for CD40-mediated IgE class switching in B cells. J Allergy Clin Immunol. 2005 Apr; 115(4):856-63. View Abstract
Human disease resulting from gene mutations that interfere with appropriate nuclear factor-kappaB activation. Immunol Rev. 2005 Feb; 203:21-37. View Abstract
TACI and BAFF-R mediate isotype switching in B cells. J Exp Med. 2005 Jan 03; 201(1):35-9. View Abstract
Characteristics of mycobacterial infection in patients with immunodeficiency and nuclear factor-kappaB essential modulator mutation, with or without ectodermal dysplasia. J Am Acad Dermatol. 2004 Nov; 51(5):718-22. View Abstract
Human nuclear factor kappa B essential modulator mutation can result in immunodeficiency without ectodermal dysplasia. J Allergy Clin Immunol. 2004 Sep; 114(3):650-6. View Abstract
The anaphylatoxin C3a downregulates the Th2 response to epicutaneously introduced antigen. J Clin Invest. 2004 Aug; 114(3):399-407. View Abstract
RIP links TLR4 to Akt and is essential for cell survival in response to LPS stimulation. J Exp Med. 2004 Aug 02; 200(3):399-404. View Abstract
Erk/Src phosphorylation of cortactin acts as a switch on-switch off mechanism that controls its ability to activate N-WASP. Mol Cell Biol. 2004 Jun; 24(12):5269-80. View Abstract
The presentation and natural history of immunodeficiency caused by nuclear factor kappaB essential modulator mutation. J Allergy Clin Immunol. 2004 Apr; 113(4):725-33. View Abstract
Induction of activation-induced cytidine deaminase gene expression by IL-4 and CD40 ligation is dependent on STAT6 and NFkappaB. Int Immunol. 2004 Mar; 16(3):395-404. View Abstract
Impaired IgA class switching in APRIL-deficient mice. Proc Natl Acad Sci U S A. 2004 Mar 16; 101(11):3903-8. View Abstract
WIP regulates signaling via the high affinity receptor for immunoglobulin E in mast cells. J Exp Med. 2004 Feb 02; 199(3):357-68. View Abstract
Allergy and hypersensitivity. Nature versus nurture in allergy and hypersensitivity. Curr Opin Immunol. 2003 Dec; 15(6):603-8. View Abstract
Impaired signaling via the high-affinity IgE receptor in Wiskott-Aldrich syndrome protein-deficient mast cells. Int Immunol. 2003 Dec; 15(12):1431-40. View Abstract
The mature activating natural killer cell immunologic synapse is formed in distinct stages. Proc Natl Acad Sci U S A. 2003 Nov 25; 100(24):14151-6. View Abstract
The X-linked hyper-IgM syndrome: clinical and immunologic features of 79 patients. Medicine (Baltimore). 2003 Nov; 82(6):373-84. View Abstract
IL-10 is critical for Th2 responses in a murine model of allergic dermatitis. J Clin Invest. 2003 Oct; 112(7):1058-66. View Abstract
Disseminated varicella infection due to the vaccine strain of varicella-zoster virus, in a patient with a novel deficiency in natural killer T cells. J Infect Dis. 2003 Oct 01; 188(7):948-53. View Abstract
The regulation of immunoglobulin E class-switch recombination. Nat Rev Immunol. 2003 Sep; 3(9):721-32. View Abstract
Acute chylothorax in children: selective retention of memory T cells and natural killer cells. J Pediatr. 2003 Aug; 143(2):243-9. View Abstract
WAVE2 deficiency reveals distinct roles in embryogenesis and Rac-mediated actin-based motility. EMBO J. 2003 Jul 15; 22(14):3602-12. View Abstract
Desloratadine inhibits allergen-induced airway inflammation and bronchial hyperresponsiveness and alters T-cell responses in murine models of asthma. J Allergy Clin Immunol. 2003 Jul; 112(1):149-58. View Abstract
C4b-binding protein (C4BP) activates B cells through the CD40 receptor. Immunity. 2003 Jun; 18(6):837-48. View Abstract
WIP participates in actin reorganization and ruffle formation induced by PDGF. J Cell Sci. 2003 Jun 15; 116(Pt 12):2443-51. View Abstract
Structural requirements of SLP-76 in signaling via the high-affinity immunoglobulin E receptor (Fc epsilon RI) in mast cells. Mol Cell Biol. 2003 Apr; 23(7):2395-406. View Abstract
Mechanism of recruitment of WASP to the immunological synapse and of its activation following TCR ligation. Mol Cell. 2002 Dec; 10(6):1269-81. View Abstract
The binding site for TRAF2 and TRAF3 but not for TRAF6 is essential for CD40-mediated immunoglobulin class switching. Immunity. 2002 Sep; 17(3):265-76. View Abstract
Wiskott-Aldrich syndrome protein is required for NK cell cytotoxicity and colocalizes with actin to NK cell-activating immunologic synapses. Proc Natl Acad Sci U S A. 2002 Aug 20; 99(17):11351-6. View Abstract
Deficient natural killer cell cytotoxicity in patients with IKK-gamma/NEMO mutations. J Clin Invest. 2002 Jun; 109(11):1501-9. View Abstract
Upregulation of IL-13 concentration in vivo by the IL13 variant associated with bronchial asthma. J Allergy Clin Immunol. 2002 Jun; 109(6):980-7. View Abstract
CCR3 is essential for skin eosinophilia and airway hyperresponsiveness in a murine model of allergic skin inflammation. J Clin Invest. 2002 Mar; 109(5):621-8. View Abstract
WIP deficiency reveals a differential role for WIP and the actin cytoskeleton in T and B cell activation. Immunity. 2002 Feb; 16(2):193-204. View Abstract
Differential role of SLP-76 domains in T cell development and function. Proc Natl Acad Sci U S A. 2002 Jan 22; 99(2):884-9. View Abstract
Mast cells regulate IFN-gamma expression in the skin and circulating IgE levels in allergen-induced skin inflammation. J Allergy Clin Immunol. 2002 Jan; 109(1):106-13. View Abstract
IL-3 induces B7.2 (CD86) expression and costimulatory activity in human eosinophils. J Immunol. 2001 Dec 01; 167(11):6097-104. View Abstract
An obligate role for T-cell receptor alphabeta+ T cells but not T-cell receptor gammadelta+ T cells, B cells, or CD40/CD40L interactions in a mouse model of atopic dermatitis. J Allergy Clin Immunol. 2001 Feb; 107(2):359-66. View Abstract
Roles of SLP-76, phosphoinositide 3-kinase, and gelsolin in the platelet shape changes initiated by the collagen receptor GPVI/FcR gamma-chain complex. Blood. 2000 Dec 01; 96(12):3786-92. View Abstract
Allergy, a disease of the internal and external environments Curr Opin Immunol. 2000 Dec; 12(6):615-7. View Abstract
CD40L, but not CD40, is required for allergen-induced bronchial hyperresponsiveness in mice. Am J Respir Cell Mol Biol. 2000 Nov; 23(5):646-51. View Abstract
Multicenter, double-blind, placebo-controlled, multiple-challenge evaluation of reported reactions to monosodium glutamate. J Allergy Clin Immunol. 2000 Nov; 106(5):973-80. View Abstract
Identification and characterization of two CD40-inducible enhancers in the mouse TRAF1 gene locus. Mol Immunol. 2000 Nov; 37(16):961-73. View Abstract
The role of SLP-76 and LAT in lymphocyte development. Curr Opin Immunol. 2000 Apr; 12(2):173-8. View Abstract
Review of alleged reaction to monosodium glutamate and outcome of a multicenter double-blind placebo-controlled study. J Nutr. 2000 04; 130(4S Suppl):1058S-62S. View Abstract
Adapter proteins SLP-76 and BLNK both are expressed by murine macrophages and are linked to signaling via Fcgamma receptors I and II/III. Proc Natl Acad Sci U S A. 2000 Feb 15; 97(4):1725-30. View Abstract
Allelic exclusion of the T cell receptor beta locus requires the SH2 domain-containing leukocyte protein (SLP)-76 adaptor protein. J Exp Med. 1999 Oct 18; 190(8):1093-102. View Abstract
IgE in asthma and atopy: cellular and molecular connections. J Clin Invest. 1999 Oct; 104(7):829-35. View Abstract
Ku in the cytoplasm associates with CD40 in human B cells and translocates into the nucleus following incubation with IL-4 and anti-CD40 mAb. Immunity. 1999 Sep; 11(3):339-48. View Abstract
The human WASP-interacting protein, WIP, activates the cell polarity pathway in yeast. J Biol Chem. 1999 Jun 11; 274(24):17103-8. View Abstract
SLP-76 deficiency impairs signaling via the high-affinity IgE receptor in mast cells. J Clin Invest. 1999 Jun; 103(12):1737-43. View Abstract
Structure of the murine TRAF1 gene. Mol Immunol. 1999 Jun; 36(9):611-7. View Abstract
Contribution of CD40-CD154-mediated costimulation to an alloresponse in vivo. Transplantation. 1999 May 15; 67(9):1284-7. View Abstract
Effect of intravenous immunoglobulin on steroid consumption in patients with severe asthma: a double-blind, placebo-controlled, randomized trial. J Allergy Clin Immunol. 1999 May; 103(5 Pt 1):810-5. View Abstract
Roles of TH1 and TH2 cytokines in a murine model of allergic dermatitis. J Clin Invest. 1999 Apr; 103(8):1103-11. View Abstract
Regulation of IgE synthesis: the molecular basis and implications for clinical modulation. Allergy Asthma Proc. 1999 Jan-Feb; 20(1):1-8. View Abstract
The mouse genome contains two expressed intronless retroposed pseudogenes for the sentrin/sumo-1/PIC1 conjugating enzyme Ubc9. Mol Immunol. 1998 Nov; 35(16):1057-67. View Abstract
Role of JAK3 in CD40-mediated signaling. Blood. 1998 Oct 01; 92(7):2435-40. View Abstract
The X-linked lymphoproliferative-disease gene product SAP regulates signals induced through the co-receptor SLAM. Nature. 1998 Oct 01; 395(6701):462-9. View Abstract
The Wiskott-Aldrich syndrome protein-interacting protein (WIP) binds to the adaptor protein Nck. J Biol Chem. 1998 Aug 14; 273(33):20992-5. View Abstract
Impaired viability and profound block in thymocyte development in mice lacking the adaptor protein SLP-76. Cell. 1998 Jul 24; 94(2):229-38. View Abstract
IgE-mediated allergy and desensitization to factor IX in hemophilia B. J Allergy Clin Immunol. 1998 Jul; 102(1):113-7. View Abstract
Epicutaneous sensitization with protein antigen induces localized allergic dermatitis and hyperresponsiveness to methacholine after single exposure to aerosolized antigen in mice. J Clin Invest. 1998 Apr 15; 101(8):1614-22. View Abstract
Molecular mechanisms of immunoglobulin E regulation. Int Arch Allergy Immunol. 1998 Apr; 115(4):257-69. View Abstract
CD40 ligation and IL-4 use different mechanisms of transcriptional activation of the human lymphotoxin alpha promoter in B cells. Eur J Immunol. 1998 Mar; 28(3):901-6. View Abstract
WIP, a protein associated with wiskott-aldrich syndrome protein, induces actin polymerization and redistribution in lymphoid cells. Proc Natl Acad Sci U S A. 1997 Dec 23; 94(26):14671-6. View Abstract
Targeted gene disruption of murine CD7. Int Immunol. 1997 Dec; 9(12):1875-83. View Abstract
Impaired CD19 expression and signaling, enhanced antibody response to type II T independent antigen and reduction of B-1 cells in CD81-deficient mice. Proc Natl Acad Sci U S A. 1997 Sep 30; 94(20):10844-9. View Abstract
Homodimerization of the human interleukin 4 receptor alpha chain induces Cepsilon germline transcripts in B cells in the absence of the interleukin 2 receptor gamma chain. Proc Natl Acad Sci U S A. 1997 May 27; 94(11):5866-71. View Abstract
Jak3 is associated with CD40 and is critical for CD40 induction of gene expression in B cells. Immunity. 1997 Apr; 6(4):379-87. View Abstract
Searching for genes involved in the pathogenesis of primary immunodeficiency diseases: lessons from mouse knockouts. J Clin Immunol. 1997 Mar; 17(2):109-26. View Abstract
Impaired B cell maturation in mice lacking Bruton's tyrosine kinase (Btk) and CD40. Int Immunol. 1997 Mar; 9(3):395-405. View Abstract
CD30 induction of human immunodeficiency virus gene transcription is mediated by TRAF2. Proc Natl Acad Sci U S A. 1997 Feb 18; 94(4):1390-5. View Abstract
HIV glycoprotein gp120 inhibits TCR-CD3-mediated activation of fyn and lck. Int Immunol. 1997 Jan; 9(1):53-64. View Abstract
Association of human fas (CD95) with a ubiquitin-conjugating enzyme (UBC-FAP). J Biol Chem. 1996 Dec 06; 271(49):31037-43. View Abstract
Large-volume leukapheresis in pediatric patients: processing more blood diminishes the apparent magnitude of intra-apheresis recruitment. Transfusion. 1996 Oct; 36(10):879-85. View Abstract
The superantigen toxic shock syndrome toxin-1 induces CD40 ligand expression and modulates IgE isotype switching. Int Immunol. 1996 Oct; 8(10):1503-10. View Abstract
Induction of alloantigen-specific tolerance by B cells from CD40-deficient mice. Proc Natl Acad Sci U S A. 1996 May 14; 93(10):4994-8. View Abstract
Mechanisms of inhibition of IgE synthesis by nedocromil sodium: nedocromil sodium inhibits deletional switch recombination in human B cells. J Allergy Clin Immunol. 1996 May; 97(5):1141-50. View Abstract
CD40 expression and function in murine B cell ontogeny. Int Immunol. 1996 Mar; 8(3):405-411. View Abstract
Natural Killer (NK) cell deficiency associated with an epitope-deficient Fc receptor type IIIA (CD16-II). Clin Exp Immunol. 1996 Mar; 103(3):408-13. View Abstract
The CD40L promoter contains nuclear factor of activated T cells-binding motifs which require AP-1 binding for activation of transcription. J Biol Chem. 1996 Feb 16; 271(7):3763-70. View Abstract
Characterization of a 23-kDa protein associated with CD40. Proc Natl Acad Sci U S A. 1995 Dec 05; 92(25):11633-6. View Abstract
Cross-linking of Fc gamma receptors activates HIV-1 long terminal repeat-driven transcription in human monocytes. Int Immunol. 1995 Oct; 7(10):1665-70. View Abstract
Defective expression of early activation genes in cartilage-hair hypoplasia (CHH) with severe combined immunodeficiency (SCID). Clin Exp Immunol. 1995 Oct; 102(1):6-10. View Abstract
CD40-mediated lymphotoxin alpha expression in human B cells is tyrosine kinase dependent. Eur J Immunol. 1995 Sep; 25(9):2438-44. View Abstract
Bacterial superantigens induce the proliferation of resting gamma/delta receptor bearing T cells. Immunol Invest. 1995 Aug; 24(5):713-24. View Abstract
Transcriptional activation of the human TNF-alpha promoter by superantigen in human monocytic cells: role of NF-kappa B. J Immunol. 1995 Jul 15; 155(2):902-8. View Abstract
CD40 ligand expression is developmentally regulated in human thymocytes. Clin Immunol Immunopathol. 1995 Jul; 76(1 Pt 1):52-8. View Abstract
Association of the high-affinity receptor for IgG with the protein tyrosine kinases Hck and Lyn. Int Arch Allergy Immunol. 1995 May-Jun; 107(1-3):135. View Abstract
Activation of tumor necrosis factor-alpha and lymphotoxin-alpha via anti-CD40 in human B cells. Int Arch Allergy Immunol. 1995 May-Jun; 107(1-3):368-9. View Abstract
Expression of the CD40 ligand in T lymphocytes and induction of IgE isotype switching. Int Arch Allergy Immunol. 1995 May-Jun; 107(1-3):43-4. View Abstract
A point mutation in exon 2 of the CD40 ligand gene causes the simultaneous expression of two defective mRNA species in X-linked hyperimmunoglobulinemia M. Hum Mol Genet. 1995 Apr; 4(4):759-61. View Abstract
Interleukin-4 receptor expression by human B cells: functional analysis with a human interleukin-4 toxin, DAB389IL-4. J Allergy Clin Immunol. 1995 Apr; 95(4):893-900. View Abstract
gamma/delta T lymphocytes express CD40 ligand and induce isotype switching in B lymphocytes. J Exp Med. 1995 Mar 01; 181(3):1239-44. View Abstract
Role of protein tyrosine kinases and phosphatases in isotype switching: crosslinking CD45 to CD40 inhibits IgE isotype switching in human B cells. Immunol Lett. 1995 Feb; 45(1-2):99-106. View Abstract
X-linked agammaglobulinemia and immunoglobulin deficiency with normal or elevated IgM: immunodeficiencies of B cell development and differentiation. Adv Immunol. 1995; 60:37-56. View Abstract
CD40-deficient mice generated by recombination-activating gene-2-deficient blastocyst complementation. Proc Natl Acad Sci U S A. 1994 Dec 06; 91(25):12135-9. View Abstract
CD40 ligation induces lymphotoxin alpha gene expression in human B cells. Int Immunol. 1994 Dec; 6(12):1883-90. View Abstract
Protein tyrosine kinase activation and protein kinase C translocation are functional components of CD40 signal transduction in resting human B cells. Immunol Invest. 1994 Nov; 23(6-7):437-48. View Abstract
A toxic shock syndrome toxin-1 peptide that shows homology to amino acids 180-193 of mycobacterial heat shock protein 65 is presented as conventional antigen. Immunol Invest. 1994 Nov; 23(6-7):381-91. View Abstract
Immunosuppressant deoxyspergualin inhibits antigen processing in monocytes. J Immunol. 1994 Nov 01; 153(9):3908-16. View Abstract
Role of protein tyrosine kinases in CD40/interleukin-4-mediated isotype switching to IgE. J Allergy Clin Immunol. 1994 Oct; 94(4):784-92. View Abstract
MHC class II signaling in B-cell activation. Immunol Today. 1994 Sep; 15(9):418-22. View Abstract
Physical and functional association of the high affinity immunoglobulin G receptor (Fc gamma RI) with the kinases Hck and Lyn. J Exp Med. 1994 Sep 01; 180(3):1165-70. View Abstract
Engagement of the common leukocyte antigen CD45 induces homotypic adhesion of activated human T cells. J Immunol. 1994 Aug 15; 153(4):1593-602. View Abstract
Decreased expression of the ligand for CD40 in newborn lymphocytes. Eur J Immunol. 1994 Aug; 24(8):1925-8. View Abstract
Disodium cromoglycate inhibits S mu-->S epsilon deletional switch recombination and IgE synthesis in human B cells. J Exp Med. 1994 Aug 01; 180(2):663-71. View Abstract
Structure of the murine CD40 ligand gene. Mol Immunol. 1994 Aug; 31(12):895-900. View Abstract
Molecular pathology of X-linked immunoglobulin deficiency with normal or elevated IgM (HIGMX-1). Immunol Rev. 1994 Apr; 138:87-104. View Abstract
Engagement of MHC class II molecules by staphylococcal superantigens activates src-type protein tyrosine kinases. Eur J Immunol. 1994 Mar; 24(3):651-8. View Abstract
Cyclosporin A inhibits CD40 ligand expression in T lymphocytes. J Clin Invest. 1994 Mar; 93(3):1315-20. View Abstract
Early activation events induced by the staphylococcal superantigen toxic shock syndrome toxin-1 in human peripheral blood monocytes. Clin Immunol Immunopathol. 1994 Feb; 70(2):137-44. View Abstract
Signal transduction via CD40 involves activation of lyn kinase and phosphatidylinositol-3-kinase, and phosphorylation of phospholipase C gamma 2. J Exp Med. 1994 Feb 01; 179(2):673-80. View Abstract
Role of CD40-CD40-ligand interaction in Ig-isotype switching. Curr Opin Immunol. 1993 Dec; 5(6):963-7. View Abstract
Sequential switching from mu to epsilon via gamma 4 in human B cells stimulated with IL-4 and hydrocortisone. J Immunol. 1993 Nov 01; 151(9):4528-33. View Abstract
Physical association between the high-affinity IgG receptor (Fc gamma RI) and the gamma subunit of the high-affinity IgE receptor (Fc epsilon RI gamma). Proc Natl Acad Sci U S A. 1993 Oct 01; 90(19):8847-50. View Abstract
Aspartame is no more likely than placebo to cause urticaria/angioedema: results of a multicenter, randomized, double-blind, placebo-controlled, crossover study. J Allergy Clin Immunol. 1993 Oct; 92(4):513-20. View Abstract
Activator protein-1 (AP-1) is stimulated by microbial superantigens in human monocytic cells. Eur J Immunol. 1993 Sep; 23(9):2129-35. View Abstract
Therapy for patients with recurrent infections and low serum IgG3 levels. J Allergy Clin Immunol. 1993 Aug; 92(2):353-5. View Abstract
Deletions in the ligand for CD40 in X-linked immunoglobulin deficiency with normal or elevated IgM (HIGMX-1). Int Immunol. 1993 Jul; 5(7):769-73. View Abstract
Microbial superantigens induce NF-kappa B in the human monocytic cell line THP-1. J Immunol. 1993 Jun 15; 150(12):5604-13. View Abstract
Molecular basis of a multiple lymphokine deficiency in a patient with severe combined immunodeficiency. Proc Natl Acad Sci U S A. 1993 May 15; 90(10):4728-32. View Abstract
Chromosomal localization of the gene for human B-cell antigen CD40. Somat Cell Mol Genet. 1993 May; 19(3):295-8. View Abstract
A protein of the AP-1 family is a component of nuclear factor of activated T cells. J Immunol. 1993 Apr 15; 150(8 Pt 1):3284-90. View Abstract
Defective expression of the CD40 ligand in X chromosome-linked immunoglobulin deficiency with normal or elevated IgM. Proc Natl Acad Sci U S A. 1993 Mar 15; 90(6):2170-3. View Abstract
Signal transduction by microbial superantigens via MHC class II molecules. Immunol Rev. 1993 Feb; 131:43-59. View Abstract
Severe combined immunodeficiency with selective T-cell cytokine genes. Pediatr Res. 1993 Jan; 33(1 Suppl):S20-2; discussion S22-3. View Abstract
Regulation of IgE synthesis: from the membrane to the genes. Springer Semin Immunopathol. 1993; 15(1):5-16. View Abstract
Localization of the genetic defect in X-linked immunoglobulin deficiency with normal or elevated IgM (HIGMX-1) to the CD40 ligand gene. Trans Assoc Am Physicians. 1993; 106:91-5. View Abstract
IgE regulation and lymphokine patterns in aging humans. J Allergy Clin Immunol. 1992 Oct; 90(4 Pt 1):630-6. View Abstract
Role of protein kinase activation in the induction of B cell adhesion by MHC class II ligands. J Immunol. 1992 Sep 15; 149(6):1853-8. View Abstract
Protein tyrosine phosphorylation induced via the IgG receptors Fc gamma Ri and Fc gamma RII in the human monocytic cell line THP-1. J Immunol. 1992 Sep 01; 149(5):1751-7. View Abstract
Regulation of IgE synthesis in humans. J Allergy Clin Immunol. 1992 Aug; 90(2):143-50. View Abstract
Signals delivered via MHC class II molecules synergize with signals delivered via TCR/CD3 to cause proliferation and cytokine gene expression in T cells. J Immunol. 1992 Jul 01; 149(1):65-70. View Abstract
Engagement of MHC class I molecules induces cell adhesion via both LFA-1-dependent and LFA-1-independent pathways. J Immunol. 1992 Apr 01; 148(7):2045-9. View Abstract
Role of protein tyrosine phosphorylation in monokine induction by the staphylococcal superantigen toxic shock syndrome toxin-1. J Immunol. 1992 Apr 01; 148(7):2237-41. View Abstract
Developmental regulation of transmembrane signaling via the T cell antigen receptor/CD3 complex in human T lymphocytes. J Immunol. 1992 Mar 01; 148(5):1315-21. View Abstract
A toxic shock syndrome toxin-1 peptide that shows homology to mycobacterial heat shock protein 18 is presented as conventional antigen to T cells by multiple HLA-DR alleles. J Immunol. 1992 Feb 15; 148(4):1025-30. View Abstract
Cellular and molecular mechanisms of immune activation by microbial superantigens: studies using toxic shock syndrome toxin-1. Chem Immunol. 1992; 55:146-71. View Abstract
Molecular analysis of the induction of immunoglobulin E synthesis in human B cells by interleukin 4 and engagement of CD40 antigen. J Exp Med. 1992 Jan 01; 175(1):289-92. View Abstract
Lymphokine profile in bone marrow transplant recipients. Blood. 1991 Dec 01; 78(11):3076-80. View Abstract
Cardiotoxicity during treatment of severe childhood asthma. Pediatrics. 1991 Dec; 88(6):1180-6. View Abstract
Deletional switch recombination occurs in interleukin-4-induced isotype switching to IgE expression by human B cells. Proc Natl Acad Sci U S A. 1991 Sep 01; 88(17):7528-32. View Abstract
Staphylococcal exotoxins deliver activation signals to human T-cell clones via major histocompatibility complex class II molecules. Proc Natl Acad Sci U S A. 1991 Sep 01; 88(17):7533-7. View Abstract
Regulation of IgE synthesis in humans: a tale of two signals. J Allergy Clin Immunol. 1991 Sep; 88(3 Pt 1):285-95. View Abstract
Hydrocortisone and IL-4 induce IgE isotype switching in human B cells. J Immunol. 1991 Sep 01; 147(5):1557-60. View Abstract
Engagement of CD14 on human monocytes terminates T cell proliferation by delivering a negative signal to T cells. J Immunol. 1991 Aug 15; 147(4):1134-8. View Abstract
Transcriptional activation of IL-1 beta and tumor necrosis factor-alpha genes by MHC class II ligands. J Immunol. 1991 Apr 01; 146(7):2310-5. View Abstract
Engagement of MHC-class II molecules by staphylococcal exotoxins delivers a comitogenic signal to human B cells. J Immunol. 1991 Mar 01; 146(5):1661-6. View Abstract
Toxic shock syndrome toxin-1, toxic shock, and the immune system. Curr Top Microbiol Immunol. 1991; 174:63-79. View Abstract
Induction of human T cell proliferation by a monoclonal antibody to CD5. J Immunol. 1991 Jan 01; 146(1):47-52. View Abstract
CD40 and IgE: synergism between anti-CD40 monoclonal antibody and interleukin 4 in the induction of IgE synthesis by highly purified human B cells. J Exp Med. 1990 Dec 01; 172(6):1861-4. View Abstract
Primary combined immunodeficiency resulting from defective transcription of multiple T-cell lymphokine genes. Proc Natl Acad Sci U S A. 1990 Dec; 87(24):10033-7. View Abstract
Induction of germ-line and mature C epsilon transcripts in human B cells stimulated with rIL-4 and EBV. J Immunol. 1990 Nov 15; 145(10):3468-73. View Abstract
Engagement of major histocompatibility complex class II molecules induces sustained, lymphocyte function-associated molecule 1-dependent cell adhesion. J Exp Med. 1990 Nov 01; 172(5):1513-6. View Abstract
Interleukin 4 down-regulates the expression of CD14 in normal human monocytes. Eur J Immunol. 1990 Nov; 20(11):2375-81. View Abstract
Binding of toxic shock syndrome toxin-1 to murine major histocompatibility complex class II molecules. Eur J Immunol. 1990 Sep; 20(9):1911-6. View Abstract
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Endogenous interleukin 6 plays an obligatory role in interleukin 4-dependent human IgE synthesis. Eur J Immunol. 1989 Aug; 19(8):1419-24. View Abstract
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The B-cell binding site on human immunoglobulin E. Nature. 1989 Apr 20; 338(6217):649-51. View Abstract
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An immunodeficiency characterized by defective signal transduction in T lymphocytes. N Engl J Med. 1989 Mar 16; 320(11):696-702. View Abstract
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Regulation of IgE synthesis in humans. J Clin Immunol. 1989 Mar; 9(2):75-83. View Abstract
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Regulation of human IgE synthesis. Int Rev Immunol. 1989; 5(2):111-5. View Abstract
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Impaired antibody responses in the hyperimmunoglobulin E syndrome. J Allergy Clin Immunol. 1988 Jun; 81(6):1082-7. View Abstract
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Human recombinant interleukin 4 induces Fc epsilon R2/CD23 on normal human monocytes. J Exp Med. 1988 Apr 01; 167(4):1406-16. View Abstract
Clinical and immunologic aspects of the hyperimmunoglobulin E syndrome. Hematol Oncol Clin North Am. 1988 Mar; 2(1):81-100. View Abstract
Regulation of IgE synthesis in atopic disease. Hosp Pract (Off Ed). 1988 Feb 15; 23(2):91-102. View Abstract
Toxic shock syndrome toxin-1 induces inositol phospholipid turnover, protein kinase C translocation, and calcium mobilization in human T cells. J Immunol. 1988 Feb 15; 140(4):1250-5. View Abstract
Asthma, recurrent infections and IgG2 deficiency. Ann Allergy. 1988 Feb; 60(2):98, 134-6. View Abstract
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Increased in vitro bone resorption by monocytes in the hyper-immunoglobulin E syndrome. J Immunol. 1988 Jan 01; 140(1):84-8. View Abstract
Cell cooperation in haploidentical bone marrow transplant recipient. Transplant Proc. 1987 Dec; 19(6 Suppl 7):117-21. View Abstract
Requirements for activation of human peripheral blood T cells by mouse monoclonal antibodies to CD3. Clin Immunol Immunopathol. 1987 Apr; 43(1):48-64. View Abstract
The presence of IgE on macrophages and dendritic cells infiltrating into the skin lesion of atopic dermatitis. Clin Immunol Immunopathol. 1987 Mar; 42(3):328-37. View Abstract
Regulation of the human IgE antibody response. Int Rev Immunol. 1987 Jan; 2(1):75-91. View Abstract
Polymerase activity in lymphocyte culture supernatants from patients with Kawasaki disease. Prog Clin Biol Res. 1987; 250:125-30. View Abstract
Polymerase activity in lymphocyte culture supernatants from patients with Kawasaki disease. Nature. 1986 Oct 30-Nov 5; 323(6091):814-6. View Abstract
Immunoglobulin M antibodies present in the acute phase of Kawasaki syndrome lyse cultured vascular endothelial cells stimulated by gamma interferon. J Clin Invest. 1986 May; 77(5):1428-35. View Abstract
Analysis of antigen uptake and presentation by Epstein-Barr virus-transformed human lymphoblastoid B cells. Eur J Immunol. 1984 Apr; 14(4):291-8. View Abstract
Mechanisms of the failure of resting B cells to present tetanus toxoid antigen to T cells. J Immunol. 1984 Mar; 132(3):1144-50. View Abstract
Characterization of the mononuclear cell infiltrate in atopic dermatitis using monoclonal antibodies. J Allergy Clin Immunol. 1983 Jan; 71(1 Pt 1):47-56. View Abstract
Macrophage T-cell interaction in man: handling of tetanus toxoid antigen by human monocytes. J Clin Immunol. 1981 Jan; 1(1):21-9. View Abstract
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The specificity of T-cell helper factor in man. J Exp Med. 1977 Jun 01; 145(6):1436-48. View Abstract
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