ABOUT THE RESEARCHER
OVERVIEW
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.
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.
PUBLICATIONS
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- Macabre TH2 skewing in DOCK8 deficiency. J Allergy Clin Immunol. 2021 Mar 02. View abstract
- Multi-kingdom ecological drivers of microbiota assembly in preterm infants. Nature. 2021 Mar; 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 Feb 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. 2020 Dec 13. View abstract
- Successful hematopoietic stem cell transplantation in a 4-1BB deficient patient with EBV-induced lymphoproliferation. Clin Immunol. 2021 Jan; 222:108639. View abstract
- DOCK8 Expression in Regulatory T Cells Maintains their Stability and Limits Contact Hypersensitivity. J Invest Dermatol. 2020 Nov 07. View abstract
- Transferrin receptor 1 is a cellular receptor for human heme-albumin. Commun Biol. 2020 Oct 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 Feb; 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 Feb; 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 Oct; 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
- ITK deficiency presenting as autoimmune lymphoproliferative syndrome. J Allergy Clin Immunol. 2021 Feb; 147(2):743-745.e1. View abstract
- Inherited human IFN-? deficiency underlies mycobacterial disease. J Clin Invest. 2020 06 01; 130(6):3158-3171. 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
- Dysregulated actin dynamics in activated PI3Kd syndrome. Clin Immunol. 2020 01; 210:108311. 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
- Mechanical Skin Injury Promotes Food Anaphylaxis by Driving Intestinal Mast Cell Expansion. Immunity. 2019 05 21; 50(5):1262-1275.e4. 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
- Immunologic reconstitution following hematopoietic stem cell transplantation despite lymph node paucity in NF-?B-inducing kinase deficiency. J Allergy Clin Immunol. 2019 03; 143(3):1240-1243.e4. View abstract
- Hematopoietic Stem Cell Transplantation as Treatment for Patients with DOCK8 Deficiency. J Allergy Clin Immunol Pract. 2019 03; 7(3):848-855. View abstract
- Human primary immunodeficiency caused by expression of a kinase-dead p110d mutant. J Allergy Clin Immunol. 2019 02; 143(2):797-799.e2. View abstract
- Injury, dysbiosis, and filaggrin deficiency drive skin inflammation through keratinocyte IL-1a release. J Allergy Clin Immunol. 2019 04; 143(4):1426-1443.e6. 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
- IL-22 promotes allergic airway inflammation in epicutaneously sensitized mice. J Allergy Clin Immunol. 2019 02; 143(2):619-630.e7. 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
- RORa-expressing T regulatory cells restrain allergic skin inflammation. Sci Immunol. 2018 03 02; 3(21). 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. . 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
- Staphylococcus aureus Exploits Epidermal Barrier Defects in Atopic Dermatitis to Trigger Cytokine Expression. J Invest Dermatol. 2016 11; 136(11):2192-2200. View abstract
- IL-33 promotes food anaphylaxis in epicutaneously sensitized mice by targeting mast cells. J Allergy Clin Immunol. 2016 11; 138(5):1356-1366. 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
- T-cell receptor ligation causes Wiskott-Aldrich syndrome protein degradation and F-actin assembly downregulation. J Allergy Clin Immunol. 2013 Sep; 132(3):648-655.e1. 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
- Epicutaneous sensitization results in IgE-dependent intestinal mast cell expansion and food-induced anaphylaxis. J Allergy Clin Immunol. 2013 Feb; 131(2):451-60.e1-6. 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
- Clinical features and outcome of patients with IRAK-4 and MyD88 deficiency. Medicine (Baltimore). 2010 Nov; 89(6):403-425. 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
- 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
- Impaired T-cell receptor activation in IL-1 receptor-associated kinase-4-deficient patients. J Allergy Clin Immunol. 2010 Aug; 126(2):332-7, 337.e1-2. 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
- Primary immunodeficiencies: 2009 update. J Allergy Clin Immunol. 2009 Dec; 124(6):1161-78. 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
- Common variable immunodeficiency. Pediatr Res. 2009 May; 65(5 Pt 2):13R-19R. View abstract
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