Raif S Geha

Summary

Affiliation: Harvard University
Country: USA

Publications

  1. pmc Primary immunodeficiency diseases: an update from the International Union of Immunological Societies Primary Immunodeficiency Diseases Classification Committee
    Raif S Geha
    Division of Immunology, Children s Hospital, Boston, MA 02115, USA
    J Allergy Clin Immunol 120:776-94. 2007
  2. pmc IL-21R is essential for epicutaneous sensitization and allergic skin inflammation in humans and mice
    Haoli Jin
    Division of Immunology, Children s Hospital and Harvard Medical School, Boston, MA 02115, USA
    J Clin Invest 119:47-60. 2009
  3. ncbi request reprint Impaired signaling via the high-affinity IgE receptor in Wiskott-Aldrich syndrome protein-deficient mast cells
    Vadim I Pivniouk
    Division of Immunology, Children s Hospital, Massachusetts General Hospital, Harvard Medical School, Boston, MA 02115, USA
    Int Immunol 15:1431-40. 2003
  4. pmc Epicutaneous sensitization results in IgE-dependent intestinal mast cell expansion and food-induced anaphylaxis
    Lisa M Bartnikas
    Division of Immunology, Boston Children s Hospital, Boston, Mass 02115, USA
    J Allergy Clin Immunol 131:451-60.e1-6. 2013
  5. pmc Eosinophil-derived leukotriene C4 signals via type 2 cysteinyl leukotriene receptor to promote skin fibrosis in a mouse model of atopic dermatitis
    Michiko K Oyoshi
    Division of Immunology, Children s Hospital, Boston, MA 02115, USA
    Proc Natl Acad Sci U S A 109:4992-7. 2012
  6. pmc The prostaglandin D₂ receptor CRTH2 is important for allergic skin inflammation after epicutaneous antigen challenge
    Rui He
    Division of Immunology, Children s Hospital and Department of Pediatrics, Harvard Medical School, Boston, MA, USA
    J Allergy Clin Immunol 126:784-90. 2010
  7. pmc Primary immunodeficiencies: 2009 update
    Luigi D Notarangelo
    Division of Immunology, Children s Hospital Boston and Department of Pediatrics, Harvard Medical School, Boston, MA 02115, USA
    J Allergy Clin Immunol 124:1161-78. 2009
  8. pmc Cdc42 interacting protein 4 (CIP4) is essential for integrin-dependent T-cell trafficking
    Suresh Koduru
    Division of Immunology, Children s Hospital, Boston, MA 02115, USA
    Proc Natl Acad Sci U S A 107:16252-6. 2010
  9. pmc DOCK8 functions as an adaptor that links TLR-MyD88 signaling to B cell activation
    Haifa H Jabara
    Division of Immunology, Children s Hospital and Department of Pediatrics, Harvard Medical School, Boston, Massachusetts, USA
    Nat Immunol 13:612-20. 2012
  10. pmc Epicutaneous challenge of orally immunized mice redirects antigen-specific gut-homing T cells to the skin
    Michiko K Oyoshi
    Division of Immunology, Children s Hospital, Boston, Massachusetts 02115, USA
    J Clin Invest 121:2210-20. 2011

Detail Information

Publications98

  1. pmc Primary immunodeficiency diseases: an update from the International Union of Immunological Societies Primary Immunodeficiency Diseases Classification Committee
    Raif S Geha
    Division of Immunology, Children s Hospital, Boston, MA 02115, USA
    J Allergy Clin Immunol 120:776-94. 2007
    ..In its last meeting in Jackson Hole, Wyo, after 3 days of intense scientific presentations and discussions, the committee has updated the classification of PID, as reported in this article...
  2. pmc IL-21R is essential for epicutaneous sensitization and allergic skin inflammation in humans and mice
    Haoli Jin
    Division of Immunology, Children s Hospital and Harvard Medical School, Boston, MA 02115, USA
    J Clin Invest 119:47-60. 2009
    ..These results suggest an important role for IL-21R in the mobilization of skin DCs to DLNs and the subsequent allergic response to e.c. introduced antigen...
  3. ncbi request reprint Impaired signaling via the high-affinity IgE receptor in Wiskott-Aldrich syndrome protein-deficient mast cells
    Vadim I Pivniouk
    Division of Immunology, Children s Hospital, Massachusetts General Hospital, Harvard Medical School, Boston, MA 02115, USA
    Int Immunol 15:1431-40. 2003
    ..We conclude that WASP regulates FcepsilonRI-mediated granule exocytosis, cytokine production and cytoskeletal changes in mast cells...
  4. pmc Epicutaneous sensitization results in IgE-dependent intestinal mast cell expansion and food-induced anaphylaxis
    Lisa M Bartnikas
    Division of Immunology, Boston Children s Hospital, Boston, Mass 02115, USA
    J Allergy Clin Immunol 131:451-60.e1-6. 2013
    ..Sensitization to food antigen can occur through cutaneous exposure...
  5. pmc Eosinophil-derived leukotriene C4 signals via type 2 cysteinyl leukotriene receptor to promote skin fibrosis in a mouse model of atopic dermatitis
    Michiko K Oyoshi
    Division of Immunology, Children s Hospital, Boston, MA 02115, USA
    Proc Natl Acad Sci U S A 109:4992-7. 2012
    ..Strategies that block eosinophil infiltration, cys-LT production, or the CysLT(2)R might be useful in the treatment of AD...
  6. pmc The prostaglandin D₂ receptor CRTH2 is important for allergic skin inflammation after epicutaneous antigen challenge
    Rui He
    Division of Immunology, Children s Hospital and Department of Pediatrics, Harvard Medical School, Boston, MA, USA
    J Allergy Clin Immunol 126:784-90. 2010
    ..Chemoattractant receptor-homologous molecule expressed on receptor on T(H)2 cells (CRTH2) mediates chemotaxis to PGD₂ and is expressed on T(H)2 cells and eosinophils, which infiltrate skin lesions in patients with atopic dermatitis...
  7. pmc Primary immunodeficiencies: 2009 update
    Luigi D Notarangelo
    Division of Immunology, Children s Hospital Boston and Department of Pediatrics, Harvard Medical School, Boston, MA 02115, USA
    J Allergy Clin Immunol 124:1161-78. 2009
    ..Careful analysis and prompt recognition of these disorders is essential to prompt effective forms of treatment and thus to improve survival and quality of life in patients affected with PIDs...
  8. pmc Cdc42 interacting protein 4 (CIP4) is essential for integrin-dependent T-cell trafficking
    Suresh Koduru
    Division of Immunology, Children s Hospital, Boston, MA 02115, USA
    Proc Natl Acad Sci U S A 107:16252-6. 2010
    ....
  9. pmc DOCK8 functions as an adaptor that links TLR-MyD88 signaling to B cell activation
    Haifa H Jabara
    Division of Immunology, Children s Hospital and Department of Pediatrics, Harvard Medical School, Boston, Massachusetts, USA
    Nat Immunol 13:612-20. 2012
    ..Thus, DOCK8 functions as an adaptor in a TLR9-MyD88 signaling pathway in B cells...
  10. pmc Epicutaneous challenge of orally immunized mice redirects antigen-specific gut-homing T cells to the skin
    Michiko K Oyoshi
    Division of Immunology, Children s Hospital, Boston, Massachusetts 02115, USA
    J Clin Invest 121:2210-20. 2011
    ..These results indicate that cutaneous exposure to food antigens can reprogram gut-homing effector T cells in LNs to express skin-homing receptors, eliciting skin lesions upon food allergen contact in orally sensitized AD patients...
  11. pmc Transmembrane activator, calcium modulator, and cyclophilin ligand interactor drives plasma cell differentiation in LPS-activated B cells
    Esra Ozcan
    Division of Immunology, Children s Hospital, and the Department of Pediatrics, Harvard Medical School, Boston, MA 02115, USA
    J Allergy Clin Immunol 123:1277-86.e5. 2009
    ..Transmembrane activator, calcium modulator, and cyclophilin ligand interactor (TACI) expression on B cells is upregulated by Toll-like receptor (TLR) 4...
  12. pmc Toll-like receptor 9, transmembrane activator and calcium-modulating cyclophilin ligand interactor, and CD40 synergize in causing B-cell activation
    Esra Ozcan
    Division of Immunology, Children s Hospital, Harvard Medical School, Boston, MA 02115, USA
    J Allergy Clin Immunol 128:601-9.e1-4. 2011
    ..TLR9 and CD40 ligation augment TACI-driven B-cell activation, but only the mechanism of synergy between CD40 and TACI has been explored. Synergy between CD40 and TLR9 in B-cell activation is controversial...
  13. pmc Mechanical injury polarizes skin dendritic cells to elicit a T(H)2 response by inducing cutaneous thymic stromal lymphopoietin expression
    Michiko K Oyoshi
    Division of Immunology, Children s Hospital and Department of Pediatrics, Harvard Medical School, Boston, Mass 02115, USA
    J Allergy Clin Immunol 126:976-84, 984.e1-5. 2010
    ..Atopic dermatitis is characterized by scratching and by T(H)2-dominated immune response to cutaneously introduced antigens. Antigen application to skin mechanically injured by tape stripping results in T(H)2-dominated skin inflammation...
  14. ncbi request reprint Mechanism of recruitment of WASP to the immunological synapse and of its activation following TCR ligation
    Yoji Sasahara
    Division of Immunology, Children s Hospital and Department of Pediatrics, Harvard Medical School, Boston, MA 02115, USA
    Mol Cell 10:1269-81. 2002
    ..These results suggest that the ZAP-70-CrkL-WIP pathway and PKCtheta link TCR to WASP activation...
  15. pmc Transmembrane activator and calcium modulator and cyclophilin ligand interactor enhances CD40-driven plasma cell differentiation
    Emanuela Castigli
    Division of Immunology, Children s Hospital, Boston, MA 02115, USA
    J Allergy Clin Immunol 120:885-91. 2007
    ....
  16. ncbi request reprint WIP deficiency reveals a differential role for WIP and the actin cytoskeleton in T and B cell activation
    Ines M Anton
    Division of Immunology, Children s Hospital and Department of Pediatrics, Harvard Medical School, Boston, MA 02115, USA
    Immunity 16:193-204. 2002
    ..Both WIP-deficient T and B cells show a profound defect in their subcortical actin filament networks. These results suggest that WIP is important for immunologic synapse formation and T cell activation...
  17. pmc Thymic stromal lymphopoietin
    Rui He
    Division of Immunology, Children s Hospital, Harvard Medical School, Boston, Massachusetts 02115, USA
    Ann N Y Acad Sci 1183:13-24. 2010
    ..This role is supported by the finding that TSLP expression is upregulated in keratinocytes of atopic dermatitis skin lesions and in bronchial epithelial cells in asthma...
  18. pmc 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
    Michel J Massaad
    Division of Immunology, Children s Hospital, Boston, MA 02115, USA
    J Allergy Clin Immunol 127:998-1005.e1-2. 2011
    ..The majority of missense mutations in patients with WAS/XLT are located in the WIP-binding domain of WASP and might result in dissociation of the WASP-WIP complex and WASP degradation...
  19. ncbi request reprint TACI is mutant in common variable immunodeficiency and IgA deficiency
    Emanuela Castigli
    Division of Immunology, Children s Hospital and Department of Pediatrics, Harvard Medical School, 300 Longwood Avenue, Boston, Massachusetts 02115, USA
    Nat Genet 37:829-34. 2005
    ..These results suggest that TACI mutations can result in CVID and IgAD...
  20. pmc WIP regulates signaling via the high affinity receptor for immunoglobulin E in mast cells
    Alexander Kettner
    Division of Immunology, Children s Hospital, Harvard Medical School, Boston, MA 02115, USA
    J Exp Med 199:357-68. 2004
    ..These results suggest that WIP regulates FcepsilonRI-mediated mast cell activation by regulating Syk levels and actin cytoskeleton rearrangement...
  21. pmc RIP links TLR4 to Akt and is essential for cell survival in response to LPS stimulation
    Marina S Vivarelli
    Division of Immunology, Children s Hospital and Department of Pediatrics, Harvard Medical School, Boston, MA 02115, USA
    J Exp Med 200:399-404. 2004
    ..These results suggest that RIP is essential for cell survival after TLR4 signaling and links TLR4 to the phosphatidylinositol 3 kinase-Akt pathway...
  22. pmc WIP is critical for T cell responsiveness to IL-2
    Severine Le Bras
    Division of Immunology, Children s Hospital, and Department of Pediatrics, Harvard Medical School, Boston, MA 02115, USA
    Proc Natl Acad Sci U S A 106:7519-24. 2009
    ..These results indicate that WIP is essential for IL-2 signaling and responsiveness in T cells, possibly because of its critical role in TCR-triggered actin cytoskeletal reorganization...
  23. doi request reprint Transmembrane activator and calcium-modulator and cyclophilin ligand interactor mutations in common variable immunodeficiency
    John J Lee
    Division of Immunology, Children s Hospital and Department of Pediatrics, Harvard Medical School, Boston, Massachusetts 02115, USA
    Curr Opin Allergy Clin Immunol 8:520-6. 2008
    ..This review discusses the normal function of TACI and the role of TACI mutants in CVID...
  24. pmc Intronic SH2D1A mutation with impaired SAP expression and agammaglobulinemia
    Mike Recher
    Division of Immunology, Children s Hospital and Department of Pediatrics, Harvard Medical School, Boston, MA, USA
    Clin Immunol 146:84-9. 2013
    ..Despite undetectable serum immunoglobulins, the patient's B cells replicated and differentiated into antibody producing cells normally in vitro...
  25. pmc Enteropathogenic Escherichia coli and vaccinia virus do not require the family of WASP-interacting proteins for pathogen-induced actin assembly
    John J Garber
    Gastrointestinal Unit and Center for the Study of Inflammatory Bowel Disease, Massachusetts General Hospital, Boston, Massachusetts, USA
    Infect Immun 80:4071-7. 2012
    ..Overall, these data highlight similar pathogenic strategies shared by EPEC and vaccinia virus by demonstrating a requirement for both Nck and N-WASP, but not WIP or WIP family members in pathogen-induced actin assembly...
  26. pmc Exaggerated IL-17 response to epicutaneous sensitization mediates airway inflammation in the absence of IL-4 and IL-13
    Rui He
    Division of Immunology, Children s Hospital, and the Department of Pediatrics, Harvard Medical School, Boston, Mass, USA
    J Allergy Clin Immunol 124:761-70.e1. 2009
    ..Atopic dermatitis (AD) is characterized by local and systemic T(H)2 responses to cutaneously introduced allergens and is a risk factor for asthma. Blockade of T(H)2 cytokines has been suggested as therapy for AD...
  27. pmc Impaired T-cell receptor activation in IL-1 receptor-associated kinase-4-deficient patients
    Douglas R McDonald
    Division of Immunology, Children s Hospital, Boston, and the Department of Pediatrics, Harvard Medical School, Boston, Mass, USA
    J Allergy Clin Immunol 126:332-7, 337.e1-2. 2010
    ..The role of IRAK-4 in adaptive immune functions in human subjects is incompletely understood...
  28. ncbi request reprint Induction of activation-induced cytidine deaminase gene expression by IL-4 and CD40 ligation is dependent on STAT6 and NFkappaB
    Fatma Dedeoglu
    Division of Immunology, Children s Hospital and Department of Pediatrics, Harvard Medical School, Harvard Medical School, Boston, MA 02115, USA
    Int Immunol 16:395-404. 2004
    ..These results suggest that signals delivered via CD40 that activate NFkappaB synergize with signals delivered via the IL-4 receptor that activate STAT6 to induce optimal AID gene expression...
  29. pmc The murine equivalent of the A181E TACI mutation associated with common variable immunodeficiency severely impairs B-cell function
    John J Lee
    Division of Immunology, Children s Hospital and Department of Pediatrics, Harvard Medical School, Boston, MA 02115, USA
    Blood 114:2254-62. 2009
    ..These results suggest that mTACI A144E mutation and its human counterpart, A181E, disrupt TACI signaling and impair TACI-dependent B-cell functions...
  30. pmc TRAF2 and TRAF3 independently mediate Ig class switching driven by CD40
    Haifa H Jabara
    Division of Immunology, Children s Hospital, Harvard Medical School, Boston, MA 02115, USA
    Int Immunol 21:477-88. 2009
    ..These results suggest that TRAF2 and TRAF3 can each independently mediate class switch recombination (CSR) driven by CD40, but both are required for optimal CD40-driven isotype switching...
  31. pmc WIP is a chaperone for Wiskott-Aldrich syndrome protein (WASP)
    Miguel A de la Fuente
    Division of Immunology, Children s Hospital Boston, One Blackfan Circle, Boston, MA 02115, USA
    Proc Natl Acad Sci U S A 104:926-31. 2007
    ..These results demonstrate that WIP stabilizes WASP and suggest that it may also be important for its function...
  32. ncbi request reprint WIP and WASP play complementary roles in T cell homing and chemotaxis to SDF-1alpha
    Maria Dolores Gallego
    Division of Immunology, Children s Hospital, 300 Longwood Avenue, Boston, MA 02115, USA
    Int Immunol 18:221-32. 2006
    ..These results suggest that WASP and WIP are not essential for T lymphocyte rolling and adhesion, but play important and partially redundant roles in T cell chemotaxis in vitro and homing in vivo and function downstream of small GTPases...
  33. pmc TACI and BAFF-R mediate isotype switching in B cells
    Emanuela Castigli
    Division of Immunology, Children s Hospital and Department of Pediatrics, Harvard Medical School, Boston, MA 02115, USA
    J Exp Med 201:35-9. 2005
    ..The results obtained indicate that both TACI and BAFF-R are able to transduce signals that result in isotype switching...
  34. ncbi request reprint C4b-binding protein (C4BP) activates B cells through the CD40 receptor
    Scott R Brodeur
    Division of Immunology, Children s Hospital and Department of Pediatrics, Harvard Medical School, Boston, MA, USA
    Immunity 18:837-48. 2003
    ..Furthermore, C4BP colocalized with B cells in the germinal centers of human tonsils. These observations suggest that C4BP is an activating ligand for CD40 and establish a novel interface between complement and B cell activation...
  35. pmc A homozygous mucosa-associated lymphoid tissue 1 (MALT1) mutation in a family with combined immunodeficiency
    Haifa H Jabara
    Division of Immunology, Children s Hospital, Boston, MA 02115, USA
    J Allergy Clin Immunol 132:151-8. 2013
    ..Most cases are sporadic, but autosomal recessive inheritance has been described. In most cases, the cause of CID remains unknown...
  36. pmc Animal models of atopic dermatitis
    Haoli Jin
    Division of Immunology, Department of Medicine, Children s Hospital, Department of Pediatrics, Harvard Medical School, Boston, Massachusetts 02115, USA
    J Invest Dermatol 129:31-40. 2009
    ..These models have resulted in a better understanding of the pathogenesis of AD. This review discusses these models and emphasizes the role of mechanical skin injury and skin barrier dysfunction in eliciting allergic skin inflammation...
  37. ncbi request reprint The regulation of immunoglobulin E class-switch recombination
    Raif S Geha
    Division of Immunology, Children s Hospital and Department of Pediatrics, Harvard Medical School, Boston, Massachusetts 02115, USA
    Nat Rev Immunol 3:721-32. 2003
    ..Both those that are presently in use and potential future therapies to prevent IgE-mediated disease take advantage of our existing knowledge of the specific mechanisms that are required for IgE class switching...
  38. ncbi request reprint B-cell receptor cross-linking delays activation-induced cytidine deaminase induction and inhibits class-switch recombination to IgE
    Haifa H Jabara
    Division of Immunology, Children s Hospital, Boston, MA 02115, USA
    J Allergy Clin Immunol 121:191-196.e2. 2008
    ..During differentiation, B cells receive signals by antigen through the B-cell receptor (BCR) and signals that induce isotype switching...
  39. doi request reprint Common variable immunodeficiency
    Francisco A Bonilla
    Department of Pediatrics, Harvard Medical School, Boston, Massachusetts 02115, USA
    Pediatr Res 65:13R-19R. 2009
    ....
  40. pmc 3BP2 deficiency impairs the response of B cells, but not T cells, to antigen receptor ligation
    Miguel A de la Fuente
    Division of Immunology, Children s Hospital, 300 Longwood Ave, Boston, MA 02115, USA
    Mol Cell Biol 26:5214-25. 2006
    ..These results suggest that 3BP2 is important for BCR, but not for TCR signaling...
  41. ncbi request reprint TACI, isotype switching, CVID and IgAD
    Emanuela Castigli
    Division of Immunology, Children s Hospital, Department of Pediatrics, Harvard Medical School, One Blackfan Circle, Boston, MA 02115, USA
    Immunol Res 38:102-11. 2007
    ..These results suggest that TACI mutations can lead to CVID...
  42. ncbi request reprint Heterozygous N-terminal deletion of IkappaBalpha results in functional nuclear factor kappaB haploinsufficiency, ectodermal dysplasia, and immune deficiency
    Douglas R McDonald
    Division of Immunology, Children s Hospital and Department of Pediatrics, Harvard Medical School, Boston, MA 02115, USA
    J Allergy Clin Immunol 120:900-7. 2007
    ..Mutations in the IkappaB kinase gamma/NF-kappaB essential modifier have been described in male subjects with the syndrome of X-linked ectodermal dysplasia with immune deficiency that results from impaired activation of NF-kappaB...
  43. pmc The mature activating natural killer cell immunologic synapse is formed in distinct stages
    Jordan S Orange
    Department of Molecular and Cellular Biology, 7 Divinity Avenue, Harvard University, Cambridge, MA 02138, USA
    Proc Natl Acad Sci U S A 100:14151-6. 2003
    ..Thus the process of CD2, CD11a, CD11b, and F-actin accumulation in the pSMAC and perforin accumulation in the central SMAC of the NKIS are sequential processes with distinct cytoskeletal requirements...
  44. ncbi request reprint Human nuclear factor kappa B essential modulator mutation can result in immunodeficiency without ectodermal dysplasia
    Jordan S Orange
    Division of Immunology, Children s Hospital of Philadelphia, PA, USA
    J Allergy Clin Immunol 114:650-6. 2004
    ..Male subjects with these mutant NEMO molecules have an X-linked syndrome known as ectodermal dysplasia with immunodeficiency, which is characterized by severe infections, with herpesviruses, bacteria, and mycobacterial susceptibility...
  45. ncbi request reprint Molecular basis of common variable immunodeficiency
    Emanuela Castigli
    Division of Immunology, Children s Hospital, Boston, Massachusetts 02115, USA
    J Allergy Clin Immunol 117:740-6; quiz 747. 2006
    ..These results suggest that TACI mutations can lead to CVID and IgAD...
  46. pmc Deficient natural killer cell cytotoxicity in patients with IKK-gamma/NEMO mutations
    Jordan S Orange
    Division of Immunology, Children s Hospital and Department of Pediatrics, Harvard Medical School, Boston, Massachusetts 02115, USA
    J Clin Invest 109:1501-9. 2002
    ..These data suggest that NEMO participates in signaling pathways leading to NK cell cytotoxicity and that IL-2 can activate NF-kappaB and partially overcome the NK cell defect in patients with NEMO mutations...
  47. pmc Wiskott-Aldrich syndrome protein is required for NK cell cytotoxicity and colocalizes with actin to NK cell-activating immunologic synapses
    Jordan S Orange
    Department of Molecular and Cellular Biology, 7 Divinity Avenue, Harvard University, Cambridge, MA 02138, USA
    Proc Natl Acad Sci U S A 99:11351-6. 2002
    ..Thus, WASp has an important function in NK cells. In patients with WASp mutations, the resulting NK cell defects are likely to contribute to their disease...
  48. ncbi request reprint Role of APRIL in the mucosal Iga antibody response
    Raif S Geha
    Children s Hospital, Boston, Massachusetts, USA
    J Pediatr Gastroenterol Nutr 40:S27. 2005
  49. ncbi request reprint TACI mutation in common variable immunodeficiency and IgA deficiency
    Rima Rachid
    Division of Immunology, Children s Hospital, Boston, 300 Longwood Avenue, Boston, MA 02115, USA
    Curr Allergy Asthma Rep 6:357-62. 2006
    ....
  50. ncbi request reprint Jun N-terminal kinase is essential for CD40-mediated IgE class switching in B cells
    Haifa H Jabara
    Division of Immunology, Children s Hospital, KARP Building 10126, 1 Blackfan Circle, Boston, MA 02115, USA
    J Allergy Clin Immunol 115:856-63. 2005
    ..Both NF-kappaB and p38 are important for CD40-mediated CSR. The role of JNK activation in CD40-mediated isotype switching is unknown...
  51. pmc CCR3 is essential for skin eosinophilia and airway hyperresponsiveness in a murine model of allergic skin inflammation
    Weilie Ma
    Division of Immunology and Department of Pediatrics, Children s Hospital, Boston, Massachusetts 02115, USA
    J Clin Invest 109:621-8. 2002
    ..These results suggest that CCR3 plays an essential role in eosinophil recruitment to the skin and the lung and in the development of AHR...
  52. doi request reprint A girl with severe hand swelling and abdominal cramps
    Arturo Borzutzky
    Division of Immunology, Children s Hospital Boston, Harvard Medical School, Boston, MA 02115, USA
    Clin Chem 54:1733-6; discussion 1736-7. 2008
  53. pmc A 10-aa-long sequence in SLP-76 upstream of the Gads binding site is essential for T cell development and function
    Lalit Kumar
    Division of Immunology, Children s Hospital, CBR Institute for Biomedical Research, Harvard Medical School, Boston, MA 02115, USA
    Proc Natl Acad Sci U S A 102:19063-8. 2005
    ..These results indicate that the Lck binding region of SLP-76 is essential for T cell antigen receptor signaling and normal T cell development and function...
  54. ncbi request reprint 2. Update on primary immunodeficiency diseases
    Francisco A Bonilla
    Division of Immunology, Children s Hospital, Fegan Bldg 6th Floor, 300 Longwood Avenue, Boston, MA 02115, USA
    J Allergy Clin Immunol 117:S435-41. 2006
    ..Clinically defined immunodeficiencies, such as hyper-IgE syndrome and idiopathic CD4 lymphocytopenia, are also discussed. Finally, significant adverse effects in some patients have tempered initial enthusiasm for gene therapy...
  55. pmc Leukotriene B4-driven neutrophil recruitment to the skin is essential for allergic skin inflammation
    Michiko K Oyoshi
    Division of Immunology, Boston Children s Hospital, MA 02115, USA
    Immunity 37:747-58. 2012
    ..Our results demonstrate that a neutrophil-T cell axis reliant on LTB4-BLT1 interaction is required for allergic skin inflammation...
  56. pmc The C104R mutant impairs the function of transmembrane activator and calcium modulator and cyclophilin ligand interactor (TACI) through haploinsufficiency
    John J Lee
    Division of Immunology, Children s Hospital, and the Department of Pediatrics, Harvard Medical School, Boston, MA 02115, USA
    J Allergy Clin Immunol 126:1234-41.e2. 2010
    ..The murine TACI mutant C76R is the equivalent of the human TACI mutant C104R...
  57. pmc 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
    Jana L Mooster
    Division of Immunology, Children s Hospital Boston, Boston, Mass 02115, USA
    J Allergy Clin Immunol 126:127-32.e7. 2010
    ..Mutations in the coding region of the IkappaB kinase gamma/NF-kappaB essential modifier (NEMO) gene cause X-linked ectodermal dysplasia with immunodeficiency...
  58. pmc TSLP acts on infiltrating effector T cells to drive allergic skin inflammation
    Rui He
    Division of Immunology, Children s Hospital, Harvard Medical School, Boston, MA 02115, USA
    Proc Natl Acad Sci U S A 105:11875-80. 2008
    ..These findings suggest that TSLP is essential for antigen driven Th2 cytokine secretion by skin infiltrating effector T cells and could be a therapeutic target in allergic skin inflammation...
  59. pmc Finding NEMO: genetic disorders of NF-[kappa]B activation
    Jordan S Orange
    Division of Immunology, Children s Hospital, Boston, Massachusetts, USA
    J Clin Invest 112:983-5. 2003
    ....
  60. ncbi request reprint Epicutaneous sensitization with superantigen induces allergic skin inflammation
    Dhafer Laouini
    Division of Immunology, Children s Hospital, Harvard Medical School, Boston, Mass 02115, USA
    J Allergy Clin Immunol 112:981-7. 2003
    ..The skin of patients with AD is frequently colonized with enterotoxin-secreting strains of Staphylococcus aureus. Staphylococcal enterotoxins have been implicated in the exacerbations of the inflammatory skin lesions in patients with AD...
  61. ncbi request reprint WIP participates in actin reorganization and ruffle formation induced by PDGF
    Ines M Anton
    Division of Immunology, Children s Hospital, Harvard Medical School, Boston, MA 02115, USA
    J Cell Sci 116:2443-51. 2003
    ..These data suggest a role for WIP in actin reorganization to form PDGF-induced ruffles. This is the first in vivo evidence in mammalian cells for a function of WIP dependent on its ability to bind actin...
  62. ncbi request reprint 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
    Antonella Cianferoni
    Division of Immunology, Children s Hospital, Boston, MA 02115, USA
    J Allergy Clin Immunol 116:1364-71. 2005
    ..The transcription factors nuclear factor-kappaB (NF-kappaB), nuclear factor of activated T cells (NF-AT), and activating protein-1 (AP-1) play a critical role in IL-2 gene expression...
  63. pmc The H1 histamine receptor regulates allergic lung responses
    Paul J Bryce
    Division of Immunology, Children s Hospital, Harvard Medical School, Boston, Massachusetts, USA
    J Clin Invest 116:1624-32. 2006
    ..H1R-/- T cells failed to confer airway inflammation or AHR observed after transfer of WT T cells. Our data establish a role for histamine and H1R in promoting the migration of Th2 cells into sites of allergen exposure...
  64. pmc Erk/Src phosphorylation of cortactin acts as a switch on-switch off mechanism that controls its ability to activate N-WASP
    Narcisa Martinez-Quiles
    Division of Immunology, Children s Hospital, Boston, MA 02115, USA
    Mol Cell Biol 24:5269-80. 2004
    ....
  65. pmc Differential role of SLP-76 domains in T cell development and function
    Lalit Kumar
    Division of Immunology, Children s Hospital, Boston, MA 02115, USA
    Proc Natl Acad Sci U S A 99:884-9. 2002
    ..These results indicate differential roles for SLP-76 domains in T cell development, proliferation and effector functions...
  66. doi request reprint Wiskott-Aldrich syndrome: a comprehensive review
    Michel J Massaad
    Division of Immunology, Boston Children s Hospital, Boston, MA 02115, USA
    Ann N Y Acad Sci 1285:26-43. 2013
    ..The treatment of choice with the best outcome is hematopoietic stem cell transplantation, preferably from a matched related donor...
  67. pmc Ligation of CD46 to CD40 inhibits CD40 signaling in B cells
    Haifa H Jabara
    Division of Immunology, Children s Hospital, Boston, MA 02115, USA
    Int Immunol 23:215-21. 2011
    ..Furthermore, co-ligation of CD46 to CD40 blocked CD40-mediated NF-κB activation. These observations suggest that complement components may play an important role in regulating CD40 activation of B cells and the allergic response...
  68. ncbi request reprint COX-2 inhibition enhances the TH2 immune response to epicutaneous sensitization
    Dhafer Laouini
    Division of Immunology, Children s Hospital, Boston, MA 02115, USA
    J Allergy Clin Immunol 116:390-6. 2005
    ..Mechanical injury to the skin by scratching is an important feature of atopic dermatitis (AD)...
  69. pmc Filaggrin-deficient mice exhibit TH17-dominated skin inflammation and permissiveness to epicutaneous sensitization with protein antigen
    Michiko K Oyoshi
    Division of Immunology, Department of Pediatrics, Harvard Medical School, Children s Hospital, Boston, Mass 02115, USA
    J Allergy Clin Immunol 124:485-93, 493.e1. 2009
    ..Filaggrin is important for skin barrier function and is mutated in 15% to 20% of patients with atopic dermatitis...
  70. pmc The anaphylatoxin C3a downregulates the Th2 response to epicutaneously introduced antigen
    Seiji Kawamoto
    Division of Immunology, Children s Hospital and the Department of Pediatrics, Harvard Medical School, Boston, Massachusetts 02115, USA
    J Clin Invest 114:399-407. 2004
    ..These results suggest that C3a-C3aR interactions inhibit the ability of APCs to drive Th2 cell differentiation in response to epicutaneously introduced antigen and may have important implications for allergic skin diseases...
  71. ncbi request reprint The presentation and natural history of immunodeficiency caused by nuclear factor kappaB essential modulator mutation
    Jordan S Orange
    Department of Immunology, Children s Hospital, 300 Longwood Avenue, Boston, MA 02115, USA
    J Allergy Clin Immunol 113:725-33. 2004
    ..Hypomorphic mutations in the NFkappaB essential modulator (NEMO) impair NFkappaB function and are linked to both immunodeficiency and ectodermal dysplasia (ED), as well as susceptibility to atypical mycobacterial infections...
  72. pmc Impaired IgA class switching in APRIL-deficient mice
    Emanuela Castigli
    Division of Immunology, Children s Hospital, Harvard Medical School, Boston, MA 02115, USA
    Proc Natl Acad Sci U S A 101:3903-8. 2004
    ..APRIL by itself induced IgA as well as IgG1 isotype switching in CD40-deficient IgM(+)IgD(+) sorted B cells. These results suggest that APRIL down-regulates T cell-dependent antibody responses and promotes IgA class switching...
  73. ncbi request reprint CD1d restricted natural killer T cells are not required for allergic skin inflammation
    Abdallah Elkhal
    Division of Immunology, Children s Hospital, Boston MA 02115, USA
    J Allergy Clin Immunol 118:1363-8. 2006
    ..Invariant T-cell receptor-positive natural killer (iNKT) cells have been shown to be essential for the development of allergen-induced airway hyperreactivity (AHR)...
  74. doi request reprint Use of whole exome and genome sequencing in the identification of genetic causes of primary immunodeficiencies
    Janet Chou
    Division of Immunology, Boston Children s Hospital, Boston, Massachusetts 02115, USA
    Curr Opin Allergy Clin Immunol 12:623-8. 2012
    ..This review discusses the strengths and challenges of using whole genome sequencing (WGS)/whole exome sequencing (WES) for identifying novel genetic causes of primary immunodeficiencies...
  75. pmc B cell-intrinsic deficiency of the Wiskott-Aldrich syndrome protein (WASp) causes severe abnormalities of the peripheral B-cell compartment in mice
    Mike Recher
    Children s Hospital, Division of Immunology and Manton Center for Orphan Disease Research, Boston, MA, USA
    Blood 119:2819-28. 2012
    ....
  76. pmc Platelet-associated IgAs and impaired GPVI responses in platelets lacking WIP
    Hervé Falet
    Division of Translational Medicine, Brigham and Women s Hospital, One Blackfan Cir, Karp 6, Boston, MA02115, USA
    Blood 114:4729-37. 2009
    ..Together, the data indicate that platelet-associated IgAs negatively modulate GPVI signaling and function in WIP KO mice...
  77. pmc Vaccinia virus inoculation in sites of allergic skin inflammation elicits a vigorous cutaneous IL-17 response
    Michiko K Oyoshi
    Division of Immunology, Children s Hospital, Harvard Medical School, Boston, MA 02115, USA
    Proc Natl Acad Sci U S A 106:14954-9. 2009
    ..Addition of IL-17 promoted VV replication in skin explants. These results suggest that IL-17 may be a potential therapeutic target in EV...
  78. doi request reprint Primary immune deficiencies with aberrant IgE production
    Esra Ozcan
    Division of Immunology, Children s Hospital and Department of Pediatrics, Harvard Medical School, Boston, MA 02115, USA
    J Allergy Clin Immunol 122:1054-62; quiz 1063-4. 2008
    ..Insight into IgE regulation provided by the study of primary immune deficiencies with elevated IgE has important implications for allergic diseases...
  79. doi request reprint Allogeneic transplantation successfully corrects immune defects, but not susceptibility to colitis, in a patient with nuclear factor-kappaB essential modulator deficiency
    Sung Yun Pai
    Division of Hematology Oncology, Children s Hospital Boston, Boston, MA 02115, USA
    J Allergy Clin Immunol 122:1113-1118.e1. 2008
    ..Boys with X-linked ectodermal dysplasia and immunodeficiency caused by mutations of nuclear factor-kappaB essential modulator have defects in innate and adaptive immunity, and some have colitis...
  80. ncbi request reprint Human disease resulting from gene mutations that interfere with appropriate nuclear factor-kappaB activation
    Jordan S Orange
    Division of Immunology, Children s Hospital of Philadelphia, Department of Pediatrics, University of Pennsylvania School of Medicine, Philadelphia, PA, USA
    Immunol Rev 203:21-37. 2005
    ..A detailed study of these diseases can provide insight into the biochemistry of NF-kappaB activation as well as the role of NF-kappaB in human health...
  81. pmc Epicutaneous antigen exposure induces a Th17 response that drives airway inflammation after inhalation challenge
    Rui He
    Division of Immunology, Children s Hospital and the Department of Pediatrics, Harvard Medical School, Boston, MA 02115, USA
    Proc Natl Acad Sci U S A 104:15817-22. 2007
    ..Our findings suggest that initial cutaneous exposure to antigens in patients with atopic dermatitis may selectively induce the production of IL-17, which, in turn, drives inflammation of their airways...
  82. ncbi request reprint The complement component C3 plays a critical role in both Th1 and Th2 responses to antigen
    Ali Yalcindag
    Division of Immunology, Children s Hospital, Department of Pediatrics, Boston, MA 02115, USA
    J Allergy Clin Immunol 117:1455-61. 2006
    ..Complement component C3 is synthesized by keratinocytes and is activated after skin injury. C3 is also synthesized by peritoneal macrophages, which are activated by the adjuvant alum...
  83. ncbi request reprint Primary immunodeficiency diseases: an update from the International Union of Immunological Societies Primary Immunodeficiency Diseases Classification Committee Meeting in Budapest, 2005
    Luigi Notarangelo
    Department of Pediatrics, University of Brescia Spedali Civili, Italy
    J Allergy Clin Immunol 117:883-96. 2006
    ..As a result of this work, new entities have been included, and the nomenclature of some PIDs (specifically of the various forms of class-switch recombination defects, previously known as hyper-IgM syndromes) has been changed...
  84. ncbi request reprint A second-site mutation in the initiation codon of WAS (WASP) results in expansion of subsets of lymphocytes in an Wiskott-Aldrich syndrome patient
    Wei Du
    Department of Pediatric Oncology, Institute of Development, Aging and Cancer, Tohoku University, Sendai, Japan
    Hum Mutat 27:370-5. 2006
    ..To our knowledge, this is the first report describing somatic mosaicism due to a second-site mutation in the initiation codon of any inherited disorders...
  85. ncbi request reprint Disseminated varicella infection due to the vaccine strain of varicella-zoster virus, in a patient with a novel deficiency in natural killer T cells
    Ofer Levy
    Division Infectious Diseases, Children s Hospital, Harvard Medical School, Boston, Massachusetts 02115, USA
    J Infect Dis 188:948-53. 2003
    ....
  86. pmc Structural requirements of SLP-76 in signaling via the high-affinity immunoglobulin E receptor (Fc epsilon RI) in mast cells
    Alexander Kettner
    Division of Immunology, Children s Hospital and Department of Pediatrics, Harvard Medical School, Boston, Massachusetts 02115, USA
    Mol Cell Biol 23:2395-406. 2003
    ..These results point to important differences in the function of SLP-76 between T cells and mast cells...
  87. ncbi request reprint Mast cells regulate IFN-gamma expression in the skin and circulating IgE levels in allergen-induced skin inflammation
    Harri Alenius
    Division of Immunology, Children s Hospital, Harvard Medical School, Massachusetts General Hospital, 02115, USA
    J Allergy Clin Immunol 109:106-13. 2002
    ..Mast cells are important effector cells in IgE-mediated allergic reactions. They are present in normal skin and increased in skin lesions of patients with atopic dermatitis (AD)...
  88. ncbi request reprint 12. Primary immunodeficiency diseases
    Francisco A Bonilla
    Division of Immunology, Children s Hospital, Enders 809, 300 Longwood Avenue, Boston, MA 02115, USA
    J Allergy Clin Immunol 111:S571-81. 2003
    ..Early diagnosis offers the best opportunity for reduced morbidity and survival and is critical for accurate genetic counseling...
  89. pmc Targeted inactivation of the IL-4 receptor alpha chain I4R motif promotes allergic airway inflammation
    Frank Blaeser
    Deparment of Pediatrics, Washington University School of Medicine, St Louis, MO 63110, USA
    J Exp Med 198:1189-200. 2003
    ..These results define an important role for the I4R motif in regulating allergic inflammation...
  90. ncbi request reprint Cutting edge: the dependence of plasma cells and independence of memory B cells on BAFF and APRIL
    Micah J Benson
    Department of Microbiology and Immunology, Dartmouth Medical School, The Norris Cotton Cancer Center, Lebanon, NH 03756, USA
    J Immunol 180:3655-9. 2008
    ..These data define the BAFF/APRIL-dependent and -independent components of long-lived humoral immunity...
  91. ncbi request reprint Charles A. Janeway and Fred S. Rosen: the discovery of gamma globulin therapy and primary immunodeficiency diseases at Boston Children's Hospital
    Raif S Geha
    Division of Immunology, Children s Hospital, Department of Paediatrics, Harvard Medical School, Boston, MA, USA
    J Allergy Clin Immunol 116:937-40. 2005
  92. pmc Transcellular diapedesis is initiated by invasive podosomes
    Christopher V Carman
    The CBR Institute for Biomedical Research, Department of Pathology, Harvard Medical School, Boston, MA 02115, USA
    Immunity 26:784-97. 2007
    ..These findings provide insights into basic mechanisms for leukocyte trafficking and the functions of podosomes...
  93. pmc Dominant-negative effect of the heterozygous C104R TACI mutation in common variable immunodeficiency (CVID)
    Lilit Garibyan
    Division of Immunology, Children s Hospital Boston, Boston, Massachusetts 02115, USA
    J Clin Invest 117:1550-7. 2007
    ..This work demonstrates that TACI preassembles as an oligomeric complex prior to ligand binding and provides a mechanistic insight into how the heterozygous C104R TACI mutation can potentially lead to CVID...
  94. pmc Fc{epsilon}RI-mediated mast cell degranulation requires calcium-independent microtubule-dependent translocation of granules to the plasma membrane
    Keigo Nishida
    Laboratory for Cytokine Signaling, RIKEN Research Center for Allergy and Immunology, Kanagawa 230 0045, Japan
    J Cell Biol 170:115-26. 2005
    ..Finally, we show that the Fyn/Gab2/RhoA (but not Lyn/SLP-76) signaling pathway plays a critical role in the calcium-independent microtubule-dependent pathway...
  95. ncbi request reprint Characteristics of mycobacterial infection in patients with immunodeficiency and nuclear factor-kappaB essential modulator mutation, with or without ectodermal dysplasia
    Yang Shia Dai
    Department of Dermatology, National Taiwan University Hospital, Taipei, Taiwan
    J Am Acad Dermatol 51:718-22. 2004
    ..We describe clinical and histopathologic characteristics of 5 patients with nuclear factor kappaB essential modulator gene mutations and mycobacterial infections, two of whom had mycobacterial cutaneous infections...
  96. ncbi request reprint Acute chylothorax in children: selective retention of memory T cells and natural killer cells
    Jordan S Orange
    Division of Immunology, Children s Hospital, Enders Building, 300 Longwood Avenue, Boston, MA 02115, USA
    J Pediatr 143:243-9. 2003
    ..To assess for immunodeficiency in patients with hypogammaglobulinemia in the setting of draining acute chylothorax...
  97. ncbi request reprint Allergy and hypersensitivity. Nature versus nurture in allergy and hypersensitivity
    Raif S Geha
    Curr Opin Immunol 15:603-8. 2003
  98. ncbi request reprint Images in immunodeficiency
    William T Shearer
    Department of Pediatrics, Baylor College of Medicine and Texas Children s Hospital, Houston, TX 77030, USA
    J Allergy Clin Immunol 120:982-4. 2007