Lyst

Summary

Gene Symbol: Lyst
Description: lysosomal trafficking regulator
Alias: D13Sfk13, beige, lysosomal-trafficking regulator, CHS1 homolog
Species: mouse

Top Publications

  1. pmc The NIH-3 immunodeficient mouse is a model for Lyme borreliosis myositis and carditis
    D L Defosse
    Department of Microbiology, University of Minnesota Medical School, Minneapolis 55455
    Am J Pathol 141:3-10. 1992
  2. pmc Susceptibility to spontaneous pneumonitis in an inbred strain of beige and satin mice
    P W Lane
    Genetics 72:451-60. 1972
  3. pmc Influence of the lpr environment on the lymph node cell phenotypes in C57BL/6 nubg and nulpr chimeras
    F Tiberghien
    Laboratoire d Immunologie, Universite de Strasbourg, Illkirch, France
    Immunology 83:552-61. 1994
  4. ncbi Mesenchymal dysplasia: a recessive mutation on chromosome 13 of the mouse
    H O Sweet
    Jackson Laboratory, Harbor, Maine 04609 1500, USA
    J Hered 87:87-95. 1996
  5. pmc Homozygous scid/scid;beige/beige mice have low levels of spontaneous or neonatal T cell-induced B cell generation
    D E Mosier
    Division of Immunology, Medical Biology Institute, La Jolla, California 92137
    J Exp Med 177:191-4. 1993
  6. ncbi Identification of the murine beige gene by YAC complementation and positional cloning
    C M Perou
    Department of Pathology, University of Utah School of Medicine, Salt Lake City 84132, USA
    Nat Genet 13:303-8. 1996
  7. ncbi The Beige/Chediak-Higashi syndrome gene encodes a widely expressed cytosolic protein
    C M Perou
    Department of Pathology, University of Utah, Salt Lake City, Utah 84132, USA
    J Biol Chem 272:29790-4. 1997
  8. ncbi 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
  9. pmc Identification of the homologous beige and Chediak-Higashi syndrome genes
    M D Barbosa
    Department of Medicine, University of Florida, Gainesville 32610, USA
    Nature 382:262-5. 1996
  10. pmc Iris phenotypes and pigment dispersion caused by genes influencing pigmentation
    Michael G Anderson
    Department of Molecular Physiology and Biophysics, University of Iowa, Iowa City, IA, USA
    Pigment Cell Melanoma Res 21:565-78. 2008

Research Grants

Scientific Experts

  • Robert Rice
  • Leonard D Shultz
  • Xiaoyan Tang
  • H Witschi
  • G M Winslow
  • S C Hardies
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  • PRISCILLA LI NING YANG
  • Ke Zen
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  • Fabian Runkel
  • Robert Clark
  • Vivian L de Oliveira
  • John T Norton
  • Priyadarshini Chatterjee
  • Rong Fang
  • Jennifer E Lykens
  • Huiwen Zhang
  • Lucius Chiaraviglio
  • Colleen M Trantow
  • Lihui Lu
  • Michael P Verzi
  • Gema Martin-Manso
  • Elizabeth M Pierce
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  • C M Perou
  • M Kveiborg
  • Natalie K Schiller
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  • S F Kingsmore
  • Carrie Rinker-Schaeffer
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Detail Information

Publications109 found, 100 shown here

  1. pmc The NIH-3 immunodeficient mouse is a model for Lyme borreliosis myositis and carditis
    D L Defosse
    Department of Microbiology, University of Minnesota Medical School, Minneapolis 55455
    Am J Pathol 141:3-10. 1992
    ..deficiency due to the nude mutation, these mice have an x-linked defect affecting the B-cell maturation and the beige mutation resulting in the absence of NK cells. NIH-3 mice were susceptible to progressive infection with B...
  2. pmc Susceptibility to spontaneous pneumonitis in an inbred strain of beige and satin mice
    P W Lane
    Genetics 72:451-60. 1972
    Among mice of strain SB/Le, homozygous for the mutant genes beige (bg), satin (sa), and white-bellied agouti (A(w)), 70% developed progressive pneumonitis by 6 months of age...
  3. pmc Influence of the lpr environment on the lymph node cell phenotypes in C57BL/6 nubg and nulpr chimeras
    F Tiberghien
    Laboratoire d Immunologie, Universite de Strasbourg, Illkirch, France
    Immunology 83:552-61. 1994
    ..While nude/beige (nubg) recipients do not allow the development of any lymphoproliferative syndrome, this is variable in nude/lpr (..
  4. ncbi Mesenchymal dysplasia: a recessive mutation on chromosome 13 of the mouse
    H O Sweet
    Jackson Laboratory, Harbor, Maine 04609 1500, USA
    J Hered 87:87-95. 1996
    ..Mesenchymal dysplasia has been mapped to mouse chromosome (Chr) 13. These mapping crosses also confirmed that the Purkinje cell degeneration (pcd) mutation is on Chr 13...
  5. pmc Homozygous scid/scid;beige/beige mice have low levels of spontaneous or neonatal T cell-induced B cell generation
    D E Mosier
    Division of Immunology, Medical Biology Institute, La Jolla, California 92137
    J Exp Med 177:191-4. 1993
    ..We have produced mice homozygous for both the scid and beige mutations. By contrast with C.B-17 scid mice, BALB/c scid.beige mice have a < 2% incidence of "leakiness...
  6. ncbi Identification of the murine beige gene by YAC complementation and positional cloning
    C M Perou
    Department of Pathology, University of Utah School of Medicine, Salt Lake City 84132, USA
    Nat Genet 13:303-8. 1996
    The beige mutation is a murine autosomal recessive disorder, resulting in hypopigmentation, bleeding and immune cell dysfunction...
  7. ncbi The Beige/Chediak-Higashi syndrome gene encodes a widely expressed cytosolic protein
    C M Perou
    Department of Pathology, University of Utah, Salt Lake City, Utah 84132, USA
    J Biol Chem 272:29790-4. 1997
    The human autosomal recessive disorder Chediak-Higashi syndrome and its murine homologue beige are associated with the formation of giant lysosomes that cluster near the perinuclear region of cells...
  8. ncbi 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
    ....
  9. pmc Identification of the homologous beige and Chediak-Higashi syndrome genes
    M D Barbosa
    Department of Medicine, University of Florida, Gainesville 32610, USA
    Nature 382:262-5. 1996
    ..and from the lysosome and late endosome is defective in patients with Chediak-Higashi syndrome (CHS) and in mutant beige (bg) mice...
  10. pmc Iris phenotypes and pigment dispersion caused by genes influencing pigmentation
    Michael G Anderson
    Department of Molecular Physiology and Biophysics, University of Iowa, Iowa City, IA, USA
    Pigment Cell Melanoma Res 21:565-78. 2008
    ..Pigment dispersion was identified in several strains with mutant alleles known to influence melanosomes, including beige, light, and vitiligo...
  11. pmc Lyst mutation in mice recapitulates iris defects of human exfoliation syndrome
    Colleen M Trantow
    Department of Molecular Physiology, University of Iowa, Iowa City, Iowa 52242, USA
    Invest Ophthalmol Vis Sci 50:1205-14. 2009
    ..a distinctive pattern of iris transillumination defects that are recapitulated in Lyst mutant mice carrying the beige allele...
  12. ncbi Genetic and physical delineation of the region overlapping the progressive motor neuropathy (pmn) locus on mouse chromosome 13
    N Martin
    Unite de Genetique des Mammiferes, Institut Pasteur, 25 rue du Docteur Roux, F 75724 Paris Cedex 15, France
    Genomics 75:9-16. 2001
    ....
  13. pmc Defective lysosomal enzyme secretion in kidneys of Chediak-Higashi (beige) mice
    E J Brandt
    J Cell Biol 67:774-88. 1975
    The beige mouse is an animal model for the human Chediak-Higashi syndrome, a disease characterized by giant lysosomes in most cell types...
  14. ncbi The beige mutation in the mouse. I. A stem cell predetermined impairment in natural killer cell function
    J C Roder
    J Immunol 123:2168-73. 1979
    A point mutation, called beige, on linkage group 14 in C57BL/6 mice leads to a marked impairment in natural killing and antibody-dependent, cell-mediated cytolysis (ADCC) of tumor cells...
  15. pmc Comparison of susceptibility of inbred and outbred infant mice to Escherichia coli heat-stable enterotoxin STa
    A Bertin
    Laboratoire de Pathologie Infectieuse et Immunologie, Institut National de la Recherche Agronomique, Centre de Recherches de Tours, Nouzilly, France
    Infect Immun 60:3117-21. 1992
    ..Although the intestine of each strain of mice was able to respond to STa by fluid accumulation, differences in susceptibility of the STa receptor could exist and make DBA mice more resistant to enterotoxigenic E. coli diarrhea...
  16. ncbi Cryptosporidium infections in inbred strains of mice
    F J Enriquez
    Division of Biotechnology, Arizona Health Sciences Center, University of Arizona, Tucson 85724
    J Protozool 38:100S-102S. 1991
    ..and histological sections of the intestine taken on day 7 post-Cryptosporidium inoculation indicated that only the beige mouse (C57BL/6J-bgJ) harbored significant numbers of parasites compared to the other strains...
  17. ncbi A molecular genetic linkage map of mouse chromosome 13 anchored by the beige (bg) and satin (sa) loci
    M J Justice
    Mammalian Genetics Laboratory, BRI Basic Research Program, NCI Frederick Cancer Research Facility, Maryland 21701
    Genomics 6:341-51. 1990
    ..13, Ral, Rrm2-ps3, and Tcrg were determined with respect to the beige (bg) and satin (sa) loci...
  18. ncbi Spontaneous and induced primary oncogenesis in natural killer (NK)-cell-deficient beige mutant mice
    T Haliotis
    Int J Cancer 35:505-13. 1985
    ..These results suggest that a partial NK impairment in beige mutant mice early in life may lead to significantly greater rates of death with spontaneous malignant tumors late ..
  19. ncbi Genetically determined murine models of immunodeficiency
    L D Shultz
    Annu Rev Immunol 5:367-403. 1987
    ..As the gene products of the loci defined by these mutations become known, the information obtained will provide additional insight into mechanisms underlying normal immune function as well as immunologic disease processes in man...
  20. pmc Lysosomal elastase and cathepsin G in beige mice. Neutrophils of beige (Chediak-Higashi) mice selectively lack lysosomal elastase and cathepsin G
    K Takeuchi
    J Exp Med 163:665-77. 1986
    ..of the two lysosomal serine proteinases, elastase, and cathepsin G, was found in neutrophils of four independent beige mutants...
  21. ncbi Granulocyte function in the Chediak-Higashi syndrome of mice
    J I Gallin
    Blood 43:201-6. 1974
  22. ncbi Muted, a new mutant affecting coat colour and otoliths of the mouse, and its position in linkage group XIV
    M F Lyon
    Genet Res 14:163-6. 1969
  23. pmc Increased susceptibility to cytomegalovirus infection in beige mutant mice
    G R Shellam
    Proc Natl Acad Sci U S A 78:5104-8. 1981
    Mice homozygous for the beige gene (bg/bg) are a homologue of the Chédiak-Higashi syndrome of man and are known to be selectively defective in natural killer (NK) cells...
  24. ncbi Adaptive immune defects and delayed rejection of allogeneic tumor cells in beige mice
    G A Carlson
    Cell Immunol 87:348-56. 1984
    The effect of the beige (bg) mutation on adaptive allogeneic tumor rejection was examined by monitoring tumor cell survival in vivo using [131I]iododeoxyuridine-prelabeled cells...
  25. ncbi Platelet storage pool deficiency in mouse pigment mutations associated with seven distinct genetic loci
    E K Novak
    Blood 63:536-44. 1984
    ..Two of these, beige and pale ear, have a bleeding abnormality associated with a deficiency in the number of platelet dense granules...
  26. ncbi Pigment mutations in the mouse which also affect lysosomal functions lead to suppressed natural killer cell activity
    A Orn
    Scand J Immunol 15:305-10. 1982
    ..Whereas the nature of pigmentation disorder was similar in the five mutant strains (beige, pallid, reduced pigmentation, pale ear, and sepia), all mutations except sepia also led to a significant change ..
  27. ncbi Low natural in vivo resistance to syngeneic leukaemias in natural killer-deficient mice
    K Karre
    Nature 284:624-6. 1980
  28. pmc Pulmonary alveolar proteinosis. A spontaneous and inducible disease in immunodeficient germ-free mice
    T Warner
    Department of Pathology, University of Wisconsin Medical School, Madison, USA
    Am J Pathol 146:1017-24. 1995
    ..alveolar proteinosis (PAP), which resembles human PAP, was found in aging (35 to 40 weeks) germ-free SCID-beige (scid/scid-bg/bg) mice. Spontaneous PAP was not observed in germ-free SCID mice...
  29. ncbi Effects of the beige mutation on respiratory tract infection with Pseudomonas aeruginosa in mice
    E Tanaka
    Department of Infection and Inflammation, Kyoto University, Japan
    Exp Lung Res 20:351-66. 1994
    ..aeruginosa mucoid strain developed by the agarose beads method in C57BL/6J(bg/bg) mice (beige mice)...
  30. ncbi Lack of albuminuria in the early heterologous phase of anti-GBM nephritis in beige mice
    G W Feith
    Department of Pathology, University Hospital Nijmegen, The Netherlands
    Kidney Int 43:824-7. 1993
    ..As we have previously shown, this albuminuria does not occur in the beige mutant of the C57BL/6J strain which is deficient for the leukocytic neutral proteinases elastase and cathepsin G...
  31. ncbi Exercise and in vivo natural cytotoxicity against tumour cells of varying metastatic capacity
    L Jadeski
    Department of Health Studies and Gerontology, Faculty of Applied Health Sciences, University of Waterloo, Ontario, Canada
    Clin Exp Metastasis 14:138-44. 1996
    ..fibroblasts (CIRAS 1 and CIRAS 3 cell lines) in mice with (C3H/He-bg2J/+) and without (C3H/HeJ) the beige (bg) mutation which produces impaired natural killer (NK) cell function...
  32. ncbi A scanning electron microscopic investigation of genetic emphysema in tight-skin, pallid, and beige mice, three different C57 BL/6J mutants
    M Keil
    Hoechst AG, Pharma Development Pathology, Frankfurt Main, Germany
    Lab Invest 74:353-62. 1996
    Three mutants of the C57 BL/6J strain, i.e., the tight-skin (Tsk), pallid (pa), and beige (bg) mice have been reported to develop spontaneous emphysema...
  33. ncbi Role of natural killer cells on engraftment of human lymphoid cells and on metastasis of human T-lymphoblastoid leukemia cells in C57BL/6J-scid mice and in C57BL/6J-scid bg mice
    S W Christianson
    Jackson Laboratory, Bar Harbor, Maine
    Cell Immunol 171:186-99. 1996
    ..1-treated as well as in unmanipulated C57BL/6-scid/scid mice. Backcrossing of the beige (bgJ) mutation onto the C57BL/6-scid/scid genetic stock caused decreased NK cell activity accompanied by ..
  34. ncbi The Rab protein family: genetic mapping of six Rab genes in the mouse
    M D Barbosa
    Department of Medicine, University of Florida, Gainesville 32610 0221, USA mba cmg health ufl edu
    Genomics 30:439-44. 1995
    ..Yeast mutations in Rab gene homologs cause defects in vesicular transport similar to those observed in beige (bg) mice...
  35. ncbi Physical mapping of the beige critical region on mouse chromosome 13
    S F Kingsmore
    Department of Medicine, University of Florida, Gainesville, Florida 32610 0221, USA
    Mamm Genome 7:773-5. 1996
  36. ncbi Sak kinase gene structure and transcriptional regulation
    J W Hudson
    Samuel Lunenfeld Research Institute, Mount Sinai Hospital, 600 University Avenue, Toronto, Canada
    Gene 241:65-73. 2000
    ..Using various Sak promoter/luciferase constructs, the core promoter region required for expression was located within 400bp of the message Cap site, and sequence further 5' strongly suppressed transcription...
  37. ncbi A hyperosmotic stress-induced mRNA of carp cell encodes Na(+)- and Cl(-)-dependent high affinity taurine transporter
    K Takeuchi
    Division of Applied Biosciences, Graduate School of Agriculture, Kyoto University, Kitashirakawa Oiwake cho, Sakyo, Kyoto, Japan
    Biochim Biophys Acta 1464:219-30. 2000
    ..The Na(+)/Cl(-)hepatopancreas. Taurine transporter mRNA level increased up to 7.5-fold on raising the ambient osmolality from 300 to 450 mosmol/kgH(2)O. These data suggest the significant role of taurine as an osmolyte in carp cells...
  38. ncbi Inflammation in atherosclerosis: lesion formation in LDL receptor-deficient mice with perforin and Lyst(beige) mutations
    Natalie K Schiller
    Scripps Research Institute, Department of Immunology, La Jolla, Calif, USA
    Arterioscler Thromb Vasc Biol 22:1341-6. 2002
    ..cytolysis in atherosclerosis, we generated C57Bl/6 double-mutant mice by crossing LDLr-/- mice with NK cell-defective Lyst(beige) mice (creating beige,LDLr-/- mice) and with perforin-deficient mice (creating Pfp-/-,LDLr-/- mice).
  39. ncbi A missense mutation in the WD40 domain of murine Lyst is linked to severe progressive Purkinje cell degeneration
    Martina Rudelius
    Division of Neuropathology, Institute of Pathology, Technical University of Munich, Ismaninger Strasse 22, 81675 Munich, Germany
    Acta Neuropathol 112:267-76. 2006
    ..ENU) mutagenesis screen, we identified the first murine missense mutation in the lysosomal trafficking regulator gene (Lyst(Ing3618)) located at a highly conserved position in the WD40 protein domain...
  40. pmc Thrombospondin 1 promotes tumor macrophage recruitment and enhances tumor cell cytotoxicity of differentiated U937 cells
    Gema Martin-Manso
    Laboratory of Pathology, Center for Cancer Research, National Cancer Institute, NIH, Bethesda, Maryland 20892, USA
    Cancer Res 68:7090-9. 2008
    ..overexpression of TSP1 in melanoma cells increases macrophage recruitment into xenograft tumors grown in nude or beige/nude mice...
  41. pmc Immune effectors required for hepatitis B virus clearance
    Priscilla L Yang
    Department of Immunology and Microbial Science, The Scripps Research Institute, La Jolla, CA 92037, USA
    Proc Natl Acad Sci U S A 107:798-802. 2010
    ..These results provide insight into the complexity of the systems involved in HBV clearance, and they suggest unique directions for analysis of the mechanism(s) responsible for HBV persistence...
  42. pmc Perforin is a critical physiologic regulator of T-cell activation
    Jennifer E Lykens
    Division of Immunobiology, Department of Pediatrics, Cincinnati Children s Hospital Medical Center and University of Cincinnati College of Medicine, Cincinnati, OH, USA
    Blood 118:618-26. 2011
    ..We conclude that perforin-dependent cytotoxicity has an immunoregulatory role that is distinguishable from its pathogen clearance function and limits T-cell activation in the physiologic context by suppressing antigen presentation...
  43. doi Modulation of antigen presentation and B cell receptor signaling in B cells of beige mice
    Priyadarshini Chatterjee
    National Institute of Immunology, New Delhi 110067, India
    J Immunol 188:2695-702. 2012
    ..We have used the beige (Bg) mouse, which is deficient in endosome biogenesis, to evaluate the effect of potentially altered Ag ..
  44. pmc Humanized mouse model of skin inflammation is characterized by disturbed keratinocyte differentiation and influx of IL-17A producing T cells
    Vivian L de Oliveira
    Laboratory of Medical Immunology, Department of Laboratory Medicine, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands
    PLoS ONE 7:e45509. 2012
    ..This combined approach will gain our understanding of the dermal immunopathology in humans and benefit the development of novel therapeutics for controlling inflammatory skin diseases...
  45. pmc Differentiating inbred mouse strains from each other and those with single gene mutations using hair proteomics
    Robert H Rice
    Department of Environmental Toxicology and Forensic Science Graduate Program, University of California Davis, Davis, California, USA
    PLoS ONE 7:e51956. 2012
    ....
  46. pmc Cutting edge: nitrogen bisphosphonate-induced inflammation is dependent upon mast cells and IL-1
    John T Norton
    Moores Cancer Center, University of California San Diego, La Jolla, CA 92093, USA
    J Immunol 188:2977-80. 2012
    ..These results document the critical role of mast cells and IL-1 in NBP-mediated inflammatory reactions...
  47. pmc Contribution of NK cells to the innate phase of host protection against an intracellular bacterium targeting systemic endothelium
    Rong Fang
    Department of Pathology, University of Texas Medical Branch, Galveston, TX 77555 0609, USA
    Am J Pathol 181:185-95. 2012
    ..Furthermore, NK cells are involved in preventing rickettsial infection-induced endothelial cell damage, possibly via perforin production...
  48. pmc Milky spots promote ovarian cancer metastatic colonization of peritoneal adipose in experimental models
    Robert Clark
    Department of Pathology Molecular Pathogenesis and Molecular Medicine, The University of Chicago, Chicago, Illinois 60637, USA
    Am J Pathol 183:576-91. 2013
    ..Our findings support a two-step model in which both milky spots and adipose have specific roles in colonization of the omentum by ovarian cancer cells. ..
  49. ncbi Pulmonary surface-active materials in the Chediak-Higashi syndrome
    J L Prueitt
    J Lipid Res 19:410-5. 1978
    b>Beige mice express the Chediak-Higashi syndrome. Large inclusions, identified as abnormal lysosomes, are found in many cells...
  50. ncbi Formation of anomalous lysosomes in monocytes, neutrophils, and eosinophils from bone marrow of mice with Chédiak-Higashi syndrome
    C Oliver
    Lab Invest 32:17-27. 1975
    Chédiak-Higashi syndrome, a rare autosomal recessive disorder occurring in man, beige mice, and other homologues, is characterized by abnormally large lysosomes in many cell types...
  51. ncbi Lymphoblastic lymphoma in a colony of N:NIH(S)-bg-nu-xid mice
    K S Waggie
    Veterinary Resources Program, National Center for Research Resources, National Institutes of Health, Bethesda, MD 20892
    Lab Anim Sci 42:375-7. 1992
    ..All of the above mice were homo- or hemizygous for the beige and X-linked immunodeficiency mutations. The average age of the mice was 8.3 months...
  52. ncbi Soluble beta-galactoside specific lectin is developmentally regulated in lungs of neonatal black mice and beige mice
    P L Whitney
    Pulmonary Research Center, Calvin and Flavia Oak Asthma Research and Treatment Facility, University of Miami School of Medicine, Florida 33101
    Exp Lung Res 18:553-61. 1992
    The beige mouse, a mutant of the C57 black mouse, is best known as a model of the Chediak-Higashi syndrome. Recently, it was found that alveolar maturation in neonatal beige mice is impaired, resulting in abnormally large alveoli...
  53. ncbi Cytoarchitectonic abnormalities in cerebellum and hippocampal formation of beige mutant mouse
    H Guo
    Department of Anatomy, Tokai University School of Medicine, Kanagawa, Japan
    Tokai J Exp Clin Med 17:53-61. 1992
    Abnormalities in the cerebellum and hippocampal formation of the beige mouse were revealed by histological and immunohistochemical examination...
  54. pmc Adoptive transfer of the generalized lymphoproliferative disease (gld) syndrome in nude beige mice
    S Froidevaux
    Laboratoire d Immunologie, Universite Louis Pasteur, Strasbourg, France
    Immunology 75:693-9. 1992
    C57BL/6 nude beige mice (B6 nubg) were used as recipients for the transfer of haematopoietic cells from either B6 wild as control mice, or systemic lupus erythematous B6 mice homozygous for the recessive generalized lymphadenopathy ..
  55. ncbi Nidogen/entactin (Nid) maps to the proximal end of mouse chromosome 13 linked to beige (bg) and identifies a new region of homology between mouse and human chromosomes
    N A Jenkins
    ABL Basic Research Program, NCI Frederick Cancer Research and Development Center, Frederick, Maryland 21702
    Genomics 9:401-3. 1991
  56. ncbi Linkage of loci associated with two pigment mutations on mouse chromosome 13
    R F Holcombe
    Department of Medicine, LSU Medical Center, Shreveport 71130
    Genet Res 58:41-50. 1991
    ..strains of mice were typed to map multiple markers in relation to two pigment mutations on mouse chromosome 13, beige (bg) and pearl (pe)...
  57. ncbi The beige mouse: role of neutrophil elastase in the development of pulmonary emphysema
    B Starcher
    Department of Biochemistry, University of Texas Health Center, Tyler 75710
    Exp Lung Res 15:785-800. 1989
    Mutant beige mice and normal C57 black mice were given endotoxin intratracheally (IT) once a week for 10 weeks...
  58. pmc Genetics of resistance to infection with Candida albicans in mice
    G Marquis
    Department of Microbiology and Immunology, Universite de Montreal, Quebec, Canada
    Br J Exp Pathol 69:651-60. 1988
    To determine differences in susceptibility, 234 naive mice including xid and beige mutants were infected intravenously with Candida albicans and monitored with survival analysis and quantitative culture of the kidneys...
  59. ncbi Correction of symptoms of platelet storage pool deficiency in animal models for Chediak-Higashi syndrome and Hermansky-Pudlak syndrome
    E K Novak
    Blood 66:1196-201. 1985
    ..Reciprocal bone marrow transplants were carried out between normal C57BL/6J mice and two of these mutants, beige and pallid, in order to test whether the platelet defects are due to a defect in platelet progenitor cells or to ..
  60. ncbi Lysosomal mutations inhibit lipofuscinosis of the spleen in C57BL mice
    F Ahmed
    J Hered 76:311-2. 1985
    b>Beige, bg, and reduced pigmentation, rp, are recessive mutations affecting lysosomal function. Homozygosity for beige prevented lipofuscinosis of the spleen in C57BL mice and its incidence was greatly reduced by homozygosity for rp...
  61. pmc Assignment of the gene for adenine phosphoribosyltransferase to human chromosome 16 by mouse-human somatic cell hybridization
    J A Tischfield
    Proc Natl Acad Sci U S A 71:45-9. 1974
    ..This observation suggests that the human aprt gene can be assigned to chromosome 16...
  62. pmc Infection and immunoglobulin concentrations in Chediak-Higashi mice
    R J Elin
    Infect Immun 10:88-91. 1974
    ..CHS mice have an increased susceptibility to pyogenic infections, which is not due to immunoglobulin deficiency. These mice may provide a useful laboratory model for the study of increased susceptibility to infection...
  63. ncbi The fine structure of melanogenesis in coat color mutants of the mouse
    V J Hearing
    J Ultrastruct Res 43:88-106. 1973
  64. ncbi Distribution of anomalous lysosomes in the beige mouse: a homologue of Chediak-Higashi syndrome
    C Oliver
    J Histochem Cytochem 21:218-28. 1973
  65. ncbi Giant granules in leukocytes of the beige mouse
    M A Lutzner
    J Hered 58:299-300. 1967
  66. pmc Leishmaniasis in beige mice
    C E Kirkpatrick
    Infect Immun 38:1208-16. 1982
    ..One group of mice was homozygous recessive for the beige gene (bg/bg)...
  67. ncbi Mutations in mice that influence natural killer (NK) cell activity
    E A Clark
    Immunogenetics 12:601-13. 1981
    ..Mutations at six loci that reduce NK-cell activity in the homozygous state were identified, including beige (bg), hairless (hr), motheaten (me), obese (ob), steel (Sl) and, to lesser extent, dominant spotting (W)...
  68. ncbi Role of NK cells in tumour growth and metastasis in beige mice
    J E Talmadge
    Nature 284:622-4. 1980
    ..Recently the beige (bg) mouse, a morphological homologue of the human Chediak-Higashi (CH) syndrome, was shown to be deficient in NK ..
  69. ncbi Signs and lesions of experimental Sendai virus infection in two genetically distinct strains of SCID/beige mice
    D H Percy
    Department of Pathology, University of Guelph, Ontario, Canada
    Vet Pathol 31:67-73. 1994
    ..bg/bg (SCID-beige) using C...
  70. ncbi Spontaneous and experimental infections in scid and scid/beige mice
    D H Percy
    Department of Pathology, Ontario Veterinary College, University of Guelph, Canada
    Lab Anim Sci 43:127-32. 1993
    ..In addition, based on observations after experimental or naturally occurring viral infections, scid and scid/beige mice have been shown to be very susceptible to infections with viruses such as mouse hepatitis virus, Sendai virus,..
  71. ncbi Role of x-linked immunodeficiency (xid) and NK activity in rejection of human tumor xenotransplants in nude mice
    K Maruo
    Central Institute for Experimental Animals, Kanagawa, Japan
    APMIS 101:345-52. 1993
    ..The nude mice strains tested were classified as follows by expression of the beige gene and the x-linked immunodeficiency (xid) gene: 1) high NK nude (C57BL/6N, nu/nu), 2) low NK nude (C57BL/6 bg/..
  72. ncbi Studies of virus-induced myocardial injury in mice: value of the scid mutation on different genetic backgrounds and combined with other mutations
    L H Chow
    Department of Medicine, University of Western Ontario, London, Canada
    Lab Anim Sci 43:133-5. 1993
    ..The combined use of severe combined immunodeficient (scid) and doubly mutant scid/beige mice of different genetic backgrounds has provided a unique opportunity to study the consequences of viral ..
  73. ncbi Effects of in vivo morphine treatment on antibody responses in C57BL/6 bgJ/bgJ (beige) mice
    J L Bussiere
    Department of Microbiology Immunology, Temple University School of Medicine, Philadelphia, PA 19140
    Life Sci 52:PL43-8. 1993
    C57BL/6J bgJ/bgJ (beige) mice are less sensitive than other strains to the analgesic effects of morphine, although they have normal numbers of mu receptors...
  74. ncbi Sensitized T lymphocytes render DBA/2 beige mice responsive to IFN alpha/beta therapy of Friend erythroleukemia visceral metastases
    T Kaido
    Laboratory of Viral Oncology, Institut de Recherches Scientifiques sur le Cancer, Villejuif, France
    Int J Cancer 54:475-81. 1993
    ..v.) with FLC, but does not protect FLC-injected DBA/2 beige (bg/bg) mice...
  75. pmc Homozygosity mapping of the gene for Chediak-Higashi syndrome to chromosome 1q42-q44 in a segment of conserved synteny that includes the mouse beige locus (bg)
    K Fukai
    Department of Medical Genetics, University of Wisconsin Medical School, Madison, USA
    Am J Hum Genet 59:620-4. 1996
    ..Similar ultrastructural abnormalities occur in the beige mouse, which thus has been suggested to be homologous to human CHS...
  76. ncbi Isolation and sequence determination of cDNA encoding mouse rab 4 and candidate approach for the beige mutation in mice
    H Ikeda
    Department of Pediatrics, Yamagata University School of Medicine, Japan
    Biochem Mol Biol Int 40:647-51. 1996
    ..The deduced amino acid sequence is highly homologous to rab 4 from rat and human. We analyzed rab 4 as a candidate gene of the beige mouse, but we could not find any change in the sequence of the coding region of rab 4 mRNA.
  77. pmc Protective immunity to HIV-1 in SCID/beige mice reconstituted with peripheral blood lymphocytes of exposed but uninfected individuals
    C Zhang
    Department of Medical Microbiology and Immunology, University of Alberta, Edmonton, Canada
    Proc Natl Acad Sci U S A 93:14720-5. 1996
    ..we have established a humanized SCID mouse infection model whereby T-, B-, and natural killer-cell defective SCID/beige mice that have been reconstituted with normal human PBLs can be infected with HIV-1...
  78. ncbi The physical and genetic map surrounding the Lyst gene on mouse chromosome 13
    D J Misumi
    Millennium Pharmaceuticals, Inc, Cambridge, Massachusetts 02139, USA
    Genomics 40:147-50. 1997
    During the recent cloning of the mouse Lyst gene we developed both a high-resolution genetic map and a complete YAC and BAC contig of the Lyst critical region on mouse Chromosome 13...
  79. ncbi Giant renin secretory granules in beige mouse renal afferent arterioles
    B L Jensen
    Department of Physiology, University of Odense, Winslowparken 19, DK 5000 Odense, Denmark
    Cell Tissue Res 288:399-406. 1997
    The mutant beige mouse (C57BL/6 bg) has a disease characterised by abnormally enlarged cytoplasmic granules in a variety of cells...
  80. ncbi The effect of lysosomotropic amines on beige mouse cells
    S G Davis
    Department of Biology, Clark University, Worcester, Massachusetts 01610, USA
    Exp Cell Res 237:242-5. 1997
    The beige mutant in the mouse is characterized by enlarged lysosomes in many cell types due to increased fluidity of cellular membranes and organelle fusion...
  81. ncbi Genetic defects in Chediak-Higashi syndrome and the beige mouse
    R A Spritz
    Department of Medical Genetics, University of Wisconsin, Madison 53706, USA
    J Clin Immunol 18:97-105. 1998
    ..Disorders similar to human CHS occur in many mammalian species, the most important being the beige mouse, long considered a likely homologue of human CHS...
  82. pmc Infection of the laboratory mouse with the intracellular pathogen Ehrlichia chaffeensis
    G M Winslow
    Wadsworth Center, New York State Department of Health, Albany, New York 12201 2002, USA
    Infect Immun 66:3892-9. 1998
    ..B-17 and C57BL/6) mice cleared the bacteria within 10 days, but immunocompromised SCID and SCID/BEIGE mice developed persistent infection in the spleen, liver, peritoneal cavity, brain, lung, and bone marrow and ..
  83. ncbi A comparison of UVB-carcinogenesis between nude mice and nude beige mice
    Y Ishigaki
    Department of Radiation Biology, Faculty of Pharmaceutical Sciences, Kanazawa University, Japan
    J Radiat Res 39:119-27. 1998
    ..Our results suggest that NK activity plays a protective role against UVB-carcinogenesis from low daily-doses of UVB-irradiation...
  84. ncbi Enhanced human tumor cell transplantability in a new congenic immunodeficient mouse; KSN-BNX
    Y Ishigaki
    Department of Radiation Biology, Faculty of Pharmaceutical Sciences, Kanazawa University, Japan
    Folia Microbiol (Praha) 43:493-4. 1998
    We introduced two mutant genes (beige; bg that induces the deficiency of natural killer (NK) activity and xid that decreases the production of immunoglobulin) into KSN nude mice with high reproductive performances...
  85. ncbi Mechanisms of the antimetastatic effect in the liver and of the hepatocyte injury induced by alpha-galactosylceramide in mice
    R Nakagawa
    Pharmaceutical Research Laboratory, Kirin Brewery Company, Takasaki, Japan
    J Immunol 166:6578-84. 2001
    ..serum IFN-gamma concentration and alanine aminotransferase activity in NK cell-depleted C57BL/6 (B6) mice and B6-beige/beige mice similarly to its effects in control B6 mice, its enhancement of the antitumor cytotoxicity of liver ..
  86. ncbi Gene defect in ectodermal dysplasia implicates a death domain adapter in development
    D J Headon
    Department of Molecular and Cellular Biology, Baylor College of Medicine, Houston, Texas, 77030, USA
    Nature 414:913-6. 2001
    ..Our findings show that the death receptor/adapter signalling mechanism is conserved in developmental, as well as apoptotic, signalling...
  87. ncbi Mycobacterium avium subspecies paratuberculosis triggers intestinal pathophysiologic changes in beige/scid mice
    G K Mutwiri
    Department of Pathobiology, University of Guelph, Ontario, Canada
    Comp Med 51:538-44. 2001
    We investigated whether infection of beige/scid mice with Mycobacterium avium subspecies paratuberculosis can induce intestinal pathophysiologic changes. Six-week-old beige/scid mice were inoculated intraperitoneally with M...
  88. ncbi The increased bone mass in deltaFosB transgenic mice is independent of circulating leptin levels
    M Kveiborg
    Department of Cell Biology, Yale University School of Medicine, New Haven, Connecticut 06510, USA
    Endocrinology 143:4304-9. 2002
    ..This demonstration that restoring circulating levels of leptin in deltaFosB transgenic mice failed to rescue the bone phenotype further indicates that the marked increase in bone formation is autonomous to the osteoblast lineage...
  89. ncbi Slate--a new coat color mutant in the mouse
    L J Pierro
    J Hered 54:47-50. 1963
  90. ncbi Mechanisms of host defense following severe acute respiratory syndrome-coronavirus (SARS-CoV) pulmonary infection of mice
    William G Glass
    Laboratory of Host Defenses, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD 20892, USA
    J Immunol 173:4030-9. 2004
    ..Moreover, beige, CD1-/-, and RAG1-/- mice cleared the virus normally...
  91. ncbi Lung pathology of pale ear mouse (model of Hermansky-Pudlak syndrome 1) and beige mouse (model of Chediak-Higashi syndrome): severity of giant lamellar body degeneration of type II pneumocytes correlates with interstitial inflammation
    Xiaoyan Tang
    Department of Pathology, Tokyo Hospital, Tokai University School of Medicine, Tokyo, Japan
    Pathol Int 55:137-43. 2005
    ..of the present paper was to investigate the lung pathology of pale ear (ep) mouse, a mouse model of HPS1, and of beige (bg) mouse, a mouse model of Chediak-Higashi syndrome (CHS) with a reference to GLBD and associated pathologic ..
  92. ncbi Grey, a novel mutation in the murine Lyst gene, causes the beige phenotype by skipping of exon 25
    Fabian Runkel
    Anatomisches Institut, Universitat Bonn, Germany
    Mamm Genome 17:203-10. 2006
    ..mutant phenotype and the human Chediak-Higashi syndrome are caused by mutations in the murine Lyst (lysosomal trafficking regulator) gene and the human CHS gene, respectively...
  93. pmc Therapeutic targeting of CC ligand 21 or CC chemokine receptor 7 abrogates pulmonary fibrosis induced by the adoptive transfer of human pulmonary fibroblasts to immunodeficient mice
    Elizabeth M Pierce
    Associate Professor, Immunology Program, Department of Pathology, University of Michigan Medical School, Room 4057, BSRB, 109 Zina Pitcher Pl, Ann Arbor, MI 48109 0602, USA
    Am J Pathol 170:1152-64. 2007
    ..B-17 severe combined immunodeficiency (SCID)/beige (bg) mice...
  94. pmc Transcription factor foxq1 controls mucin gene expression and granule content in mouse stomach surface mucous cells
    Michael P Verzi
    Dana Farber Cancer Institute and Department of Medicine, Boston, Massachusetts, USA
    Gastroenterology 135:591-600. 2008
    ..We identified stomach-restricted expression of the forkhead transcription factor FOXQ1...
  95. doi ALK mutants in the kinase domain exhibit altered kinase activity and differential sensitivity to small molecule ALK inhibitors
    Lihui Lu
    Worldwide Discovery Research, Cephalon Inc, West Chester, Pennsylvania 19380, USA
    Biochemistry 48:3600-9. 2009
    ..Further characterization of these mutants may help to identify and develop potent ALK inhibitors active against both WT and resistant mutants of ALK...
  96. doi Identification of proteins in the ceroid-like autofluorescent aggregates from liver lysosomes of Beige, a mouse model for human Chediak-Higashi syndrome
    Huiwen Zhang
    Research Institute, The Hospital for Sick Children, Toronto, Canada
    Mol Genet Metab 99:389-95. 2010
    ..In this study, we isolated an autofluorescent ceroid-like aggregate from purified Beige mouse liver lysosomes and analyzed the composition of the aggregate by ion trap mass-spectrometry...
  97. pmc An immunocompromised murine model of chronic Bartonella infection
    Lucius Chiaraviglio
    Department of Pathology, Beth Israel Deaconess Medical Center, 330 Brookline Ave, Boston, MA 02215, USA
    Am J Pathol 176:2753-63. 2010
    ..Here, we demonstrate the ability of Bartonella taylorii to cause chronic infection in SCID/BEIGE mice...
  98. pmc Cleavage of the CD11b extracellular domain by the leukocyte serprocidins is critical for neutrophil detachment during chemotaxis
    Ke Zen
    Nanjing University, Jiangsu, China
    Blood 117:4885-94. 2011
    ..CD11b cleavage and PMN detachment and chemotaxis, were impaired in elastase/cathepsin G-deficient Beige mice; this defect could be restored by the addition of extracellular elastase...
  99. ncbi Light-induced alterations of retinal pigment epithelium in black, albino, and beige mice
    W G Robison
    Exp Eye Res 22:549-57. 1976
  100. ncbi Lysosomal mutations increase susceptibility to anaesthetics
    F Ahmed
    Department of Biology, University of Essex, Colchester, England
    Experientia 45:1133-5. 1989
    ..The two recessive mutations studied were beige (bg) and reduced pigmentation (rp)...

Research Grants8

  1. MOUSE MODELS FOR HUMAN CANCER
    William Dove; Fiscal Year: 2003
    ..abstract_text> ..
  2. Chemoprevention in Mice after Tobacco Smoke Exposure
    Hanspeter Witschi; Fiscal Year: 2004
    ..Eventually, these preclinical studies in an animal model of "former smokers" will help to evaluate the potential usefulness and also possible dosage regimens of chemopreventive agents characterized by their suppressing action. ..
  3. Antibody mediated intracellular bacterial elimination
    Gary Winslow; Fiscal Year: 2006
    ....
  4. Atheroprotection with Anti-angiogenic DNA Vaccines
    RAMONA PETROVAN; Fiscal Year: 2006
    ..Achievement of this proposal's objectives should lead to the development of highly efficient anti-atherosclerotic therapies that will greatly improve future treatment choices for atherosclerosis. ..
  5. Development of Cellular Assays of Dengue Virus Infection
    Priscilla Yang; Fiscal Year: 2008
    ..These inhibitors can be used to study how dengue virus interacts with its host, and they may also be useful in developing therapies for the treatment of dengue virus infections. [unreadable] [unreadable] [unreadable]..
  6. GENES CONTROLLING NEOPLASIA OF THE INTESTINAL EPITHELIUM
    William Dove; Fiscal Year: 2008
    ..unreadable] Aim 5e: Analyze two new polymorphic modifier loci linked to the Apc locus on mouse chromosome 18 - a recessive enhancing allele in the strain BTBR, and a semidominant resistance allele in the strain DBN2.[unreadable]..
  7. Regulation of Recall Responses During Ehrlichiosis
    Gary M Winslow; Fiscal Year: 2010
    ..By defining optimal CD4 T cell immune correlates, and the role of memory CD8 T cells during recall responses, the proposed studies will contribute to the development of therapies and vaccines for ehrlichioses and related infections. ..