IL2RG

Summary

Gene Symbol: IL2RG
Description: interleukin 2 receptor subunit gamma
Alias: CD132, CIDX, IL-2RG, IMD4, P64, SCIDX, SCIDX1, cytokine receptor common subunit gamma, CD132 antigen, IL-2 receptor subunit gamma, IL-2R subunit gamma, common cytokine receptor gamma chain, gammaC, interleukin 2 receptor, gamma
Species: human

Top Publications

  1. pmc Human immunodeficiency virus type 1 envelope glycoprotein gp120 produces immune defects in CD4+ T lymphocytes by inhibiting interleukin 2 mRNA
    N Oyaizu
    Department of Pediatrics, North Shore University Hospital Cornell University Medical College, Manhasset, NY 11030
    Proc Natl Acad Sci U S A 87:2379-83. 1990
  2. ncbi The interleukin-2 and interleukin-4 receptors studied by molecular modelling
    P Bamborough
    Oxford Centre for Molecular Science, Physical Chemistry Laboratory, UK
    Structure 2:839-51. 1994
  3. ncbi Isolation of genes differentially expressed between the Yoshida sarcoma and long-survival Yoshida sarcoma variants: origin of Yoshida sarcoma revisited
    H Tsuchiya
    Department of Experimental Pathology, Cancer Institute, Tokyo
    Jpn J Cancer Res 85:1099-104. 1994
  4. ncbi Involvement of the interleukin-2 receptor gamma subunit in interleukin-4-dependent activation of mouse hematopoietic cells and splenic B cells
    S Watanabe
    Department of Molecular and Developmental Biology, University of Tokyo, Japan
    Eur J Immunol 25:126-31. 1995
  5. ncbi Interleukin-2 receptor gamma chain: a functional component of the interleukin-7 receptor
    M Noguchi
    Section on Pulmonary and Molecular Immunology, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD 20892
    Science 262:1877-80. 1993
  6. pmc The human T-cell leukemia/lymphotropic virus type 1 p12I proteins bind the interleukin-2 receptor beta and gammac chains and affects their expression on the cell surface
    J C Mulloy
    Laboratory of Tumor Cell Biology, National Cancer Institute, Bethesda, Maryland 20892, USA
    J Virol 70:3599-605. 1996
  7. ncbi Interleukin-2 receptor beta and gamma chain dysregulation during the inhibition of CD4 T cell activation by human immunodeficiency virus-1 gp120
    L Bani
    Departement d Immunologie, Institut Pasteur, Paris, France
    Eur J Immunol 27:2188-94. 1997
  8. doi Identification and biochemical characterization of human plasma soluble IL-7R: lower concentrations in HIV-1-infected patients
    Thierry Rose
    Institut Pasteur, Unité d Immunogénétique Cellulaire, Paris France
    J Immunol 182:7389-97. 2009
  9. pmc Association of intercellular adhesion molecule 1 with the multichain high-affinity interleukin 2 receptor
    J Burton
    Metabolism Branch, National Cancer Institute, National Institutes of Health, Bethesda, MD 20892
    Proc Natl Acad Sci U S A 87:7329-33. 1990
  10. pmc Identification and cloning of a novel IL-15 binding protein that is structurally related to the alpha chain of the IL-2 receptor
    J G Giri
    Immunex Research and Development Corporation, Seattle, WA 98101, USA
    EMBO J 14:3654-63. 1995

Research Grants

  1. Double Humanization of Liver and Blood in Mice
    ELIZABETH MARIE WILSON; Fiscal Year: 2012
  2. David A Williams; Fiscal Year: 2014
  3. UTPAL P DAVE; Fiscal Year: 2016
  4. Matthew H Porteus; Fiscal Year: 2016
  5. Novel indication for myeloid progenitor use: Induction of tolerance
    Timothy C Fong; Fiscal Year: 2013
  6. Jae U Jung; Fiscal Year: 2016
  7. Gene Correction for XSCID in Hematopoietic Stem Cells
    JONATHAN WALSH; Fiscal Year: 2005
  8. DAVID MICHAEL LANGENAU; Fiscal Year: 2016
  9. BRUCE EDWARD TORBETT; Fiscal Year: 2016
  10. In vivo expansion of human hepatocytes in Fah-/-Rag-/-Il2rg-/- mice
    Markus Grompe; Fiscal Year: 2010

Detail Information

Publications237 found, 100 shown here

  1. pmc Human immunodeficiency virus type 1 envelope glycoprotein gp120 produces immune defects in CD4+ T lymphocytes by inhibiting interleukin 2 mRNA
    N Oyaizu
    Department of Pediatrics, North Shore University Hospital Cornell University Medical College, Manhasset, NY 11030
    Proc Natl Acad Sci U S A 87:2379-83. 1990
    ..Such a mechanism of gp120-induced immunosuppression, if operative in vivo, could contribute to the depressed specific immune responses associated with HIV infection...
  2. ncbi The interleukin-2 and interleukin-4 receptors studied by molecular modelling
    P Bamborough
    Oxford Centre for Molecular Science, Physical Chemistry Laboratory, UK
    Structure 2:839-51. 1994
    ..They are therefore of interest as transmembrane signalling proteins, as well as potential pharmaceutical targets...
  3. ncbi Isolation of genes differentially expressed between the Yoshida sarcoma and long-survival Yoshida sarcoma variants: origin of Yoshida sarcoma revisited
    H Tsuchiya
    Department of Experimental Pathology, Cancer Institute, Tokyo
    Jpn J Cancer Res 85:1099-104. 1994
    ..Thus, our present findings, unexpectedly, raise fundamental questions concerning the cellular origins of YS and LY variants rather than pointing to any specific mechanism to explain the LY phenomenon...
  4. ncbi Involvement of the interleukin-2 receptor gamma subunit in interleukin-4-dependent activation of mouse hematopoietic cells and splenic B cells
    S Watanabe
    Department of Molecular and Developmental Biology, University of Tokyo, Japan
    Eur J Immunol 25:126-31. 1995
    ..The induction of major histocompatibility complex class II molecules or CD23 by IL-4 was virtually unaffected by TUGm2 antibody. These results indicate that IL-2R gamma is differentially involved in various IL-4-dependent reactions...
  5. ncbi Interleukin-2 receptor gamma chain: a functional component of the interleukin-7 receptor
    M Noguchi
    Section on Pulmonary and Molecular Immunology, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD 20892
    Science 262:1877-80. 1993
    ..These findings may help explain the defects of XSCID. Given its role in more than one cytokine receptor system, the common gamma chain (gamma c) is proposed as the designation for IL-2R gamma...
  6. pmc The human T-cell leukemia/lymphotropic virus type 1 p12I proteins bind the interleukin-2 receptor beta and gammac chains and affects their expression on the cell surface
    J C Mulloy
    Laboratory of Tumor Cell Biology, National Cancer Institute, Bethesda, Maryland 20892, USA
    J Virol 70:3599-605. 1996
    ..The interactions of p12I with IL-2R beta and gamma(c) may have important implications in the immunosuppressive effect of HTLV-1 in vivo as well as in the ligand-independent HTLV-1-mediated T-cell proliferation...
  7. ncbi Interleukin-2 receptor beta and gamma chain dysregulation during the inhibition of CD4 T cell activation by human immunodeficiency virus-1 gp120
    L Bani
    Departement d Immunologie, Institut Pasteur, Paris, France
    Eur J Immunol 27:2188-94. 1997
    ..This is the first study of IL-2R beta and IL-2R gamma dysregulation in the context of HIV infection and shows that CD4 is also involved in IL-2R expression...
  8. doi Identification and biochemical characterization of human plasma soluble IL-7R: lower concentrations in HIV-1-infected patients
    Thierry Rose
    Institut Pasteur, Unité d Immunogénétique Cellulaire, Paris France
    J Immunol 182:7389-97. 2009
    ..0014) 2-fold decrease in plasma sIL-7Ralpha levels compared with those in control healthy individuals. Plasma IL-7 and sIL-7Ralpha levels did not show any obvious relationship...
  9. pmc Association of intercellular adhesion molecule 1 with the multichain high-affinity interleukin 2 receptor
    J Burton
    Metabolism Branch, National Cancer Institute, National Institutes of Health, Bethesda, MD 20892
    Proc Natl Acad Sci U S A 87:7329-33. 1990
    ....
  10. pmc Identification and cloning of a novel IL-15 binding protein that is structurally related to the alpha chain of the IL-2 receptor
    J G Giri
    Immunex Research and Development Corporation, Seattle, WA 98101, USA
    EMBO J 14:3654-63. 1995
    ..IL-15R alpha is structurally similar to IL-2R alpha; together they define a new cytokine receptor family. The distribution of IL-15 and IL-15R alpha mRNA suggests that IL-15 may have biological activities distinct from IL-2...
  11. ncbi Interaction of IL-2R beta and gamma c chains with Jak1 and Jak3: implications for XSCID and XCID
    S M Russell
    Laboratory of Molecular Immunology, National Heart, Lung, and Blood Institute, Bethesda, MD 20892
    Science 266:1042-5. 1994
    ..Thus, gamma c mutations in at least some XSCID and XCID patients prevent normal Jak3 activation, suggesting that mutations of Jak3 may result in an XSCID-like phenotype...
  12. ncbi Sharing of the interleukin-2 (IL-2) receptor gamma chain between receptors for IL-2 and IL-4
    M Kondo
    Department of Microbiology, Tohoku University School of Medicine, Sendai, Japan
    Science 262:1874-7. 1993
    ..These results suggest that the IL-2 receptor gamma chain is functionally involved in the IL-4 receptor complex...
  13. ncbi Alternate signalling pathways from the interleukin-2 receptor
    Jonathan M Ellery
    Department of Biosciences, University of Kent at Canterbury, Canterbury, Kent CT2 7NJ, UK
    Cytokine Growth Factor Rev 13:27-40. 2002
    ..This review intends to suggest a role for these differences within the context of the immune system...
  14. ncbi Interleukin-7 receptor expression and activation in nonhaematopoietic neoplastic cell lines
    Larry Cosenza
    Department of Medicine, Division of Hematology and Oncology, New England Medical Center, 750 Washington Street, Boston, MA 02111, USA
    Cell Signal 14:317-25. 2002
    ..The expression of functional IL-7R on epithelial tumour cells may represent a potential target for receptor-directed therapy...
  15. ncbi Human IL-21 and IL-4 bind to partially overlapping epitopes of common gamma-chain
    Jin Li Zhang
    Theodor Boveri Institut für Biowissenschaften, Biozentrum, Physiologische Chemie II, Universitat Wurzburg, Am Hubland, D 97074 Wurzburg, Germany
    Biochem Biophys Res Commun 300:291-6. 2003
    ..These results open the way to understand the molecular recognition mechanism in the IL-21 receptor system and also the promiscuous binding properties of gamma(c)...
  16. pmc Interleukin-2 and its receptor complex (alpha, beta and gamma chains) in in situ and infiltrative human breast cancer: an immunohistochemical comparative study
    Ignacio García-Tuñón
    Department of Cell Biology and Genetics, University of Alcala, Alcala de Henares, Madrid, Spain
    Breast Cancer Res 6:R1-7. 2004
    ....
  17. ncbi Structure of the quaternary complex of interleukin-2 with its alpha, beta, and gammac receptors
    Xinquan Wang
    Howard Hughes Medical Institute, Department of Microbiology and Immunology, Stanford University School of Medicine, 299 Campus Drive, Fairchild D319, Stanford, CA 94305, USA
    Science 310:1159-63. 2005
    ..3 angstroms, the binding of IL-2Ralpha to IL-2 stabilizes a secondary binding site for presentation to IL-2Rbeta. gammac is then recruited to the composite surface formed by the IL-2/IL-2Rbeta complex...
  18. pmc Crystal structure of the IL-2 signaling complex: paradigm for a heterotrimeric cytokine receptor
    Deborah J Stauber
    Department of Molecular Biology, The Scripps Research Institute, 10550 North Torrey Pines Road, La Jolla, CA 92037, USA
    Proc Natl Acad Sci U S A 103:2788-93. 2006
    ..The IL-2/gamma(c) interface itself exhibits the smallest buried surface and the fewest hydrogen bonds in the complex, which is consistent with its promiscuous use in other cytokine receptor complexes...
  19. ncbi Cloning of the gamma chain of the human IL-2 receptor
    T Takeshita
    Department of Microbiology, Tohoku University School of Medicine, Sendai, Japan
    Science 257:379-82. 1992
    ..Thus, the gamma chain is an indispensable component of the functional IL-2R...
  20. ncbi Interleukin-2 receptor gamma chain: a functional component of the interleukin-4 receptor
    S M Russell
    Section on Pulmonary and Molecular Immunology, National Heart, Lung, and Blood Institute, NIH, Bethesda, MD 20892
    Science 262:1880-3. 1993
    ....
  21. ncbi The interleukin-2 receptor gamma chain maps to Xq13.1 and is mutated in X-linked severe combined immunodeficiency, SCIDX1
    J M Puck
    Department of Pediatrics, University of Pennsylvania School of Medicine, Philadelphia 19104
    Hum Mol Genet 2:1099-104. 1993
    ..We considered this gene, IL2RG, a candidate for the X-linked form of severe combined immunodeficiency at the SCIDX1 locus, in which affected males have impaired lymphocyte development...
  22. ncbi Functional interleukin 4 receptor and interleukin 2 receptor common gamma-chain on human non-small cell lung cancers: novel targets for immune therapy
    R Essner
    Department of Molecular Oncology, John Wayne Cancer Institute at Saint John s Health Center, Santa Monica, CA, USA
    J Thorac Cardiovasc Surg 119:10-20. 2000
    ..We examined signal transduction pathways used by the interleukin 4 receptor that may account for growth arrest of the cell line LUst but had no effect on another non-small cell lung cancer cell line, SK-MES-1...
  23. pmc Genome wide transcriptional analysis of resting and IL2 activated human natural killer cells: gene expression signatures indicative of novel molecular signaling pathways
    Karen Dybkaer
    Department of Pathology and Microbiology, University of Nebraska Medical Center, Omaha, NE, USA
    BMC Genomics 8:230. 2007
    ..We utilized genome-wide transcriptional profiling to identify gene expression signatures and pathways in resting and IL2 activated NK cell isolated from peripheral blood of healthy donors...
  24. pmc RNA silencing and HIV: a hypothesis for the etiology of the severe combined immunodeficiency induced by the virus
    Linda B Ludwig
    Retrovirology 5:79. 2008
    ..microRNAs include mRNA for the human interleukin-2 receptor gamma chain (IL-2RG), also called the common gamma (gammac) receptor chain, because it is an integral part of 6 receptors mediating interleukin signalling (IL-2R, IL-4R, IL-..
  25. pmc Liver transcriptome profiles associated with strain-specific Ehrlichia chaffeensis-induced hepatitis in SCID mice
    Koshiro Miura
    The Ohio State University, Department of Veterinary Biosciences, 1925 Coffey Rd, Columbus, OH 43210, USA
    Infect Immun 77:245-54. 2009
    ..Genes downregulated commonly are related to host physiologic functions. The results suggest that marked modulation of host cytokine and chemokine profiles by E. chaffeensis strains underlies the distinct host liver disease...
  26. ncbi Modulation of hematopoiesis in mice with a truncated mutant of the interleukin-2 receptor gamma chain
    K Ohbo
    Department of Microbiology, Tohoku University School of Medicine, Sendai, Japan
    Blood 87:956-67. 1996
    ..These results indicate that functional loss of the gamma chain causes significant effects on the immunological system in mice...
  27. ncbi Inhibitory effects of the Trypanosoma cruzi membrane glycoprotein AGC10 on the expression of IL-2 receptor chains and secretion of cytokines by subpopulations of activated human T lymphocytes
    F Kierszenbaum
    Department of Microbiology, Michigan State University, East Lansing 48824, USA
    Eur J Immunol 29:1684-91. 1999
    ..beta) on the lymphocyte membrane and a marked decrease in the level of up-regulation of the expression of surface CD132 (IL-2R gamma or gamma(c)). These alterations occurred in fairly large proportions of CD4+ and CD8+ lymphocytes...
  28. ncbi Specificity of interleukin-2 receptor gamma chain superfamily cytokines is mediated by insulin receptor substrate-dependent pathway
    Hui Xiao
    Department of Pharmacology and Cancer Center, School of Medicine, Case Western Reserve University, Cleveland, Ohio 44106 4965, USA
    J Biol Chem 277:8091-8. 2002
    ..These data suggest that IRS-dependent signaling pathways work by recruiting different signaling molecules to determine specificity of IL-2R gamma superfamily cytokines...
  29. pmc Development of functional human blood and immune systems in NOD/SCID/IL2 receptor {gamma} chain(null) mice
    Fumihiko Ishikawa
    Department of Cancer Immunology and AIDS, Dana Farber Cancer Institute, 44 Binney St, no 770, Boston, MA 02115, USA
    Blood 106:1565-73. 2005
    ..combined immunodeficient (NOD/SCID) mouse line harboring a complete null mutation of the common cytokine receptor gamma chain (NOD/SCID/interleukin 2 receptor [IL2r] gamma(null)) efficiently supports development of functional ..
  30. pmc Lymphohaematopoietic abnormalities and systemic lymphoproliferative disorder in interleukin-2 receptor gamma chain-deficient mice
    M Ikebe
    Second Department of Pathology, Kumamoto University School of Medicine, Japan
    Int J Exp Pathol 78:133-48. 1997
    ..These pathological abnormalities suggest that the gamma chain is implicated not only in lymphoid and haematopoietic development but also in thymic epithelial cell ontogeny...
  31. ncbi Interleukin 2 receptor gamma chain (gamma(c)) knockout mice show less regularity in estrous cycle but achieve normal pregnancy without fetal compromise
    Satomi Miyazaki
    Department of Obstetrics and Gynecology, Toyama Medical and Pharmaceutical University, Japan
    Am J Reprod Immunol 47:222-30. 2002
    ....
  32. pmc Regulation of the interleukin-7 receptor alpha promoter by the Ets transcription factors PU.1 and GA-binding protein in developing B cells
    Rodney P DeKoter
    Department of Molecular Genetics, Biochemistry, and Microbiology, University of Cincinnati College of Medicine, Cincinnati, OH 45267, USA
    J Biol Chem 282:14194-204. 2007
    ..The receptor for IL-7 is heterodimeric, consisting of a common gamma chain (gammac, encoded by Il2rg) and an alpha subunit (IL-7Ralpha, encoded by Il7r)...
  33. pmc Dual mechanism of impairment of interleukin-7 (IL-7) responses in human immunodeficiency virus infection: decreased IL-7 binding and abnormal activation of the JAK/STAT5 pathway
    Olivier Juffroy
    Institut Pasteur, Unité d Immunogénétique Cellulaire, 75724 Paris Cedex 15, France
    J Virol 84:96-108. 2010
    ..IL-7 receptor (IL-7R) chains, with decreases in IL-7Ralpha/CD127 expression in the memory subset and increases in gammac/CD132 expression in all CD4(+) T cells...
  34. ncbi The murine interleukin-2 receptor gamma chain gene: organization, chromosomal localization and expression in the adult thymus
    J P DiSanto
    INSERM U132, Hopital Necker Enfants Malades, Institut Pasteur, Paris, France
    Eur J Immunol 24:3014-8. 1994
    ..receptor gamma (IL-2R gamma) chain in the man result in an X-linked severe combined immunodeficiency, SCIDX1, characterized by an absence of T-cell differentiation...
  35. pmc Development of a quantitative bead capture assay for soluble IL-7 receptor alpha in human plasma
    Sylvie Faucher
    National HIV and Retrovirology Laboratories, Public Health Agency of Canada, Ottawa, Canada
    PLoS ONE 4:e6690. 2009
    ..It binds to the IL-7R receptor, a complex of the IL-7Ralpha (CD127) and common gamma (CD132) chains...
  36. ncbi Cloning of a receptor subunit required for signaling by thymic stromal lymphopoietin
    A Pandey
    Whitehead Institute for Biomedical Research, Cambridge, MA 02142, USA
    Nat Immunol 1:59-64. 2000
    ..we report the cloning of a type I cytokine receptor subunit that is most closely related to the common cytokine receptor gamma chain (gamma c)...
  37. ncbi IL-7 receptor is present on human microvascular endothelial cells
    Danuta Dus
    Laboratory of Cellular Interactions, Ludwik Hirszfeld Institute of Immunology and Experimental Therapy, Polish Academy of Sciences, Centre of Excellence IMMUNE, R Weigla 12, 53 114, Wroclaw, Poland
    Immunol Lett 86:163-8. 2003
    ..The receptor consists of two chains: IL-7 specific alpha chain (CD127) and the common gamma(c) chain (CD132) which is a component of several other cytokine receptors: IL-2, IL-4, IL-9 and IL-15...
  38. doi Disengaging the IL-2 receptor with daclizumab enhances IL-7-mediated proliferation of CD4(+) and CD8(+) T cells
    P Monti
    Immunology of Diabetes Unit and Clinical Transplant Unit, San Raffaele Scientific Institute, Milan, Italy
    Am J Transplant 9:2727-35. 2009
    ..of IL-2, which expands T cells by linking the IL-2 receptor (IL-2R) composed of three subunits: CD25, CD122 and CD132. Daclizumab, widely used in immunosuppression, is a humanized anti-CD25 antibody that disrupts IL-2 signaling by ..
  39. ncbi A PCR-based non-radioactive X-chromosome inactivation assay for genetic counseling in X-linked primary immunodeficiencies
    G S Wengler
    Department of Pediatrics, University of Brescia, Italy
    Life Sci 61:1405-11. 1997
    The Wiskott-Aldrich syndrome (WAS), X-linked severe combined immunodeficiency (SCIDX1), and X-linked agammaglobulinemia (XLA) are severe congenital immunodeficiencies with X-linked inheritance...
  40. ncbi Carrier and prenatal diagnosis of X-linked severe combined immunodeficiency: mutation detection methods and utilization
    J M Puck
    Laboratory for Gene Transfer, National Human Genome Research Institute, National Institutes of Health, Bethesda, MD 20892 4442, USA
    Hum Genet 99:628-33. 1997
    b>IL2RG, the gene encoding the common gamma chain, gamma c, of the receptor for interleukin-2 and other cytokines, has been identified as the disease gene for severe combined immunodeficiency (SCID) of the X-linked type...
  41. ncbi Cloning and expression of the murine homologue of a putative human X-linked nuclear protein gene closely linked to PGK1 in Xq13.3
    J Gecz
    INSERM U242, Marseille, France
    Hum Mol Genet 3:39-44. 1994
    ..Genes involved in the phenotypes have been isolated for only two of them (Menkes and SCIDX). It was therefore interesting to isolate and characterize new genes from the region...
  42. doi IL-7 receptor is expressed on adult pre-B-cell acute lymphoblastic leukemia and other B-cell derived neoplasms and correlates with expression of proliferation and survival markers
    Sarah C Sasson
    National Centre in HIV Epidemiology and Clinical Research, University of New South Wales, Sydney, Australia
    Cytokine 50:58-68. 2010
    ..Surface expression of IL-7R components CD127 and CD132, and intracellular Ki-67 and Bcl-2 were examined by flow cytometry on peripheral blood or bone marrow mononuclear ..
  43. pmc A role for thymic stromal lymphopoietin in CD4(+) T cell development
    Amin Al-Shami
    Laboratory of Molecular Immunology, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD 20892, USA
    J Exp Med 200:159-68. 2004
    ..receptor alpha chain and a distinctive subunit, TSLP receptor (TSLPR), which is most related to the common cytokine receptor gamma chain, gamma(c)...
  44. ncbi Inherited disorders of cytokines
    Capucine Picard
    Unité d Immunologie et d Hématologie Pédiatriques, Hopital Necker Enfants Malades, and Laboratoire de Génétique Humaine des Maladies Infectieuses, Université de Paris René Descartes INSERM U550, Faculte de Medecine Necker, Paris, France
    Curr Opin Pediatr 16:648-58. 2004
    ..Cytokines are soluble mediators involved in the development or function of the immune system. This paper reviews the literature on childhood-onset inherited disorders associated with impaired cytokine-mediated immunity...
  45. ncbi Loss of IL-7 receptor alpha on CD4+ T cells defines terminally differentiated B cell-helping effector T cells in a B cell-rich lymphoid tissue
    Hyung W Lim
    Laboratory of Immunology and Hematopoiesis, Department of Comparative Pathobiology, Purdue Cancer Center, Purdue University, West Lafayette, IN 47907, USA
    J Immunol 179:7448-56. 2007
    ..IL-7 uses a receptor composed of IL-7Ralpha (CD127) and the common gamma-chain (CD132) to transmit its signal...
  46. doi Interleukin-4 downregulates CD127 expression and activity on human thymocytes and mature CD8+ T cells
    Angela M Crawley
    Ottawa Hospital Research Institute, Ottawa, Ontario, Canada
    Eur J Immunol 40:1396-407. 2010
    ..g. HIV, HCV) and cancer. Many IL-2Rgamma-sharing (gammaC) cytokines decrease CD127 expression on CD4+ and CD8+ T cells in mice (IL-2, IL-4, IL-7, IL-15) and in humans (IL-..
  47. ncbi Down-regulation of human B lymphocyte activities by a Trypanosoma cruzi membrane glycoprotein
    F Kierszenbaum
    Department of Microbiology and Molecular Genetics, Michigan State University, East Lansing, MI 48824, USA
    Int J Parasitol 31:265-71. 2001
    ..This alteration was accompanied by others such as decreased expression of the CD122 and CD132 chains of the IL-2R complex...
  48. pmc Complementary signaling through flt3 and interleukin-7 receptor alpha is indispensable for fetal and adult B cell genesis
    Ewa Sitnicka
    Hematopoietic Stem Cell Laboratory, Lund Strategic Research Center for Stem Cell Biology and Cell Therapy, Lund University Hospital, BMC 10, Klinikgatan 26, 221 84 Lund, Sweden
    J Exp Med 198:1495-506. 2003
    ..cells and Igs are sustained at normal levels throughout life of mice deficient in IL-7, IL-7Ralpha, common cytokine receptor gamma chain, or flt3 ligand (FL), we report here that adult mice double deficient in IL-7Ralpha and FL ..
  49. ncbi Molecular mechanisms involved in interleukin-4-induced human neutrophils: expression and regulation of suppressor of cytokine signaling
    Claude Ratthé
    INRS Institut Armand Frappier, Universite du Quebec, 245 Boul Hymus, Pointe Claire PQ, Canada, H9R 1G6
    J Leukoc Biol 81:1287-96. 2007
    Interleukin-4 (IL-4) is a CD132-dependent cytokine known to activate the Jak-STAT pathway in different cells and cell lines...
  50. doi Human bone marrow mesenchymal stem cells and chondrocytes promote and/or suppress the in vitro proliferation of lymphocytes stimulated by interleukins 2, 7 and 15
    C Bocelli-Tyndall
    Institute of Surgical Research and Hospital Management and Department of Biomedicine, University Hospital Basel, Basel, Switzerland
    Ann Rheum Dis 68:1352-9. 2009
    ....
  51. ncbi Cloning of rat thymic stromal lymphopoietin receptor (TSLPR) and characterization of genomic structure of murine Tslpr gene
    Blagoy Blagoev
    Protein Interaction Laboratory, Center for Experimental Bioinformatics, University of Southern Denmark, Campusvej 55, M, DK 5230, Odense, Denmark
    Gene 284:161-8. 2002
    ..it shares a common genomic organization of coding exons with the murine IL-2 receptor common gamma chain (Il2rg)...
  52. ncbi A novel immunodeficient mouse model--RAG2 x common cytokine receptor gamma chain double mutants--requiring exogenous cytokine administration for human hematopoietic stem cell engraftment
    F Mazurier
    Laboratoire de Pathologie Moléculaire et Thérapie Génique, UMR CNRS 5540, Fédération de Recherche 60 Biologie des Greffes, Universite Victor Segalen, Bordeaux, France
    J Interferon Cytokine Res 19:533-41. 1999
    ..developed a new immunodeficient mouse model by combining recombinase activating gene-2 (RAG2) and common cytokine receptor gamma chain (gamma c) mutations...
  53. ncbi The common gamma chain (gamma c) is a required signaling component of the IL-21 receptor and supports IL-21-induced cell proliferation via JAK3
    Tania Habib
    Division of Hematology, University of Washington School of Medicine, Seattle, Washington 98195, USA
    Biochemistry 41:8725-31. 2002
    The common cytokine receptor gamma chain (gamma c), an essential component of the receptors for IL-2, IL-4, IL-7, IL-9, and IL-15, is critical for the development and function of lymphocytes...
  54. ncbi Effect of factor VIII concentrate on leucocyte cytokine receptor expression in vitro: relevance to inhibitor formation and tolerance induction
    G Hodge
    Haematology Oncology Department, Women s and Children s Hospital, North Adelaide, South Australia, Australia
    Haemophilia 12:133-9. 2006
    ..Inhibition of CD132 in the presence of pdFVIII may result in immune tolerance in some recipients of pdFVIII...
  55. ncbi The molecular basis of X-linked severe combined immunodeficiency: the role of the interleukin-2 receptor gamma chain as a common gamma chain, gamma c
    W J Leonard
    Section on Pulmonary and Molecular Immunology, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD 20892
    Immunol Rev 138:61-86. 1994
    ....
  56. ncbi Gnotobiotics and immunopathology: the use of the gnotobiotic environment to study acquired and inherited immunodeficiency diseases
    Steven Krakowka
    Department of Veterinary Biosciences, College of Veterinary Medicine, The Ohio State University, Columbus, OH 43210, USA
    Vet Immunol Immunopathol 108:165-75. 2005
    ..This fatal inherited defect is caused by mutation in the common gamma chain (IL2RG) gene and renders affected animals profoundly immunodeficient...
  57. doi Immunologic analysis of HIV-uninfected Taiwanese children with BCG-induced disease
    Wen I Lee
    Primary Immunodeficiency Care and Research Institute, Chang Gung Memory Hospital, College of Medicine, Chang Gung University, Taoyuan, Taiwan
    J Clin Immunol 29:319-29. 2009
    ..The aim of this study was to evaluate immunity in HIV-uninfected children with bacille Calmette-Guerin-induced disease (BCG-ID) over an 8-year period, with particular emphasis on underlying diseases...
  58. ncbi Defective IL7R expression in T(-)B(+)NK(+) severe combined immunodeficiency
    A Puel
    Laboratory of Molecular Immunology, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Maryland 20892 1674, USA
    Nat Genet 20:394-7. 1998
    ..4), which encodes the common cytokine receptor gamma chain (gamma(c)) that is shared by the IL-2, IL-4, IL-7, IL-9 and IL-15 receptors...
  59. ncbi Restoration of lymphoid populations in a murine model of X-linked severe combined immunodeficiency by a gene-therapy approach
    M Lo
    Laboratory of Molecular Immunology, National Heart, Lung, and Blood Institute, National Institutes of Health NIH, Bethesda, MD 20892 1674, USA
    Blood 94:3027-36. 1999
    ..This disease results from mutations in the IL2RG gene, which encodes the common cytokine receptor gamma chain, gamma(c). Previously, we generated gamma(c)-deficient mice as a murine model of XSCID...
  60. pmc Correction of canine X-linked severe combined immunodeficiency by in vivo retroviral gene therapy
    Suk See Ting-De Ravin
    Laboratory of Host Defenses, NIAID, NIH, Building 10 CRC, Room 5 3750, 10 Center Drive, Bethesda, MD 20892 1456, USA
    Blood 107:3091-7. 2006
    ..This is the first demonstration that in vivo gene therapy targeting HSCs can restore both cellular and humoral immunity in a large-animal model of a fatal immunodeficiency...
  61. doi Mutations causing severe combined immunodeficiency: detection with a custom resequencing microarray
    Tonya Lebet
    Department of Pediatrics, University of California San Francisco School of Medicine, San Francisco, California 94143 0519, USA
    Genet Med 10:575-85. 2008
    ..A resequencing microarray could facilitate mutation detection, increasing the chance of diagnosing infants early for optimal rescue by hematopoietic stem cell transplantation...
  62. doi Clinical and immunologic consequences of a somatic reversion in a patient with X-linked severe combined immunodeficiency
    Carsten Speckmann
    Center for Paediatrics and Adolescent Medicine, University of Freiburg, Freiburg, Germany
    Blood 112:4090-7. 2008
    ..is a life-threatening disorder caused by mutations in the gene encoding the interleukin-2 receptor gamma chain (IL2RG)...
  63. pmc Short stature in partially corrected X-linked severe combined immunodeficiency--suboptimal response to growth hormone
    Suk See De Ravin
    Laboratory of Host Defenses, National Institute of Allergy and Infectious Diseases NIAID, Bethesda, MD 20892, USA
    J Pediatr Endocrinol Metab 21:1057-63. 2008
    X-linked severe combined immunodeficiency (XSCID) results from defects in the common cytokine receptor gamma chain (gamma c) required for signaling by receptors for interleukin (IL)-2, -4, -7, -9, -15, and -21...
  64. ncbi Analysis of interleukin receptor gene expression in mouse fetal liver by quantitative polymerase chain reaction
    G Q Jia
    Center for Blood Research, Inc, Harvard Medical School, Boston, MA 02115, USA
    Eur J Immunol 25:2096-100. 1995
    ..Their expression seems to precede that of the genes encoding the specific subunits of these interleukin receptor systems...
  65. ncbi Cloning and sequencing of the cDNA encoding a mouse IL-2 receptor gamma
    N Kobayashi
    Institute for Molecular and Cellular Biology, Osaka University, Japan
    Gene 130:303-4. 1993
    ..The primary amino acid sequence deduced from the nucleotide sequence exhibits 70% overall similarity with human IL-2R gamma and, in particular, the predicted cytoplasmic region shows a higher degree of similarity (about 84.7%)...
  66. ncbi Cloning of the mouse interleukin 2 receptor gamma chain: demonstration of functional differences between the mouse and human receptors
    S Kumaki
    Department of Microbiology, Tohoku University School of Medicine, Sendai, Japan
    Biochem Biophys Res Commun 193:356-63. 1993
    ..These results indicate that the mouse functional IL-2 receptor is a complex comprising three distinct subunits, alpha, beta and gamma chains, but the beta gamma heterodimer is not functional and different from the human heterodimer...
  67. ncbi Signalling by cytokines interacting with the interleukin-2 receptor gamma chain
    J B Demoulin
    Ludwig Institute for Cancer Research, Brussels, Belgium
    Cytokines Cell Mol Ther 4:243-56. 1998
    The interleukin-2 (IL-2) receptor gamma chain (gammac) is shared by receptor complexes used by IL-2, IL-4, IL-7, IL-9 and IL-15, all of which are cytokines involved in lymphocyte development and/or activation...
  68. pmc Developmental dissociation of thymic dendritic cell and thymocyte lineages revealed in growth factor receptor mutant mice
    H R Rodewald
    Basel Institute for Immunology, Grenzacherstrasse 487, CH 4005 Basel, Switzerland
    Proc Natl Acad Sci U S A 96:15068-73. 1999
    ..Mice deficient for two growth factor receptor molecules [c-kit and the common cytokine receptor gamma chain (gamma(c))] lack all thymocytes including T cell progenitors...
  69. pmc Cloning of a type I cytokine receptor most related to the IL-2 receptor beta chain
    K Ozaki
    Laboratory of Molecular Immunology, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD 20892 1674, USA
    Proc Natl Acad Sci U S A 97:11439-44. 2000
    ..In these latter cells, heterodimerization of IL-2Rbeta and the common cytokine receptor gamma chain (gamma(c)) cytoplasmic domains allows potent proliferation, whereas such heterodimerization of NILR ..
  70. ncbi Advances in the understanding and treatment of human severe combined immunodeficiency
    R H Buckley
    Department of Pediatrics, Duke University Medical Center, Durham, NC 27710, USA
    Immunol Res 22:237-51. 2000
    ..in any one of at least seven different genes, including those for adenosine deaminase, the common cytokine receptor gamma chain, Janus kinase 3, IL-7 receptor alpha chain, recombinase activation genes 1 and 2, and CD45...
  71. pmc Synergy of IL-21 and IL-15 in regulating CD8+ T cell expansion and function
    Rong Zeng
    Laboratory of Molecular Immunology, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD 20892, USA
    J Exp Med 201:139-48. 2005
    Interleukin (IL)-21 is the most recently recognized of the cytokines that share the common cytokine receptor gamma chain (gamma(c)), which is mutated in humans with X-linked severe combined immunodeficiency...
  72. ncbi Gene therapy: therapeutic gene causing lymphoma
    Niels Bjarne Woods
    Laboratory of Genetics, The Salk Institute for Biological Studies, La Jolla, California 92037, USA
    Nature 440:1123. 2006
    ..Using a mouse model for gene therapy of X-SCID, we find that the corrective therapeutic gene IL2RG itself can act as a contributor to the genesis of T-cell lymphomas, with one-third of animals being affected...
  73. ncbi Ectopic retroviral expression of LMO2, but not IL2Rgamma, blocks human T-cell development from CD34+ cells: implications for leukemogenesis in gene therapy
    K Pike-Overzet
    Department of Immunology, Erasmus University Medical Center, Rotterdam, The Netherlands
    Leukemia 21:754-63. 2007
    ..Instead, restoration of normal IL7-receptor signaling may allow progression of T-cell development to stages where ectopic LMO2 expression causes aberrant thymocyte growth...
  74. doi Human acute leukemia cells injected in NOD/LtSz-scid/IL-2Rgamma null mice generate a faster and more efficient disease compared to other NOD/scid-related strains
    Alice Agliano
    Division of Hematology Oncology, Department of Medicine, European Institute of Oncology, Milan, Italy
    Int J Cancer 123:2222-7. 2008
    ..Cg-Prkdc(scid)B2m(tm1Unc)/J, abbreviated NOD/scid/beta2 null, NSB; and NOD.Cg-Prkdc(scid)Il2rg(tm1Wjll)/SzJ, abbreviated NOD/scid/IL-2Rgamma null, NSG)...
  75. doi Establishment of a humanized model of liver using NOD/Shi-scid IL2Rgnull mice
    Hiroshi Suemizu
    Biomedical Research Department, Central Institute for Experimental Animals, 1430 Nogawa, Miyamae, Kawasaki, Kanagawa, Japan
    Biochem Biophys Res Commun 377:248-52. 2008
    Severely immunodeficient NOD/Shi-scid IL2Rg(null) (NOG) mice are used as recipients for human tissue transplantation, which produces chimeric mice with various types of human tissue...
  76. ncbi Customised molecular diagnosis of primary immune deficiency disorders in New Zealand: an efficient strategy for a small developed country
    Rohan Ameratunga
    LabPlus, Auckland Hospital, Park Rd, Grafton, Auckland, New Zealand
    N Z Med J 122:46-53. 2009
    ..Timely diagnosis can be life-saving in children with PIDs such as severe combined immune deficiency...
  77. doi Transcriptional expression profile of cultured human embryonic stem cells in vitro and in vivo
    Marlen Keil
    Max Delbruck Center for Molecular Medicine, Berlin Buch, Robert Rossle Str 10, 13125, Berlin, Germany
    In Vitro Cell Dev Biol Anim 48:165-74. 2012
    ..SA002 cells that showed spontaneous partial differentiation in vitro had a low teratoma formation capacity in vivo. Cells that were partially differentiated led to slower growing teratomas with more uniform histology...
  78. pmc Human responses against HER-2-positive cancer cells in human immune system-engrafted mice
    C De Giovanni
    Department of Experimental Pathology, Cancer Research Section, Alma Mater Studiorum Universita di Bologna, Italy
    Br J Cancer 107:1302-9. 2012
    ..Here, we used HIS mice to study human immune responses against human HER-2-positive cancer cells and their ability to control tumour growth and metastasis...
  79. pmc Application of IL-2 therapy to target T regulatory cell function
    Ethan M Shevach
    Laboratory of Immunology, National Institutes of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD 20892, USA
    Trends Immunol 33:626-32. 2012
    ..Regulatory T cells (Treg cells) express all three chains (CD25, CD122, and CD132) of the IL-2 receptor complex and are dependent on IL-2 for survival and function...
  80. pmc A potential novel spontaneous preterm birth gene, AR, identified by linkage and association analysis of X chromosomal markers
    Minna K Karjalainen
    Department of Pediatrics, Institute of Clinical Medicine, University of Oulu, Oulu, Finland
    PLoS ONE 7:e51378. 2012
    ..candidate genes, those encoding the androgen receptor (AR) and the interleukin-2 receptor gamma subunit (IL2RG), located near this locus were analyzed as candidates for SPTB in subsequent case-control association analyses...
  81. doi Potent in vitro and in vivo activity of sorafenib in multiple myeloma: induction of cell death, CD138-downregulation and inhibition of migration through actin depolymerization
    Josefina Udi
    Department of Haematology and Oncology, Freiburg University Medical Centre, Freiburg, Germany
    Br J Haematol 161:104-16. 2013
    ....
  82. pmc Interleukin-7 is produced by afferent lymphatic vessels and supports lymphatic drainage
    Maria Iolyeva
    Institute of Pharmaceutical Sciences, Swiss Federal Institute of Technology, ETH Zurich, Zurich, Switzerland
    Blood 122:2271-81. 2013
    ..Here, we report that LECs not only produce IL-7, but also express the IL-7 receptor chains IL-7Rα and CD132. Stimulation with recombinant IL-7 enhanced LEC in vitro activity and induced lymphangiogenesis in the cornea of ..
  83. doi Uterine natural killer cells pace early development of mouse decidua basalis
    A P Hofmann
    Department of Biomedical and Molecular Sciences, Queen s University, Kingston, ON, Canada K7L3N6
    Mol Hum Reprod 20:66-76. 2014
    ..5-9.5 implant sites from allogeneically mated alymphoid or normal BALB/c mice (Rag2(-/-)Il2rg(-/-); NK-T-B- versus +/+) by whole mount immunohistochemistry revealed delays in Rag2(-/-)Il2rg(-/-) uterine lumen ..
  84. doi Generation of a patient-derived chordoma xenograft and characterization of the phosphoproteome in a recurrent chordoma
    Jason M Davies
    Department of Neurological Surgery
    J Neurosurg 120:331-6. 2014
    ..The authors sought to establish and characterize a primary xenograft model for chordoma that faithfully recapitulates the molecular features of human chordoma...
  85. pmc TALENs facilitate targeted genome editing in human cells with high specificity and low cytotoxicity
    Claudio Mussolino
    Institute for Cell and Gene Therapy, University Medical Center Freiburg, 79106 Freiburg, Germany Center for Chronic Immunodeficiency, University Medical Center Freiburg, 79108 Freiburg, Germany Institute of Experimental Hematology, Hannover Medical School, 30625 Hannover, Germany
    Nucleic Acids Res 42:6762-73. 2014
    ..Here, we have engineered TALEN pairs targeting three human loci (CCR5, AAVS1 and IL2RG) and performed a detailed analysis of their activity, toxicity and specificity...
  86. pmc Microphysical space of a liver sinusoid device enables simplified long-term maintenance of chimeric mouse-expanded human hepatocytes
    Steven P Maher
    Bioengineering Center at USF, Charles Stark Draper Laboratory, 3802 Spectrum Blvd ste 201, Tampa, FL, 33612, USA
    Biomed Microdevices 16:727-36. 2014
    ..By sourcing human hepatocytes expanded in the Fah, Rag2, and Il2rg-knockout (FRG™-KO) humanized mouse model, utilizing a few hundred hepatocytes within each channel, and ..
  87. doi A novel intronic splice site deletion of the IL-2 receptor common gamma chain results in expression of a dysfunctional protein and T-cell-positive X-linked Severe combined immunodeficiency
    P E A Gray
    Immunology and Infectious Diseases, Sydney Children s Hospital, Sydney, NSW, Australia School of Women s and Children s Health, University of New South Wales, Sydney, NSW, Australia
    Int J Immunogenet 42:11-4. 2015
    ..pair intronic deletion close to the canonical splice site of exon 5, which resulted in a variety of truncated IL2RG mRNA species...
  88. doi CD8-positive lymphocytes in graft-versus-host disease of humanized NOD.Cg-Prkdc(scid)Il2rg(tm1Wjl)/SzJ mice
    S T Laing
    Comparative Pathology Laboratory, University of California at Davis, Davis, CA, USA
    J Comp Pathol 152:238-42. 2015
    ..NSG-hu-BLT mice represent an excellent model of GvHD, but its prevalence may compromise their use in other fields of biomedical research. ..
  89. pmc The status of donor cancer tissues affects the fate of patient-derived colorectal cancer xenografts in NOG mice
    Etsuko Fujii
    Research Division, Chugai Pharmaceutical Co, Ltd, 200 Kajiwara, Kamakura, Kanagawa 247 8530, Japan
    Exp Anim 64:181-90. 2015
    ..The NOD.Cg-Prkdc(scid) Il2rg(tm1Sug)/Jic (NOG) mouse is an efficient host for PDXs...
  90. pmc Tamoxifen prevents apoptosis and follicle loss from cyclophosphamide in cultured rat ovaries
    Joanna Piasecka-Srader
    Department of Pathology and Laboratory Medicine, University of Kansas Medical Center, Kansas City, Kansas
    Biol Reprod 92:132. 2015
    ..of lipoxygenase and prostaglandin production and signaling (Alox5, Pla2g1b, Ptgfr), cytokine binding (Il1r1, Il2rg ), apoptosis (Tnfrsf1a), second messenger signaling (Mapk1, Mapk14, Plcg1), as well as tissue remodeling and ..
  91. pmc De Novo Generated Human Red Blood Cells in Humanized Mice Support Plasmodium falciparum Infection
    Anburaj Amaladoss
    Infectious Diseases Interdisciplinary Research Group, Singapore Massachusetts Institute of Technology Alliance for Research and Technology, Singapore, 138602, Singapore
    PLoS ONE 10:e0129825. 2015
    ..Engraftment of NOD-scid Il2rg-/- mice with human hematopoietic stem cells leads to reconstitution of human immune cells...
  92. pmc IL-15 receptor α signaling constrains the development of IL-17-producing γδ T cells
    Sara L Colpitts
    Department of Immunology, Center for Integrated Immunology and Vaccine Research, UConn Health, Farmington, CT 06030
    Proc Natl Acad Sci U S A 112:9692-7. 2015
    ..Phenotypic analysis has shown that CD44(high) γδ-17 cells express IL-15Rα and the common gamma chain (CD132), yet lack the IL-2/15Rβ chain (CD122)...
  93. doi The frequency of multipotent CD133(+)CD45RA(-)CD34(+) hematopoietic stem cells is not increased in fetal liver compared with adult stem cell sources
    Stefan Radtke
    Clinical Research Division, Fred Hutchinson Cancer Research Center, Seattle, WA Institute for Transfusion Medicine, University Hospital Essen, University of Duisburg Essen, Essen, North Rhine Westphalia, Germany
    Exp Hematol 44:502-7. 2016
    ..We noted that engraftment in NSG (NOD scid gamma [NOD.Cg-Prkdc(scid) Il2rg(tm1Wjl)/SzJ]) mice was driven mostly by LMPPs, confirming recent findings that repopulation in mice is not a ..
  94. ncbi Reduced IL-7R T Cell Expression and Increased Plasma sCD127 in Late Presenting HIV-Infected Individuals
    Hans J Hartling
    Department of Infectious Diseases, Rigshospitalet, Copenhagen University Hospital, Copenhagen, Denmark Viro Immunology Research Unit Department of Clinical Immunology, Rigshospitalet, Copenhagen University Hospital, Copenhagen, Denmark and Department of Infectious Diseases, Copenhagen University Hospital, Hvidovre, Denmark
    J Acquir Immune Defic Syndr 74:81-90. 2017
    ..Interleukin-7 (IL-7) and the corresponding receptor, IL-7 receptor (IL-7R) made up of CD127 and CD132, are crucial for T cell homeostasis...
  95. pmc Demodex musculi Infestation in Genetically Immunomodulated Mice
    Peter C Smith
    Section of Comparative Medicine, Yale University School of Medicine, New Haven, Connecticut, USA
    Comp Med 66:278-85. 2016
    ..Cg-Prkdc(scid)Il2r(tm1Wjl)/SzJ (NSG) and C;129S4 Rag2(tm1.1Flv) Il2rg(tm1.1Flv)/J mice with varying degrees of blepharitis, conjunctivitis, and facial pruritis...
  96. ncbi Mutation of Jak3 in a patient with SCID: essential role of Jak3 in lymphoid development
    S M Russell
    Laboratory of Molecular Immunology, National Heart, Lung, and Blood Institute NHLBI, National Institutes of Health NIH, Bethesda, MD 20892, USA
    Science 270:797-800. 1995
    Males with X-linked severe combined immunodeficiency (XSCID) have defects in the common cytokine receptor gamma chain (gamma c) gene that encodes a shared, essential component of the receptors of interleukin-2 (IL-2), IL-4, IL-7, IL-9, ..
  97. pmc Lymphoid development in mice with a targeted deletion of the interleukin 2 receptor gamma chain
    J P DiSanto
    Institut National de la Sante et de la Recherche Medicale, Unité 132, Hopital Necker Enfants Malades, Paris, France
    Proc Natl Acad Sci U S A 92:377-81. 1995
    ..Mutations in IL-2R gamma in man appear responsible for the X chromosome-linked immunodeficiency SCIDX1, characterized by a defect in T-cell and natural killer (NK)-cell differentiation with the presence of poorly ..
  98. pmc Interleukin-13 is a potent activator of JAK3 and STAT6 in cells expressing interleukin-2 receptor-gamma and interleukin-4 receptor-alpha
    M G Malabarba
    Division of Basic Science, IRSP, SAIC Frederick, MD, USA
    Biochem J 319:865-72. 1996
    ..Consequently, we predict that partial disruption of IL13 signal transduction also contributes to the severe combined immuno-deficiency syndromes associated with inactivation of the IL2R gamma or JAK3 genes...
  99. ncbi Chromosome mapping and expression of the human interleukin-13 receptor
    J Guo
    INSERM U211, Institut de Biologie, Nantes, France
    Genomics 42:141-5. 1997
    ..1) and the interleukin-9 receptor gene (Xq28). The lack of nucleotide sequence similarity suggests unrelated evolutionary pathways between these receptor genes...
  100. ncbi Occurrence of cytokine receptors on different lymphoid leukaemic cells
    K Koubek
    Clinical Department, Institute of Haematology and Blood Transfusion, Prague, Czech Republic
    Eur J Haematol 63:1-10. 1999
    ..140 kD), CD126 (IL-6R, 80 kD), CD127 (IL-7R, 75 kD), CDw128 (IL-8R), CD130 (gp130 subunit), CDw131 (common beta), CD132 (IL-2Rgamma), CD134 (OX40) and also CD95 (Fas antigen) on the lymphoid leukaemic cells...
  101. ncbi Common cytokine receptor gamma chain (gammac)-deficient B cells persist in T cell-deficient gammac-mice and respond to a T-independent antigen
    C A Vosshenrich
    Institute for Genetics, Cologne, Germany
    Eur J Immunol 30:1614-22. 2000
    Defects in the common cytokine receptor gamma chain (gammac) in man result in X-linked severe combined immunodeficiency disease (SCIDX1) characterized by an absence of alphabeta T cells, gammadelta T cells and NK cells, with the presence ..

Research Grants23

  1. Double Humanization of Liver and Blood in Mice
    ELIZABETH MARIE WILSON; Fiscal Year: 2012
    ..Fah) knockout mice[2], bearing additional mutations in Rag2 and the interleukin common gamma chain receptor (Il2rg) designated as FRGN to generate such chimeric animals...
  2. David A Williams; Fiscal Year: 2014
    ..Inclusion criteria include patients with a definitive diagnosis of SCIDX1 in whom HLA-matched family donors are unavailable and who are either patients >3...
  3. UTPAL P DAVE; Fiscal Year: 2016
    ..is gone but leukemic cells remain hyper- responsive to cytokines that utilize the IL2R common gamma chain (IL2RG), suggesting a role for host factors in tumor progression...
  4. Matthew H Porteus; Fiscal Year: 2016
    ..Specific aim 1 is focused o developing nuclease mediated gene targeting for the IL2RG gene. Specific aim 2 is focused on developing nuclease mediated gene targeting for the ADA gene...
  5. Novel indication for myeloid progenitor use: Induction of tolerance
    Timothy C Fong; Fiscal Year: 2013
    ..Important, MP from B10;B6-Rag2-/-Il2rg- /- mice, which are incapable of producing functional B, T or NK cells, induce tolerance and clearly show that ..
  6. Jae U Jung; Fiscal Year: 2016
    ..3) We have developed an "infectious" KSHV bacterial artificial chromosome (BAC16) and NOD/SCID IL2Rg-/- (NSG) "humanized" mouse model that allows efficient viral genetic manipulation and in vivo persistence study, ..
  7. Gene Correction for XSCID in Hematopoietic Stem Cells
    JONATHAN WALSH; Fiscal Year: 2005
    ..We propose to generate mouse models for SCID where the IL2RG locus contains small insertions, a restriction site for the yeast enzyme I-scel which creates a stop codon, or ..
  8. DAVID MICHAEL LANGENAU; Fiscal Year: 2016
    ..gene 2 (rag2), Janus kinase 3 (jak3), DNA-dependent protein kinase (prkdc), and interieukin 2-receptor gamma (IL2rg)...
  9. BRUCE EDWARD TORBETT; Fiscal Year: 2016
    ..engraftability and safety of genetically corrected iPSC-derived blood cells following transplantation in NOD/SCD/IL2Rg-/- (NSG) mice...
  10. In vivo expansion of human hepatocytes in Fah-/-Rag-/-Il2rg-/- mice
    Markus Grompe; Fiscal Year: 2010
    ..Commercialization of our technology will greatly reduce the cost of and accelerate new drug development, thus contributing to public health. ..
  11. Sanjay Vashee; Fiscal Year: 2014
    ..to that of laboratory-adapted strains and other clinical isolates in vitro and in vivo using humanized NOD/SCID/IL2Rg-nul mice...
  12. Cytokine signal transduction in immune system
    Hodaka Fujii; Fiscal Year: 2007
    ..Detailed information gained from the proposed experiments will be valuable for elucidation of molecular mechanisms of oncogenesis and autoimmune diseases caused by dysregulation of cytokine signaling. ..
  13. Cell Death and Clonal Survival in Myelodysplastic Syndr*
    H Deeg; Fiscal Year: 2008
    ..To achieve these objectives, we must better understand the underlying mechanisms of MDS and thereby identify ways by which the disease process can be arrested, progression prevented, and the disease hopefully be cured. ..
  14. WT1 Expression in Patients with MDS Treated with ATG an*
    H Deeg; Fiscal Year: 2007
    ..unreadable] [unreadable]..
  15. NOVEL THERAPEUTIC STRATEGIES IN MYELODYSPLASTIC SYNDROME
    H Deeg; Fiscal Year: 2003
    ..Optimization of therapy, in turn, should reduce morbidity and mortality associated with MDS and improve the quality of life of patients with MDS. ..
  16. Molecular Determinants of Virulence Porcine Circovirus 2
    Steven Krakowka; Fiscal Year: 2007
    ..Collectively, these data will provide critical insight into pathogenic mechanisms of disease mediated by mammalian circoviruses and will provide key guidance for the future study of human circovirus pathogens. ..
  17. Mast Cells and IBD Pathogenesis
    LAURA HALE; Fiscal Year: 2007
    ..unreadable] [unreadable] [unreadable]..
  18. SNP Analysis of Endometriosis Candidate Genes
    Idhaliz Flores; Fiscal Year: 2010
    ....
  19. Environmentally-Induced Ocular Surface Inflammation Mimicking Sjogren's Syndrome
    Michael J Stern; Fiscal Year: 2010
    ..We further propose that the severity of this ALKC is worsened by defective self tolerance mechanisms. ..
  20. Chemoprevention of IBD-Associated Colon Cancer
    Laura P Hale; Fiscal Year: 2010
    ..The proposed studies will further elucidate mechanisms that can lead to novel interventions to prevent IBD-associated colon cancers, providing great benefit to IBD patients and their families. ..
  21. Bromelain Proteinase Activity in IBD
    LAURA HALE; Fiscal Year: 2006
    ..They will thus strengthen the scientific knowledge base needed to inform the practice of complementary and alternative medicine. ..
  22. Novel Oral Adjuvant for Dental Vaccines
    LAURA HALE; Fiscal Year: 2005
    ..abstract_text> ..
  23. Bromelain as a Biologically-Based Therapy for IBD
    LAURA HALE; Fiscal Year: 2005
    ..This research will thus strengthen the biomedical research knowledge base needed to inform the practice of complementary and alternative medicine. ..