Leonard D Shultz

Summary

Affiliation: The Jackson Laboratory
Country: USA

Publications

  1. ncbi request reprint Mechanisms of anemia in SHP-1 protein tyrosine phosphatase-deficient "viable motheaten" mice
    Bonnie L Lyons
    The Jackson Laboratory, Bar Harbor, ME 04609, USA
    Exp Hematol 31:234-43. 2003
  2. ncbi request reprint Regulation of human short-term repopulating cell (STRC) engraftment in NOD/SCID mice by host CD122+ cells
    Leonard D Shultz
    The Jackson Laboratory, 600 Main Street, Bar Harbor, Maine 04609, USA
    Exp Hematol 31:551-8. 2003
  3. pmc Humanized mice for immune system investigation: progress, promise and challenges
    Leonard D Shultz
    Jackson Laboratory, 600 Main Street, Bar Harbor, Maine 04609, USA
    Nat Rev Immunol 12:786-98. 2012
  4. ncbi request reprint NOD/LtSz-Rag1nullPfpnull mice: a new model system with increased levels of human peripheral leukocyte and hematopoietic stem-cell engraftment
    Leonard D Shultz
    The Jackson Laboratory, Bar Harbor, ME 04609, USA
    Transplantation 76:1036-42. 2003
  5. ncbi request reprint NOD/LtSz-Rag1null mice: an immunodeficient and radioresistant model for engraftment of human hematolymphoid cells, HIV infection, and adoptive transfer of NOD mouse diabetogenic T cells
    L D Shultz
    The Jackson Laboratory, Bar Harbor, ME 04609, USA
    J Immunol 164:2496-507. 2000
  6. ncbi request reprint Humanized mice in translational biomedical research
    Leonard D Shultz
    The Jackson Laboratory, 600 Main Street, Bar Harbor, Maine 04609, USA
    Nat Rev Immunol 7:118-30. 2007
  7. ncbi request reprint Mutations at the mouse ichthyosis locus are within the lamin B receptor gene: a single gene model for human Pelger-Huët anomaly
    Leonard D Shultz
    The Jackson Laboratory, Bar Harbor, ME 04609, USA
    Hum Mol Genet 12:61-9. 2003
  8. ncbi request reprint Human lymphoid and myeloid cell development in NOD/LtSz-scid IL2R gamma null mice engrafted with mobilized human hemopoietic stem cells
    Leonard D Shultz
    The Jackson Laboratory, Bar Harbor, ME 04609, USA
    J Immunol 174:6477-89. 2005
  9. ncbi request reprint Absence of CD5 dramatically reduces progression of pulmonary inflammatory lesions in SHP-1 protein-tyrosine phosphatase-deficient 'viable motheaten' mice
    Melissa J Joliat
    The Jackson Laboratory, Bar Harbor, ME 04609, USA
    J Autoimmun 18:105-17. 2002
  10. ncbi request reprint Multiple defects in innate and adaptive immunologic function in NOD/LtSz-scid mice
    L D Shultz
    Jackson Laboratory, Bar Harbor, ME 04609
    J Immunol 154:180-91. 1995

Detail Information

Publications100

  1. ncbi request reprint Mechanisms of anemia in SHP-1 protein tyrosine phosphatase-deficient "viable motheaten" mice
    Bonnie L Lyons
    The Jackson Laboratory, Bar Harbor, ME 04609, USA
    Exp Hematol 31:234-43. 2003
    ..The aim of this study was to investigate the mechanisms underlying anemia in me(v)/me(v) mice...
  2. ncbi request reprint Regulation of human short-term repopulating cell (STRC) engraftment in NOD/SCID mice by host CD122+ cells
    Leonard D Shultz
    The Jackson Laboratory, 600 Main Street, Bar Harbor, Maine 04609, USA
    Exp Hematol 31:551-8. 2003
    ..The objective of these studies was to deplete NK cells both by genetic manipulation of the hosts and by antibody depletion of cell populations that may regulate engraftment with human STRC...
  3. pmc Humanized mice for immune system investigation: progress, promise and challenges
    Leonard D Shultz
    Jackson Laboratory, 600 Main Street, Bar Harbor, Maine 04609, USA
    Nat Rev Immunol 12:786-98. 2012
    ..In this Review, we discuss recent advances in the development and utilization of humanized mice, the lessons learnt, the remaining challenges and the promise of using humanized mice for the in vivo study of human immunology...
  4. ncbi request reprint NOD/LtSz-Rag1nullPfpnull mice: a new model system with increased levels of human peripheral leukocyte and hematopoietic stem-cell engraftment
    Leonard D Shultz
    The Jackson Laboratory, Bar Harbor, ME 04609, USA
    Transplantation 76:1036-42. 2003
    ....
  5. ncbi request reprint NOD/LtSz-Rag1null mice: an immunodeficient and radioresistant model for engraftment of human hematolymphoid cells, HIV infection, and adoptive transfer of NOD mouse diabetogenic T cells
    L D Shultz
    The Jackson Laboratory, Bar Harbor, ME 04609, USA
    J Immunol 164:2496-507. 2000
    ..These data demonstrate the advantages of NOD/LtSz-Rag1null mice as a radiation and lymphoma-resistant model for long-term analyses of engrafted human hematolymphoid cells or diabetogenic NOD lymphoid cells...
  6. ncbi request reprint Humanized mice in translational biomedical research
    Leonard D Shultz
    The Jackson Laboratory, 600 Main Street, Bar Harbor, Maine 04609, USA
    Nat Rev Immunol 7:118-30. 2007
    ....
  7. ncbi request reprint Mutations at the mouse ichthyosis locus are within the lamin B receptor gene: a single gene model for human Pelger-Huët anomaly
    Leonard D Shultz
    The Jackson Laboratory, Bar Harbor, ME 04609, USA
    Hum Mol Genet 12:61-9. 2003
    ..These spontaneous Lbr mutations confirm the molecular basis of human PHA and provide a small animal model for determination of the precise function of LBR in normal and pathological states...
  8. ncbi request reprint Human lymphoid and myeloid cell development in NOD/LtSz-scid IL2R gamma null mice engrafted with mobilized human hemopoietic stem cells
    Leonard D Shultz
    The Jackson Laboratory, Bar Harbor, ME 04609, USA
    J Immunol 174:6477-89. 2005
    ..Thus, NOD-scid IL2Rgamma(null) mice engrafted with human mobilized blood stem cells provide a new in vivo long-lived model of robust multilineage human HSC engraftment...
  9. ncbi request reprint Absence of CD5 dramatically reduces progression of pulmonary inflammatory lesions in SHP-1 protein-tyrosine phosphatase-deficient 'viable motheaten' mice
    Melissa J Joliat
    The Jackson Laboratory, Bar Harbor, ME 04609, USA
    J Autoimmun 18:105-17. 2002
    ..Study of CD5(null)me(v)/ me(v) mice may provide further insight into the role of CD5 in cell signaling and may help explain the observed association of CD5(+) B-cells with autoimmune disease...
  10. ncbi request reprint Multiple defects in innate and adaptive immunologic function in NOD/LtSz-scid mice
    L D Shultz
    Jackson Laboratory, Bar Harbor, ME 04609
    J Immunol 154:180-91. 1995
    ..B-17/Sz-scid/scid animals. The multiple defects in innate and adaptive immunity unique to the NOD/LtSz-scid/scid mouse provide an excellent in vivo environment for reconstitution with human hematopoietic cells...
  11. ncbi request reprint T cell developmental defects in 'viable motheaten' mice deficient in SHP-1 protein-tyrosine phosphatase. Developmental defects are corrected in vitro in the presence of normal hematopoietic-origin stromal cells and in vivo by exogenous IL-7
    Sherri W Christianson
    The Jackson Laboratory, 600 Main Street, Bar Harbor, ME 04609, USA
    J Autoimmun 18:119-30. 2002
    ..We conclude that abnormal T cell development in me(v)/ me(v) mice may in part be due to defects in the ability of bone marrow-derived accessory cells to provide bioavailable IL-7 to developing thymocytes...
  12. ncbi request reprint Genetic disassociation of autoimmunity and resistance to costimulation blockade-induced transplantation tolerance in nonobese diabetic mice
    Todd Pearson
    Program in Immunology and Virology, University of Massachusetts Medical School, Worcester, MA 01655, USA
    J Immunol 171:185-95. 2003
    ..The outcomes of tolerance induction protocols tested in NOD mice may not accurately predict outcomes in human subjects...
  13. pmc The mouse mutation "thrombocytopenia and cardiomyopathy" (trac) disrupts Abcg5: a spontaneous single gene model for human hereditary phytosterolemia/sitosterolemia
    Thomas H Chase
    The Jackson Laboratory, Bar Harbor, ME, USA
    Blood 115:1267-76. 2010
    ..The trac mutation provides a new clinically significant animal model for human phytosterolemia and provides a new means for studying the role of phytosterols in hematologic diseases and testing therapeutic interventions...
  14. 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
    ..Thus, the NOD/SCID/IL2rgamma(null) newborn system might be an important experimental model to study the human hemato-lymphoid system...
  15. ncbi request reprint Deficiency of SHP-1 protein-tyrosine phosphatase in "viable motheaten" mice results in retinal degeneration
    Bonnie L Lyons
    The Jackson Laboratory, Bar Harbor, Maine 04609, USA
    Invest Ophthalmol Vis Sci 47:1201-9. 2006
    ..These mice exhibit immune dysfunction, hyperproliferation of myeloid cells, and regenerative anemia. This study focused on the role of SHP-1 in retinal homeostasis...
  16. ncbi request reprint Establishment and characterization of a new osteogenic cell line (MOS-J) from a spontaneous C57BL/6J mouse osteosarcoma
    Melissa J Joliat
    Jackson Laboratory, 600 Main Street, Bar Harbor, ME 04609, USA
    In Vivo 16:223-8. 2002
    ..These characteristics indicate the potential utility of the MOS-J osteosarcoma cell line as a model for studies of human osteosarcoma and bone biology...
  17. pmc Generation of functional human T-cell subsets with HLA-restricted immune responses in HLA class I expressing NOD/SCID/IL2r gamma(null) humanized mice
    Leonard D Shultz
    The Jackson Laboratory, Bar Harbor, ME 04609
    Proc Natl Acad Sci U S A 107:13022-7. 2010
    ....
  18. ncbi request reprint Rejection of human islets and human HLA-A2.1 transgenic mouse islets by alloreactive human lymphocytes in immunodeficient NOD-scid and NOD-Rag1(null)Prf1(null) mice
    Scott J Banuelos
    Department of Surgery, University of Massachusetts Medical School, Worcester, MA 01605, USA
    Clin Immunol 112:273-83. 2004
    ..host disease (GVHD). We propose that diabetic NOD-RagI(null)Prf1(null) mice co-engrafted with HLA-A2 mouse transgenic islets and allogeneic human PBMC provide an effective in vivo model of human islet allograft rejection...
  19. ncbi request reprint Prolonged survival of neonatal porcine islet xenografts in mice treated with a donor-specific transfusion and anti-CD154 antibody
    Michael C Appel
    Department of Medicine, Division of Diabetes, University of Massachusetts Medical School, Worcester, MA 01605, USA
    Transplantation 77:1341-9. 2004
    ....
  20. ncbi request reprint Long-term engraftment and expansion of tumor-derived memory T cells following the implantation of non-disrupted pieces of human lung tumor into NOD-scid IL2Rgamma(null) mice
    Michelle R Simpson-Abelson
    State University of New York, Department of Microbiology and Immunology and The Witebsky Center for Microbial Pathogenesis and Immunology, University at Buffalo, Buffalo, NY 14214, USA
    J Immunol 180:7009-18. 2008
    ..We conclude that the NOD-scid IL2Rgamma(null) tumor xenograft model provides an opportunity to study tumor and tumor-stromal cell interactions in situ for prolonged periods...
  21. pmc Retrotransposon insertion in the T-cell acute lymphocytic leukemia 1 (Tal1) gene is associated with severe renal disease and patchy alopecia in Hairpatches (Hpt) mice
    Vishnu Hosur
    The Jackson Laboratory, Bar Harbor, Maine, United States of America
    PLoS ONE 8:e53426. 2013
    ..The Hairpatches mouse model allows an investigation into the effects of Tal1, a transcription factor characterized by complex regulation patterns, and its effects on renal disease...
  22. ncbi request reprint The flaky skin (fsn) mutation in mice: map location and description of the anemia
    W G Beamer
    Jackson Laboratory, Bar Harbor, ME 04609 1500, USA
    Blood 86:3220-6. 1995
    ..The mechanism whereby fsn induces the reported pleiotropic effects has yet to be elucidated...
  23. ncbi request reprint Epidermal dendritic cell populations in the flaky skin mutant mouse
    J P Sundberg
    Jackson Laboratory, Bar Harbor, ME 04609
    Immunol Invest 22:389-401. 1993
    ..These studies suggest that the psoriasiform dermatitis in the flaky skin mouse mutation is associated with abnormalities at the level of hematopoietic progenitor cells...
  24. pmc Creation of "humanized" mice to study human immunity
    Todd Pearson
    Diabetes Division, University of Massachusetts Medical School, Worcester, Massachusetts, USA
    Curr Protoc Immunol . 2008
    ....
  25. ncbi request reprint Accelerated wound healing of alkali-burned corneas in MRL mice is associated with a reduced inflammatory signature
    Motonobu Ueno
    Jackson Laboratory, Bar Harbor, ME 04609, USA
    Invest Ophthalmol Vis Sci 46:4097-106. 2005
    ..The present study was conducted to investigate healing of alkali-burned corneas in MRL/MpJ (MRL) mice...
  26. pmc Cancer stem cells are enriched in the side population cells in a mouse model of glioma
    Molly A Harris
    The Jackson Laboratory, Bar Harbor, Maine 04609, USA
    Cancer Res 68:10051-9. 2008
    ..In conclusion, this study shows that CSCs exist in a mouse glioma model, suggesting that this model can be used to study the molecular and cellular characteristics of CSCs in vivo and to further test the CSC hypothesis...
  27. ncbi request reprint Anti-mouse CD154 antibody treatment facilitates generation of mixed xenogeneic rat hematopoietic chimerism, prevents wasting disease and prolongs xenograft survival in mice
    Hideyuki Masaki
    Division of Diabetes, University of Massachusetts Medical School, Worcester, MA, USA
    Xenotransplantation 13:224-32. 2006
    ..The goal of this study was to determine if co-stimulation blockade would facilitate chimerism and xenograft tolerance in irradiation-conditioned concordant recipients...
  28. ncbi request reprint Evaluation of donor-specific transfusion sources: unique failure of bone marrow cells to induce prolonged skin allograft survival with anti-CD154 monoclonal antibody
    Thomas G Markees
    Department of Medicine, University of Massachusetts Medical School, Worcester, MA 01605, USA
    Transplantation 78:1601-8. 2004
    ..It is unknown if other lymphohematopoietic cell populations can be used as a DST...
  29. ncbi request reprint Development of new-generation HU-PBMC-NOD/SCID mice to study human islet alloreactivity
    Marie King
    Diabetes Division, University of Massachusetts Medical School, 373 Plantation Street, Suite 218, Worcester, MA 01605, USA
    Ann N Y Acad Sci 1103:90-3. 2007
    ..Herein, we describe the challenges of recapitulating human immunity in humanized mice and features of NOD-scid Il2rgamma(null) mice that help overcome them...
  30. pmc T cell-specific siRNA delivery suppresses HIV-1 infection in humanized mice
    Priti Kumar
    Immune Disease Institute and Department of Pediatrics, Harvard Medical School, Boston, MA 02115, USA
    Cell 134:577-86. 2008
    ..Moreover, scFvCD7-9R could deliver antiviral siRNAs to naive T cells in Hu-HSC mice and effectively suppress viremia in infected mice. Thus, siRNA therapy for HIV infection appears to be feasible in a preclinical animal model...
  31. pmc In childhood acute lymphoblastic leukemia, blasts at different stages of immunophenotypic maturation have stem cell properties
    Christoph le Viseur
    Department of Pediatric Hematology and Oncology, University Children s Hospital Munster, 48129 Munster, Germany
    Cancer Cell 14:47-58. 2008
    ..Sorted blast populations mirrored normal B precursor cells with transcription of a number of stage-appropriate genes. These observations inform a model for leukemia-propagating stem cells in childhood ALL...
  32. pmc Expanded CD34+ human umbilical cord blood cells generate multiple lymphohematopoietic lineages in NOD-scid IL2rgamma(null) mice
    Lisa J Giassi
    Division of Diabetes, Department of Medicine, University of Massachusetts Medical School, Worcester, Massachusetts 01605, USA
    Exp Biol Med (Maywood) 233:997-1012. 2008
    ..Ex vivo expanded CD34(+) human UCB cells have the capacity to generate multiple hematopoietic lineages and a functional human immune system upon transplantation into TNFalpha-treated NOD-scid IL2rgamma(null) mice...
  33. ncbi request reprint Human cancer growth and therapy in immunodeficient mouse models
    Leonard D Shultz
    The Jackson Laboratory, Bar Harbor, Maine 04609
    Cold Spring Harb Protoc 2014:pdb.top073585. 2014
    ..Moreover, NSG mice can be engrafted with functional human immune systems, permitting for the first time the potential to study primary human tumors in vivo in the presence of a human immune system. ..
  34. pmc Dystrophin and dysferlin double mutant mice: a novel model for rhabdomyosarcoma
    Vishnu Hosur
    The Jackson Laboratory, Bar Harbor, ME, USA
    Cancer Genet 205:232-41. 2012
    ..The present finding bolsters the correlation between MD and sarcomas, and provides a model not only to examine the cellular origins but also to identify mechanisms and signal transduction pathways triggering development of RMS...
  35. pmc Dysferlin deficiency and the development of cardiomyopathy in a mouse model of limb-girdle muscular dystrophy 2B
    Thomas H Chase
    The Jackson Laboratory, Bar Harbor, Maine 04609, USA
    Am J Pathol 175:2299-308. 2009
    ..This damage results in transient functional deficits at 10 months of age, but, unlike in skeletal muscle, the cell injury is sublethal and causes only mild cardiomyopathy even at advanced ages...
  36. ncbi request reprint Emv30null NOD-scid mice. An improved host for adoptive transfer of autoimmune diabetes and growth of human lymphohematopoietic cells
    D V Serreze
    Jackson Laboratory, Bar Harbor, Maine 04609, USA
    Diabetes 44:1392-8. 1995
    ..Significantly, the ability of standard NOD T-cells to transfer IDDM to the Emv30null NOD-scid stock was not impaired.(ABSTRACT TRUNCATED AT 250 WORDS)..
  37. ncbi request reprint Alloimmune injury and rejection of human skin grafts on human peripheral blood lymphocyte-reconstituted non-obese diabetic severe combined immunodeficient beta2-microglobulin-null mice
    Nicole A Turgeon
    University of Massachusetts Medical School, Worcester, Massachussetts 01605, USA
    Exp Biol Med (Maywood) 228:1096-104. 2003
    ..It is proposed that Hu-PBL-NOD-scid B2mnull mice engrafted with human skin will provide a useful model for analysis of interventions designed to modulate human allograft rejection...
  38. ncbi request reprint Regulation of skin and islet allograft survival in mice treated with costimulation blockade is mediated by different CD4+ cell subsets and different mechanisms
    Scott J Banuelos
    Department of Surgery, University of Massachusetts Medical School, 373 Plantation Street, Worcester, MA 01605, USA
    Transplantation 78:660-7. 2004
    ..Induction of skin allograft survival requires the presence of CD4 cells and deletion of alloreactive CD8 cells. The specific roles of CD4 and CD4CD25 cells and the mechanism(s) by which they act are not fully understood...
  39. ncbi request reprint Human cord blood--derived cells generate insulin-producing cells in vivo
    Shuro Yoshida
    Department of Medicine and Biosystemic Science, Kyushu University Graduate School of Medicine, 3 1 1 Maidashi, Higashi ku, Fukuoka 812 8582, Japan
    Stem Cells 23:1409-16. 2005
    ..Taken together, human CB contains progenitor cells, which can generate insulin-producing cells in recipient pancreatic tissues across a xenogeneic histocompatibility barrier by fusion-dependent and -independent mechanisms...
  40. ncbi request reprint An assay for human hematopoietic stem cells based on transplantation into nonobese diabetic recombination activating gene-null perforin-null mice
    Hitoshi Minamiguchi
    Department of Veterans Affairs Medical Center, Medical University of South Carolina, Charleston, South Carolina 29401 5799, USA
    Biol Blood Marrow Transplant 11:487-94. 2005
    ..Accordingly, conditioned newborn NOD- Rag1 null Prf1 null mice provide a desirable model for an assay of long-term transplantable human HSCs...
  41. ncbi request reprint A new Hu-PBL model for the study of human islet alloreactivity based on NOD-scid mice bearing a targeted mutation in the IL-2 receptor gamma chain gene
    Marie King
    Department of Medicine, University of Massachusetts Medical School, 373 Plantation Street, Worcester, MA 01655, USA
    Clin Immunol 126:303-14. 2008
    ..These data suggest that humanized NOD-scid Il2rgamma(null) mice may represent an important surrogate for investigating in vivo mechanisms of human islet allograft rejection...
  42. pmc TLR agonists abrogate costimulation blockade-induced prolongation of skin allografts
    Thomas B Thornley
    Division of Diabetes, Department of Medicine, University of Massachusetts Medical School, Worcester, MA 01605, USA
    J Immunol 176:1561-70. 2006
    ..We conclude that TLR signaling abrogates the effects of costimulation blockade by preventing alloreactive CD8+ T cell apoptosis through a mechanism not dependent on TNFR2 or IL-12R signaling...
  43. ncbi request reprint Subcapsular transplantation of tissue in the kidney
    Leonard D Shultz
    The Jackson Laboratory, Bar Harbor, Maine 04609
    Cold Spring Harb Protoc 2014:pdb.prot078089. 2014
    ..Furthermore, the retroperitoneal location of the kidney, together with its separation from other organs, is advantageous both for imaging and biopsy. ..
  44. pmc Rhbdf2 mutations increase its protein stability and drive EGFR hyperactivation through enhanced secretion of amphiregulin
    Vishnu Hosur
    The Jackson Laboratory, Bar Harbor, ME 04609
    Proc Natl Acad Sci U S A 111:E2200-9. 2014
    ..This work underscores the physiological prominence of iRhom2 in controlling EGFR signaling events involved in wound healing and neoplastic growth, and yields insight into the function of key iRhom2 domains. ..
  45. pmc Humanized mice as a preclinical tool for infectious disease and biomedical research
    Leonard D Shultz
    The Jackson Laboratory, Bar Harbor, Maine, USA
    Ann N Y Acad Sci 1245:50-4. 2011
    ..Immunodeficient mice bearing an IL2rγ(null) mutation permit engraftment of a functional human immune system and study of human-specific infectious agents that was not previously possible...
  46. ncbi request reprint Expression of terminal differentiation proteins defines stages of mouse mammary gland development
    I Mikaelian
    Igor Mikaelian, Box 98, The Jackson Laboratory, 600 Main Street, Bar Harbor, ME 04609 1500, USA
    Vet Pathol 43:36-49. 2006
    ..Finally, consistent with results of earlier studies, keratins 1, 10, 13, and 15, and filaggrin, involucrin, and loricrin were not detected at any stage of mammary gland development...
  47. ncbi request reprint Multiple low-dose streptozocin-induced diabetes in NOD-scid/scid mice in the absence of functional lymphocytes
    I C Gerling
    Jackson Laboratory, Bar Harbor, ME 04609
    Diabetes 43:433-40. 1994
    ..We conclude that IDDM induced by MD-STZ is not mediated by T-cell- or B-cell-dependent autoimmune mechanisms in a fashion analogous to the spontaneous IDDM characteristic of NOD mice...
  48. ncbi request reprint Lanceolate hair-J (lahJ): a mouse model for human hair disorders
    J P Sundberg
    The Jackson Laboratory, Bar Harbor, Maine 04609 1500, USA
    Exp Dermatol 9:206-18. 2000
    ..Homozygotes for either of these allelic mouse mutations have elevated serum IgE levels, a feature also common with human Netherton's syndrome...
  49. ncbi request reprint CSF-1 regulation of Il6 gene expression by murine macrophages: a pivotal role for GM-CSF
    R Evans
    The Jackson Laboratory, Bar Harbor, Maine 04609, USA
    J Leukoc Biol 64:810-6. 1998
    ....
  50. ncbi request reprint Development and progression of psoriasiform dermatitis and systemic lesions in the flaky skin (fsn) mouse mutant
    J P Sundberg
    Jackson Laboratory, Bar Harbor, ME 04609 1500, USA
    Pathobiology 65:271-86. 1997
    ..This spontaneous mouse mutation provides a useful model for modulating neovascularization and keratinocyte hyperproliferation, especially since the cutaneous changes resemble some forms of psoriasis in humans...
  51. ncbi request reprint Severe defects in immunity and hematopoiesis caused by SHP-1 protein-tyrosine-phosphatase deficiency
    L D Shultz
    Jackson Laboratory, Bar Harbor, ME 04609, USA
    Trends Biotechnol 15:302-7. 1997
    ..This natural model for a specific protein-tyrosine-phosphatase deficiency is being widely utilized to determine the role of SHP-1 in the negative regulation of multiple signaling pathways in a number of hematopoietic lineages...
  52. pmc The nonobese diabetic scid mouse: model for spontaneous thymomagenesis associated with immunodeficiency
    M Prochazka
    Jackson Laboratory, Bar Harbor, ME 04609
    Proc Natl Acad Sci U S A 89:3290-4. 1992
    ..We propose that the unusual features of T-cell ontogeny characteristic of the NOD inbred strain synergize with the scid-imparted block in thymocyte development, leading to activation of the NOD-unique Emv-30 to initiate thymomagenesis...
  53. ncbi request reprint Spontaneous mutations in the mouse Sharpin gene result in multiorgan inflammation, immune system dysregulation and dermatitis
    R E Seymour
    The Jackson Laboratory, Bar Harbor, ME 04609, USA
    Genes Immun 8:416-21. 2007
    ..These are the first examples of disease-causing mutations in the Sharpin gene and demonstrate the importance of SHARPIN protein in normal immune development and control of inflammation...
  54. ncbi request reprint Distinct osteoclast precursors in the bone marrow and extramedullary organs characterized by responsiveness to Toll-like receptor ligands and TNF-alpha
    Shin ichi Hayashi
    Department of Molecular and Cellular Biology, School of Life Science, Faculty of Medicine, Institute of Regenerative Medicine and Biofunction, Graduate School of Medical Science, Tottori University, Tottori, Japan
    J Immunol 171:5130-9. 2003
    ....
  55. doi request reprint 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
    ..These models will be instrumental to studies on leukemia-initiating stem cells, leukemia biology, preclinical treatment studies, and to obtain patient-specific preclinical models to design and investigate patient-tailored therapies...
  56. ncbi request reprint Humanized NOD/LtSz-scid IL2 receptor common gamma chain knockout mice in diabetes research
    Leonard D Shultz
    Division of Diabetes, Department of Medicine, 373 Plantation Street, Biotech 2, Suite 218, Worcester, MA 01605, USA
    Ann N Y Acad Sci 1103:77-89. 2007
    ..Overall, development of these new generations of humanized mice should facilitate in vivo studies of the human immune system as well as permit the investigation of the pathogenesis and effector phases of human T1D...
  57. ncbi request reprint Targeting gastrin-releasing peptide receptors on small cell lung cancer cells with a bispecific molecule that activates polyclonal T lymphocytes
    Jiehua Zhou
    Division of Blood and Marrow Transplantation, Department of Medicine and Moores University of California at San Diego Cancer Center, University of California San Diego, La Jolla, California, USA
    Clin Cancer Res 12:2224-31. 2006
    ..We constructed a bispecific molecule, OKT3xAntag2, by conjugating a monoclonal antibody OKT3 (anti-CD3) with a bombesin/GRP antagonist (Antag2) and evaluated cytotoxicity against SCLC cells...
  58. ncbi request reprint The leukotriene B4 receptor (BLT1) is required for effector CD8+ T cell-mediated, mast cell-dependent airway hyperresponsiveness
    Christian Taube
    Division of Cell Biology, Department of Pediatrics, National Jewish Medical and Research Center, Denver, CO 80206, USA
    J Immunol 176:3157-64. 2006
    ..Together, these data define the important role for mast cells and the LTB4-BLT1 pathway in the development of CD8+ T cell-mediated allergic responses in the lung...
  59. ncbi request reprint Human cord blood- and bone marrow-derived CD34+ cells regenerate gastrointestinal epithelial cells
    Fumihiko Ishikawa
    Department of Medicine and Biosystemic Science, Kyushu University Graduate School of Medicine, Fukuoka, Japan
    FASEB J 18:1958-60. 2004
    ..On the basis of these findings, it is concluded that purified human cord blood and bone marrow CD34+ progenitor cells can generate gastrointestinal epithelial cells across allogeneic and xenogeneic histocompatibility barriers...
  60. pmc Appearance of human plasma cells following differentiation of human B cells in NOD/SCID mouse spleen
    Kentaro Kikuchi
    Division of Rheumatology, Allergy and Clinical Immunology, University of California at Davis School of Medicine, TB 192, Davis, CA 95616, USA
    Clin Dev Immunol 10:197-202. 2003
    ..The model will be useful for studying the differentiation of human B cells and generation of plasma cells...
  61. pmc Disruption of the Jnk2 (Mapk9) gene reduces destructive insulitis and diabetes in a mouse model of type I diabetes
    Anja Jaeschke
    Howard Hughes Medical Institute and Program in Molecular Medicine, University of Massachusetts Medical School, Worcester, MA 01605, USA
    Proc Natl Acad Sci U S A 102:6931-5. 2005
    ..We conclude that JNK protein kinases may have important roles in diabetes, including functions of JNK1 in type 2 diabetes and JNK2 in type 1 diabetes...
  62. ncbi request reprint Autoimmune diabetes and resistance to xenograft transplantation tolerance in NOD mice
    Ethel J Gordon
    University of Massachusetts Medical School, Diabetes Division, Department of Medicine, 373 Plantation St, Biotech 2, Suite 218, Worcester, MA 01605, USA
    Diabetes 54:107-15. 2005
    ..We conclude that the resistance of NOD mice to xenograft tolerance induction involves some mechanisms that also participate in the expression of autoimmunity and other mechanisms that are distinct...
  63. pmc Chronic proliferative dermatitis in Sharpin null mice: development of an autoinflammatory disease in the absence of B and T lymphocytes and IL4/IL13 signaling
    Christopher S Potter
    The Jackson Laboratory, Bar Harbor, Maine, United States of America
    PLoS ONE 9:e85666. 2014
    ..Both the cutaneous and systemic inflammation is enhanced by loss of IL4 and IL13 signaling indicating that these cytokines normally play an anti-inflammatory role in SHARPIN-deficient mice...
  64. ncbi request reprint Initiation of autoimmune diabetes in NOD/Lt mice is MHC class I-dependent
    D V Serreze
    The Jackson Laboratory, Bar Harbor, ME 04609, USA
    J Immunol 158:3978-86. 1997
    ....
  65. ncbi request reprint Brugian infections in the peritoneal cavities of laboratory mice: kinetics of infection and cellular responses
    T V Rajan
    The University of Connecticut Health Center, 263 Farmington Avenue, 06030 3105, USA
    Exp Parasitol 100:235-47. 2002
    ..These granulomas may represent an important mechanism by which worms are eliminated...
  66. pmc Mouse neutrophils lacking lamin B-receptor expression exhibit aberrant development and lack critical functional responses
    Peter Gaines
    University of Massachusetts Lowell, Department of Biological Sciences, Lowell, MA 01854, USA peter_uml edu
    Exp Hematol 36:965-76. 2008
    ..In this study, we have investigated roles for LBR in mediating neutrophil development and activation of multiple neutrophil functions, including chemotaxis and the respiratory burst...
  67. ncbi request reprint Bombesin/gastrin-releasing peptide receptor: a potential target for antibody-mediated therapy of small cell lung cancer
    Jiehua Zhou
    Division of Blood and Marrow Transplantation, Department of Medicine, University of California San Diego, La Jolla, California 92093 0960, USA
    Clin Cancer Res 9:4953-60. 2003
    ..Targeted immunotherapy combined with chemotherapy enhanced cell killing...
  68. pmc Inflammation and autoimmunity caused by a SHP1 mutation depend on IL-1, MyD88, and a microbial trigger
    Ben A Croker
    Department of Genetics, The Scripps Research Institute, 10550 N Torrey Pines Road, La Jolla, CA 92037, USA
    Proc Natl Acad Sci U S A 105:15028-33. 2008
    ..The SHP1-deficient phenotype is driven by microbes, which activate TLR signaling pathways to elicit IL-1 production. IL-1 signaling via MyD88 elicits inflammatory disease...
  69. pmc Type 1 IFN mediates cross-talk between innate and adaptive immunity that abrogates transplantation tolerance
    Thomas B Thornley
    Department of Medicine, Division of Diabetes, University of Massachusetts Medical School, Worcester, MA 01655, USA
    J Immunol 179:6620-9. 2007
    ..Blocking of type 1 IFN-dependent pathways in patients may improve allograft survival in the presence of exogenous TLR ligands...
  70. ncbi request reprint Lipopolysaccharide-induced osteoclastogenesis in Src homology 2-domain phosphatase-1-deficient viable motheaten mice
    Shin ichi Hayashi
    Division of Immunology, Department of Molecular and Cellular Biology, School of Life Science, Faculty of Medicine, Tottori University, 86 Nishi Machi, Yonago, Tottori 683 8503, Japan
    Endocrinology 145:2721-9. 2004
    ..These results indicate that a defect of Src homology 2-domain phosphatase-1 function not only accelerates physiological osteoclast development by RANKL/RANK, but also acquires a novel pathway for osteoclastogenesis by LPS...
  71. ncbi request reprint A new murine model of humoral immuno-deficiency specifically affects class switching to T-independent antigens
    Igor I Kuzin
    Department of Medicine, University of Rochester School of Medicine and Dentistry, Rochester 14625, USA
    Eur J Immunol 34:1807-16. 2004
    ..This new mouse model will be of interest for the understanding of mechanisms involved in TI immune responses and may provide new insights into the molecular basis of human Ig deficiencies...
  72. pmc Humanized mice: are we there yet?
    Francesca Macchiarini
    Division of Allergy, Immunology, and Transplantation, National Institute of Allergy and Infectious Diseases, Bethesda, MD 20892, USA
    J Exp Med 202:1307-11. 2005
    ....
  73. ncbi request reprint TGF-beta1 inhibits T-bet induction by IFN-gamma in murine CD4+ T cells through the protein tyrosine phosphatase Src homology region 2 domain-containing phosphatase-1
    Il Kyoo Park
    Department of Pathology, Dartmouth Medical School, Lebanon, NH 03756, USA
    J Immunol 175:5666-74. 2005
    ..Together, these data show that TGF-beta1 suppresses IFN-gamma signaling and transcriptional responses in CD4+ T cells through the PTP Shp-1...
  74. ncbi request reprint Islet allograft survival induced by costimulation blockade in NOD mice is controlled by allelic variants of Idd3
    Todd Pearson
    The University of Massachusetts Medical School, Worcester, Massachusetts 01605, USA
    Diabetes 53:1972-8. 2004
    ....
  75. ncbi request reprint Purified human hematopoietic stem cells contribute to the generation of cardiomyocytes through cell fusion
    Fumihiko Ishikawa
    Department of Medicine and Biosystemic Science, Kyushu University Graduate School of Medical Sciences, 3 1 1 Maidashi, Higashi ku, Fukuoka 812 8582, Japan
    FASEB J 20:950-2. 2006
    ....
  76. pmc Loss of function of the mouse Sharpin gene results in Peyer's patch regression
    Rosemarie Seymour
    The Jackson Laboratory, Bar Harbor, Maine, United States of America
    PLoS ONE 8:e55224. 2013
    ..These findings indicate that SHARPIN expression is required for the normal development and maintenance, but not initiation, of PP...
  77. pmc Enhanced humoral and HLA-A2-restricted dengue virus-specific T-cell responses in humanized BLT NSG mice
    Smita Jaiswal
    Division of Infectious Diseases and Immunology, University of Massachusetts Medical School, Worcester, MA, USA
    Immunology 136:334-43. 2012
    ..The BLT-NSG mice will allow assessment of human immune responses to DENV vaccines and the effects of previous immunity on subsequent DENV infections...
  78. pmc A deficiency of ceramide biosynthesis causes cerebellar purkinje cell neurodegeneration and lipofuscin accumulation
    Lihong Zhao
    The Jackson Laboratory, Bar Harbor, Maine, United States of America
    PLoS Genet 7:e1002063. 2011
    ....
  79. ncbi request reprint A null mutation in inositol polyphosphate 4-phosphatase type I causes selective neuronal loss in weeble mutant mice
    A Nystuen
    The Jackson Laboratory, 600 Main Street, Bar Harbor, ME 04609, USA
    Neuron 32:203-12. 2001
    ..Although other mutations in phosphoinositide enzymes are associated with synaptic defects without neuronal loss, weeble shows that Inpp4a is critical for the survival of a subset of neurons during postnatal development in mice...
  80. ncbi request reprint Mapping of the flaky skin (fsn) mutation on distal mouse chromosome 17
    S C Pelsue
    Jackson Laboratory, Bar Harbor, Maine 04609 1500, USA
    Mamm Genome 6:758. 1995
  81. ncbi request reprint Lymphadenopathy, elevated serum IgE levels, autoimmunity, and mast cell accumulation in flaky skin mutant mice
    S C Pelsue
    The Jackson Laboratory, Bar Harbor, ME 04609, USA
    Eur J Immunol 28:1379-88. 1998
    ..These marked immunological abnormalities suggest that the fsn locus encodes a major immunoregulatory molecule important in multiple immune and hematopoietic functions...
  82. ncbi request reprint Mutations in the gene encoding the lamin B receptor produce an altered nuclear morphology in granulocytes (Pelger-Huët anomaly)
    Katrin Hoffmann
    Gene Mapping Center, Max Delbrück Center for Molecular Medicine and Franz Volhard Clinic, HELIOS Klinikum, Charite, Humboldt University, Berlin, Germany
    Nat Genet 31:410-4. 2002
    ..Our findings have implications for understanding nuclear envelope-heterochromatin interactions, the pathogenesis of Pelger-like conditions in leukemia, infection and toxic drug reactions, and the evolution of neutrophil nuclear shape...
  83. ncbi request reprint In vivo distribution of human adipose-derived mesenchymal stem cells in novel xenotransplantation models
    Todd E Meyerrose
    Washington University School of Medicine, Division of Oncology, Hematopoietic Development and Malignancy Section, St Louis, Missouri 63110, USA
    Stem Cells 25:220-7. 2007
    ..AMSC are easily obtained and highly amenable to current transduction protocols for retroviral transduction, making them an excellent avenue for cell-based therapies that involve a wide range of end tissue targets...
  84. pmc The developmental program of human dendritic cells is operated independently of conventional myeloid and lymphoid pathways
    Fumihiko Ishikawa
    Research Unit for Human Disease Model, Rikagaku Kenkyusho RIKEN Research Center for Allergy and Immunology, Yokohama, Japan
    Blood 110:3591-660. 2007
    ..We propose that human DCs use unique and flexible developmental programs that cannot be categorized into the conventional myeloid or lymphoid pathway...
  85. ncbi request reprint SHP-1 regulates STAT6 phosphorylation and IL-4-mediated function in a cell type-specific manner
    Zan Huang
    The Department of Cell Biology, Loyola University Chicago, Stritch School of Medicine, Maywood, IL 60153, USA
    Cytokine 29:118-24. 2005
    ..Thus, our data suggest that SHP-1 may regulate IL-4-induced STAT6 phosphorylation and IL-4-mediated functions in a cell type-specific manner...
  86. ncbi request reprint Curly bare (cub), a new mouse mutation on chromosome 11 causing skin and hair abnormalities, and a modifier gene (mcub) on chromosome 5
    Kenneth R Johnson
    The Jackson Laboratory, 600 Main Street, Bar Harbor, ME 04609, USA
    Genomics 81:6-14. 2003
    ..The similarity of the wavy coat phenotype to those of Tgfa and Egfr mutations and the map positions of cub and mcub suggest candidate genes that interact in the EGF receptor signal transduction pathway...
  87. ncbi request reprint Enhanced human CD4+ T cell engraftment in beta2-microglobulin-deficient NOD-scid mice
    S W Christianson
    The Jackson Laboratory, Bar Harbor, ME 04609, USA
    J Immunol 158:3578-86. 1997
    ....
  88. ncbi request reprint Mutations at the murine motheaten locus are within the hematopoietic cell protein-tyrosine phosphatase (Hcph) gene
    L D Shultz
    Jackson Laboratory, Bar Harbor, Maine 04609
    Cell 73:1445-54. 1993
    ..These findings provide the first available animal models for a specific protein-tyrosine phosphatase deficiency, thus facilitating determination of the precise role of this signaling molecule in hematopoiesis...
  89. ncbi request reprint Age-related alterations in the lymphohematopoietic and B-lineage precursor populations in NZB mice
    Zhe Xiong Lian
    Division of Rheumatology Allergy and Clinical Immunology, University of California at Davis, Davis, California 95616, USA
    Stem Cells 20:293-300. 2002
    ....
  90. ncbi request reprint Constant rate of steady-state self-antigen trafficking from skin to regional lymph nodes
    Miya Yoshino
    Division of Immunology, Department of Molecular and Cellular Biology, School of Life Science, Faculty of Medicine, Tottori University, Yonago, 683 8503, Japan
    Int Immunol 18:1541-8. 2006
    ....
  91. ncbi request reprint Treatment and transfer of emphysema by a new bone marrow transplantation method from normal mice to Tsk mice and vice versa
    Yasushi Adachi
    First Department of Pathology, Kansai Medical University, Moriguchi City, Osaka, Japan, 570 0023
    Stem Cells 24:2071-7. 2006
    ..Six months after IBM-BMT, the [Tsk-->C3H] mice showed emphysema. These results strongly suggest that emphysema in Tsk mice originates from defects of stem cells in the bone marrow...
  92. pmc Primed peritoneal B lymphocytes are sufficient to transfer protection against Brugia pahangi infection in mice
    Natalia Paciorkowski
    Department of Pathology, University of Connecticut Health Center, Farmington, Connecticut 06030 3105, USA
    Infect Immun 71:1370-8. 2003
    ..pahangi infection. The data demonstrate a critical role of B lymphocytes in antifilarial immunity in naïve mice and in the memory response in primed mice...
  93. pmc Increased frequency of pre-pro B cells in the bone marrow of New Zealand Black (NZB) mice: implications for a developmental block in B cell differentiation
    Zhe Xiong Lian
    Division of Rheumatology, Allergy and Clinical Immunology, University of California at Davis School of Medicine, 95616, USA
    Dev Immunol 9:35-45. 2002
    ..The decreased frequency of precursor B cells in the Pre-Pro B cell population occurs at the most primitive stage of B cell differentiation...
  94. ncbi request reprint Defective repair of radiation-induced chromosomal damage in scid/scid mice
    J E Disney
    Jackson Laboratory, Bar Harbor, ME 04609
    Cytogenet Cell Genet 59:39-44. 1992
    ..The scid mutation may provide important clues for understanding the relationship between mitotic recombination and DNA repair in higher eukaryotic cells...
  95. ncbi request reprint Gene for ovarian granulosa cell tumor susceptibility, Gct, in SWXJ recombinant inbred strains of mice revealed by dehydroepiandrosterone
    W G Beamer
    Jackson Laboratory, Bar Harbor, Maine 04609
    Cancer Res 48:5092-5. 1988
    ..These data support a heritable basis for GC tumorigenesis in the SWR model involving a small number of genes...
  96. ncbi request reprint Granulosa cell tumorigenesis in genetically hypogonadal-immunodeficient mice grafted with ovaries from tumor-susceptible donors
    W G Beamer
    Jackson Laboratory, Bar Harbor, Maine 04609
    Cancer Res 53:3741-6. 1993
    ..We hypothesize that ovarian androgens synthesized in response to normal gonadotropin stimulation initiate spontaneous tumorigenesis in the genetically susceptible ovary...
  97. ncbi request reprint Genetic mapping of the IL-12 alpha chain gene (Il12a) on mouse chromosome 3
    P A Schweitzer
    The Jackson Laboratory, Bar Harbor, Maine 04609, USA
    Mamm Genome 7:394-5. 1996
  98. ncbi request reprint The interleukin-12 beta subunit (p40) maps to mouse chromosome 11
    N Noben-Trauth
    The Jackson Laboratory, 600 Main Street, Bar Harbor, Maine 04609, USA
    Mamm Genome 7:392. 1996
  99. ncbi request reprint Chemotherapy-resistant human AML stem cells home to and engraft within the bone-marrow endosteal region
    Fumihiko Ishikawa
    Research Unit for Human Disease Models, RIKEN Research Center for Allergy and Immunology, 1 7 22 Suehiro cho Tsurumi ku, Yokohama 230 0045, Japan
    Nat Biotechnol 25:1315-21. 2007
    ..These results indicate the potential utility of this AML xenograft model in the development of novel therapeutic strategies targeted at LS cells...
  100. ncbi request reprint The inner nuclear membrane protein lamin B receptor forms distinct microdomains and links epigenetically marked chromatin to the nuclear envelope
    Dimitra Makatsori
    Laboratory of Biology, The University of Ioannina, School of Medicine, 45 110 Ioannina, Greece
    J Biol Chem 279:25567-73. 2004
    ..The organization of these membrane assemblies is affected significantly in heterozygous ic (ichthyosis) mutants, resulting in a variety of structural abnormalities and nuclear defects...