JAGGED1

Summary

Gene Symbol: JAGGED1
Description: jagged 1
Alias: AGS, AGS1, AHD, AWS, CD339, HJ1, JAGL1, protein jagged-1
Species: human

Top Publications

  1. ncbi A molecular biologic approach to study the fine specificity of antibodies directed to the MN human blood group antigens
    D P Blackall
    Department of Pathology, University of Pennsylvania, Philadelphia 19104
    J Immunol 152:2241-7. 1994
  2. ncbi The human homolog of rat Jagged1 expressed by marrow stroma inhibits differentiation of 32D cells through interaction with Notch1
    L Li
    Stower Institute for Medical Research, Department of Molecular Biotechnology, University of Washington, Seattle 98195, USA
    Immunity 8:43-55. 1998
  3. ncbi The DSL domain in mutant JAG1 ligand is essential for the severity of the liver defect in Alagille syndrome
    Z R Yuan
    National Children s Medical Research Center, Tokyo, Japan
    Clin Genet 59:330-7. 2001
  4. ncbi Clinical double-blind trial on the dermal tolerance and user acceptability of six alcohol-based hand disinfectants for hygienic hand disinfection
    A Kramer
    Institut fur Hygiene und Umweltmedizin, Ernst Moritz Arndt Universitat Greifswald, Hainstr 26, Germany
    J Hosp Infect 51:114-20. 2002
  5. ncbi Exon 6 of human Jagged-1 encodes an autonomously folding unit
    Corrado Guarnaccia
    International Centre for Genetic Engineering and Biotechnology ICGEB, Protein Structure and Bioinformatics Group, Area Science Park, Padriciano 99, I 34012 Trieste, Italy
    FEBS Lett 574:156-60. 2004
  6. ncbi Mutations in the human Jagged1 gene are responsible for Alagille syndrome
    T Oda
    Laboratory of Gene Transfer, National Human Genome Research Institutes of Health, Bethesda, Maryland 20892 4442, USA
    Nat Genet 16:235-42. 1997
  7. pmc Targeting the notch ligand JAGGED1 in both tumor cells and stroma in ovarian cancer
    Adam D Steg
    Department of Obstetrics and Gynecology, University of Alabama at Birmingham, Birmingham, Alabama, USA
    Clin Cancer Res 17:5674-85. 2011
  8. doi Immunohistochemical analysis of NOTCH1 and JAGGED1 expression in multiple myeloma and monoclonal gammopathy of undetermined significance
    Anita Skrtic
    Department of Pathology and Cytology, University of Zagreb, School of Medicine, University Hospital Merkur, Zagreb, Croatia
    Hum Pathol 41:1702-10. 2010
  9. ncbi Twelve novel JAG1 gene mutations in Polish Alagille syndrome patients
    Dorota Jurkiewicz
    Children s Memorial Health Institute, Department of Medical Genetics, Warsaw, Poland
    Hum Mutat 25:321. 2005
  10. ncbi Mutation analysis of Jagged1 (JAG1) in Alagille syndrome patients
    R P Colliton
    Division of Human Genetics, The Children s Hospital of Philadelphia, Philadelphia, PA 19104, USA
    Hum Mutat 17:151-2. 2001

Detail Information

Publications363 found, 100 shown here

  1. ncbi A molecular biologic approach to study the fine specificity of antibodies directed to the MN human blood group antigens
    D P Blackall
    Department of Pathology, University of Pennsylvania, Philadelphia 19104
    J Immunol 152:2241-7. 1994
    The human MN blood group Ags on glycophorin A are linear complex glycopeptide Ags determined by a combination of amino acid polymorphisms and O-glycans. M Ag has Ser and Gly, and N Ag has Leu and Glu, at positions 1 and 5, respectively...
  2. ncbi The human homolog of rat Jagged1 expressed by marrow stroma inhibits differentiation of 32D cells through interaction with Notch1
    L Li
    Stower Institute for Medical Research, Department of Molecular Biotechnology, University of Washington, Seattle 98195, USA
    Immunity 8:43-55. 1998
    A cDNA clone encoding the human homolog of rat Jagged1 was isolated from normal human marrow...
  3. ncbi The DSL domain in mutant JAG1 ligand is essential for the severity of the liver defect in Alagille syndrome
    Z R Yuan
    National Children s Medical Research Center, Tokyo, Japan
    Clin Genet 59:330-7. 2001
    Alagille syndrome (AGS) is a congenital multi-system anomaly mainly characterized by paucity of intrahepatic bile ducts caused by haploinsufficiency of the Jagged 1 gene (JAG1)...
  4. ncbi Clinical double-blind trial on the dermal tolerance and user acceptability of six alcohol-based hand disinfectants for hygienic hand disinfection
    A Kramer
    Institut fur Hygiene und Umweltmedizin, Ernst Moritz Arndt Universitat Greifswald, Hainstr 26, Germany
    J Hosp Infect 51:114-20. 2002
    Six commercially available alcohol-based hand rubs [AHD 2000, Desderman, Mucasept A, Manorapid (Poly-Alkohol, Spitacid, and Sterillium] were investigated in a clinical double-blind trial involving 10 participants who had no previous ..
  5. ncbi Exon 6 of human Jagged-1 encodes an autonomously folding unit
    Corrado Guarnaccia
    International Centre for Genetic Engineering and Biotechnology ICGEB, Protein Structure and Bioinformatics Group, Area Science Park, Padriciano 99, I 34012 Trieste, Italy
    FEBS Lett 574:156-60. 2004
    ..We show that it is structured in solution, as suggested by circular dichroism and NMR spectroscopy, and displays an EGF-like disulfide bond topology, as determined by disulfide mapping...
  6. ncbi Mutations in the human Jagged1 gene are responsible for Alagille syndrome
    T Oda
    Laboratory of Gene Transfer, National Human Genome Research Institutes of Health, Bethesda, Maryland 20892 4442, USA
    Nat Genet 16:235-42. 1997
    ..Within this region we identified JAG1, the human homologue of rat Jagged1, which encodes a ligand for the Notch receptor...
  7. pmc Targeting the notch ligand JAGGED1 in both tumor cells and stroma in ovarian cancer
    Adam D Steg
    Department of Obstetrics and Gynecology, University of Alabama at Birmingham, Birmingham, Alabama, USA
    Clin Cancer Res 17:5674-85. 2011
    b>Jagged1, a Notch ligand, is expressed on both tumor epithelial and endothelial cells and therefore may be amenable to dual targeting of the tumor stroma and malignant cell compartments of the tumor microenvironment.
  8. doi Immunohistochemical analysis of NOTCH1 and JAGGED1 expression in multiple myeloma and monoclonal gammopathy of undetermined significance
    Anita Skrtic
    Department of Pathology and Cytology, University of Zagreb, School of Medicine, University Hospital Merkur, Zagreb, Croatia
    Hum Pathol 41:1702-10. 2010
    ..is implicated in the pathogenesis of multiple myeloma expressing high level of active Notch proteins NOTCH1 and JAGGED1 in tumor plasma cells...
  9. ncbi Twelve novel JAG1 gene mutations in Polish Alagille syndrome patients
    Dorota Jurkiewicz
    Children s Memorial Health Institute, Department of Medical Genetics, Warsaw, Poland
    Hum Mutat 25:321. 2005
    Alagille syndrome (AGS) is an autosomal dominant disorder with developmental abnormalities of the liver, heart, eyes, vertebrae, and face...
  10. ncbi Mutation analysis of Jagged1 (JAG1) in Alagille syndrome patients
    R P Colliton
    Division of Human Genetics, The Children s Hospital of Philadelphia, Philadelphia, PA 19104, USA
    Hum Mutat 17:151-2. 2001
    Alagille syndrome (AGS) is an autosomal dominant disorder caused by mutations in Jagged1 (JAG1), a ligand in the evolutionarily conserved Notch signaling pathway...
  11. ncbi DHPLC mutation analysis of Jagged1 (JAG1) reveals six novel mutations in Australian alagille syndrome patients
    Mandy L Heritage
    Joint Clinical Sciences Program, The Queensland Institute of Medical Research and The University of Queensland, Brisbane, Queensland, Australia
    Hum Mutat 20:481. 2002
    ..Mutations in the Jagged1 gene have been found to result in the AGS phenotype...
  12. pmc Jagged-1 and Notch3 juxtacrine loop regulates ovarian tumor growth and adhesion
    Jung Hye Choi
    Department of Pathology, Johns Hopkins Medical Institutions, Baltimore, MD 21231, USA
    Cancer Res 68:5716-23. 2008
    ....
  13. doi Notch-3 and Notch-4 signaling rescue from apoptosis human B-ALL cells in contact with human bone marrow-derived mesenchymal stromal cells
    Armel Hervé Nwabo Kamdje
    Stem Cell Research Laboratory, Section of Hematology, Department of Medicine, University of Verona, Verona, Italy
    Blood 118:380-9. 2011
    ..Our data suggest that the stromal cell-mediated antiapoptotic effect on B- ALL cells is mediated by Notch-3 and -4 or Jagged-1/-2 and DLL-1 in a synergistic manner...
  14. pmc A CADASIL-mutated Notch 3 receptor exhibits impaired intracellular trafficking and maturation but normal ligand-induced signaling
    Helena Karlstrom
    Department of Cell and Molecular Biology, Medical Nobel Institute, Karolinska Institute, Von Eulers vag 3, SE 171 77 Stockholm, Sweden
    Proc Natl Acad Sci U S A 99:17119-24. 2002
    ..Taken together, these data suggest that trafficking and localization rather than signaling of mNotch 3 are affected in mNotch 3(R142C)...
  15. pmc Rel/NF-kappaB can trigger the Notch signaling pathway by inducing the expression of Jagged1, a ligand for Notch receptors
    J Bash
    Center for Advanced Biotechnology and Medicine, 679 Hoes Lane, Piscataway, NJ 08854 5638, USA
    EMBO J 18:2803-11. 1999
    b>Jagged1 belongs to the DSL family of ligands for Notch receptors that control the proliferation and differentiation of various cell lineages. However, little is known about the transcription factors that regulate its expression...
  16. pmc PDZ-domain-mediated interaction of the Eph-related receptor tyrosine kinase EphB3 and the ras-binding protein AF6 depends on the kinase activity of the receptor
    B Hock
    Chemotherapeutisches Forschungsinstitut, Georg Speyer Haus, Paul Ehrlich Strasse 42 44, 60596 Frankfurt, Germany
    Proc Natl Acad Sci U S A 95:9779-84. 1998
    ....
  17. doi Analysis of JAG1 gene variant in Chinese patients with Alagille syndrome
    Honglian Wang
    Children s Hospital of Fudan University, Shanghai, China Institute of Biomedical Sciences, Fudan University, Shanghai, China
    Gene 499:191-3. 2012
    Alagille syndrome (AGS) is an autosomal dominant disorder characterized by bile duct paucity...
  18. doi JAG1 mutations are found in approximately one third of patients presenting with only one or two clinical features of Alagille syndrome
    K Guegan
    Department of Molecular Genetics Department of Clinical Genetics, Royal Devon and Exeter NHS Foundation Trust, Barrack Road, Exeter, UK
    Clin Genet 82:33-40. 2012
    ....
  19. ncbi Identification of 36 novel Jagged1 (JAG1) mutations in patients with Alagille syndrome
    Albrecht Röpke
    Institut fur Humangenetik und Medizinische Biologie, Martin Luther Universitat Halle Wittenberg, Halle, Germany
    Hum Mutat 21:100. 2003
    ..The previously described Jagged1 (JAG1) gene on chromosome 20p12 has been identified as being responsible for AGS...
  20. ncbi Jagged-1 mutation analysis in Italian Alagille syndrome patients
    G Pilia
    Istituto di Ricerca sulle Talassemie ed Anemie Mediterranee CNR, Cagliari, Italy
    Hum Mutat 14:394-400. 1999
    Alagille syndrome (AGS) is an autosomal dominant disorder with developmental abnormalities affecting the liver, heart, eyes, vertebrae, and craniofacial region...
  21. ncbi Fifteen novel mutations in the JAGGED1 gene of patients with Alagille syndrome
    C Crosnier
    INSERM Unité 347 affiliée au CNRS, F 94276 Le Kremlin Bicetre, France
    Hum Mutat 17:72-3. 2001
    Mutations in the human JAGGED1 gene cause Alagille syndrome, an autosomal dominant developmental disorder. The gene encodes a transmembrane protein which is a ligand of Notch receptors...
  22. ncbi Jagged1 (JAG1) mutation detection in an Australian Alagille syndrome population
    M L Heritage
    Joint Clinical Sciences Program, Queensland Institute of Medical Research and University of Queensland, Brisbane, Queensland, Australia
    Hum Mutat 16:408-16. 2000
    ..Mutations in the Jagged1 gene (JAG1) have been found to result in the AGS phenotype and both protein truncating mutations and missense ..
  23. ncbi Soluble Jagged 1 represses the function of its transmembrane form to induce the formation of the Src-dependent chord-like phenotype
    D Small
    Center for Molecular Medicine, Maine Medical Center Research Institute, Scarborough, Maine 04074, USA
    J Biol Chem 276:32022-30. 2001
    ....
  24. pmc Molecular basis for Jagged-1/Serrate ligand recognition by the Notch receptor
    Pat Whiteman
    Department of Biochemistry, University of Oxford, South Parks Road, Oxford OX1 3QU, United Kingdom
    J Biol Chem 288:7305-12. 2013
    ..As a proof of principle, we have generated a Notch-1-specific monoclonal antibody that blocks binding, thus paving the way for antibody tools for research and therapeutic applications...
  25. ncbi Notch ligands are substrates for protein O-fucosyltransferase-1 and Fringe
    Vladislav M Panin
    Howard Hughes Medical Institute, Waksman Institute and Department of Molecular Biology and Biochemistry, Rutgers, The State University, Piscataway, New Jersey 08854, USA
    J Biol Chem 277:29945-52. 2002
    ..Indeed, O-fucose on mammalian Delta 1 and Jagged1 can be elongated with Manic Fringe in vivo, and Drosophila Delta and Serrate are substrates for Drosophila Fringe ..
  26. doi The intracellular domain of Jagged-1 interacts with Notch1 intracellular domain and promotes its degradation through Fbw7 E3 ligase
    Mi Yeon Kim
    Hormone Research Center, School of Biological Sciences and Technology, Chonnam National University, Gwangju, Republic of Korea
    Exp Cell Res 317:2438-46. 2011
    ..Furthermore, JICD accelerates the protein degradation of Notch1-IC via Fbw7-dependent proteasomal pathway. These results indicate that JICD functions as a negative regulator in Notch1 signaling via the promotion of Notch1-IC degradation...
  27. ncbi Jagged1-induced Notch signaling drives proliferation of multiple myeloma cells
    Franziska Jundt
    Department of Hematology and Oncology, Charite, Campus Virchow Klinikum, University Medicine Berlin, Berlin, Germany
    Blood 103:3511-5. 2004
    ..Here we demonstrate that Notch receptors and their ligand Jagged1 are highly expressed in cultured and primary MM cells, whereas nonneoplastic counterparts show low to ..
  28. pmc TNF induction of jagged-1 in endothelial cells is NFkappaB-dependent
    Douglas A Johnston
    Department of Molecular Biology and Biochemistry, University of California Irvine, 92697, USA
    Gene 435:36-44. 2009
    ..Full promoter activity also depends on an AP-1 site at -2.0 kb. These results indicate that canonical NFkappaB signaling is required for TNF induction of the notch ligand jagged-1 in EC...
  29. ncbi Mouse jagged1 physically interacts with notch2 and other notch receptors. Assessment by quantitative methods
    K Shimizu
    Department of Hematology and Oncology, Graduate School of Medicine, University of Tokyo, Tokyo, 113 8655 Japan
    J Biol Chem 274:32961-9. 1999
    ..In this study, we cloned a cDNA for mouse Jagged1 (mJagged1)...
  30. ncbi Mutations in JAGGED1 gene are predominantly sporadic in Alagille syndrome
    C Crosnier
    INSERM Unité 347 affiliée au Centre National de la Recherche Scientifique, Departement de Pediatrie, Hopital de Bicetre, Le Kremlin Bicetre, France
    Gastroenterology 116:1141-8. 1999
    Mutations in the JAGGED1 gene are responsible for the Alagille syndrome, an autosomal dominant disorder characterized by neonatal jaundice, intrahepatic cholestasis, and developmental disorders affecting the liver, heart, vertebrae, eyes,..
  31. ncbi Jagged1 (JAG1) mutations in Alagille syndrome: increasing the mutation detection rate
    D M Warthen
    Division of Human Genetics and Molecular Biology, The Children s Hospital of Philadelphia and University of Pennsylvania School of Medicine, 19104, USA
    Hum Mutat 27:436-43. 2006
    Alagille syndrome (AGS) is caused by heterozygous mutations in JAG1, and mutations have been previously reported in about 70% of patients who meet clinical diagnostic criteria...
  32. ncbi The Notch ligands, Jagged and Delta, are sequentially processed by alpha-secretase and presenilin/gamma-secretase and release signaling fragments
    Matthew J LaVoie
    Center for Neurologic Diseases, Harvard Medical School and Brigham and Women s Hospital, Boston, Massachusetts 02215, USA
    J Biol Chem 278:34427-37. 2003
    ..Thus, Notch and its cognate ligands are processed by the same molecular machinery and may antagonistically regulate each other's signaling...
  33. doi Down-regulation of Notch-1 and Jagged-1 inhibits prostate cancer cell growth, migration and invasion, and induces apoptosis via inactivation of Akt, mTOR, and NF-kappaB signaling pathways
    Zhiwei Wang
    Department of Pathology, Karmanos Cancer Institute, Wayne State University, Detroit, Michigan 48201, USA
    J Cell Biochem 109:726-36. 2010
    ..Our results further suggest that inactivation of Notch signaling pathways by innovative strategies could be a potential targeted approach for the treatment of metastatic prostate cancer...
  34. doi Plasminogen activator uPA is a direct transcriptional target of the JAG1-Notch receptor signaling pathway in breast cancer
    Mamiko Shimizu
    Campbell Family Institute for Breast Cancer Research, Ontario Cancer Institute, Toronto, Ontario, Canada
    Cancer Res 71:277-86. 2011
    ..These data suggest that JAG1-induced Notch activation results in breast cancer progression through upregulation of the plasminogen activator system, directly linking these 2 important pathways of poor prognosis...
  35. pmc The CD46-Jagged1 interaction is critical for human TH1 immunity
    Gaelle Le Friec
    Division of Transplantation Immunology and Mucosal Biology, MRC Centre for Transplantation, King s College London, Guy s Hospital, London, UK
    Nat Immunol 13:1213-21. 2012
    ..Here we identified the Notch family member Jagged1 as a physiological ligand for CD46...
  36. ncbi Crosstalk between tumor and endothelial cells promotes tumor angiogenesis by MAPK activation of Notch signaling
    Qinghua Zeng
    Laboratory of Molecular Signaling and Apoptosis, Department of Biologic and Materials Sciences, University of Michigan, Ann Arbor, MI 48109, USA
    Cancer Cell 8:13-23. 2005
    ..Here, we report that the Notch ligand Jagged1 induced by growth factors via mitogen-activating protein kinase (MAPK) in head and neck squamous cell carcinoma (..
  37. ncbi Manic fringe and lunatic fringe modify different sites of the Notch2 extracellular region, resulting in different signaling modulation
    K Shimizu
    Department of Hematology, Graduate School of Medicine, University of Tokyo, Tokyo 113 8655, Japan
    J Biol Chem 276:25753-8. 2001
    ..Although both manic fringe (mFng) and lunatic fringe (lFng) decreased the binding of Jagged1 to Notch2 and not that of Delta1, the decrease by mFng was greater in degree than that by lFng...
  38. pmc Squamous odontogenic tumor of the mandible: a case report demonstrating immunoexpression of Notch1, 3, 4, Jagged1 and Delta1
    C H Siar
    Department of Oral Pathology, Oral Medicine and Periodontology, Faculty of Dentistry, University of Malaya, Kuala Lumpur, Malaysia
    Eur J Med Res 15:180-4. 2010
    ..Although Notch receptors and ligands have been shown to play a role in cell fate decisions in ameloblastomas, the role of these cell signaling molecules in SOT is unknown...
  39. pmc Human ligands of the Notch receptor
    G E Gray
    Department of Cell Biology, Yale University School of Medicine, New Haven, Connecticut, USA
    Am J Pathol 154:785-94. 1999
    ..have cloned and analyzed the expression of three human homologues of the Notch ligands Delta and Serrate, human Jagged1 (HJ1), human Jagged2 (HJ2), and human Delta1 (H-Delta-1), and determined their chromosomal localizations...
  40. ncbi Jagged: a mammalian ligand that activates Notch1
    C E Lindsell
    Department of Biological Chemistry School of Medicine, University of California, Los Angeles 90024
    Cell 80:909-17. 1995
    ..Our data support the hypothesis that Notch-ligand interactions function in maintaining mammalian cells in an undifferentiated state...
  41. ncbi Physical interaction of Delta1, Jagged1, and Jagged2 with Notch1 and Notch3 receptors
    K Shimizu
    Department of Hematology, Graduate School of Medicine, Tokyo, Japan
    Biochem Biophys Res Commun 276:385-9. 2000
    The Delta/Serrate/LAG-2 (DSL) domain-containing proteins, Delta1, Jagged1, and Jagged2, are considered to be ligands for Notch receptors...
  42. ncbi JAGGED1 expression is associated with prostate cancer metastasis and recurrence
    Sandro Santagata
    Department of Pathology, Brigham and Women s Hospital, Harvard Medical School, Boston, Massachusetts 02115, USA
    Cancer Res 64:6854-7. 2004
    ..Here we show that JAGGED1, a NOTCH receptor ligand, is significantly more highly expressed in metastatic prostate cancer as compared with ..
  43. ncbi The C-terminal PDZ-ligand of JAGGED1 is essential for cellular transformation
    Janice M Ascano
    Department of Molecular Genetics, Biochemistry, and Microbiology, University of Cincinnati, College of Medicine, Ohio 45267 0524, USA
    J Biol Chem 278:8771-9. 2003
    b>JAGGED1 is a member of the Delta/Serrate/Lag-2 (DSL) family of proteins that are cell-bound ligands for Notch receptors...
  44. pmc Complementation of human papillomavirus type 16 E6 and E7 by Jagged1-specific Notch1-phosphatidylinositol 3-kinase signaling involves pleiotropic oncogenic functions independent of CBF1;Su(H);Lag-1 activation
    Karthikeyan Veeraraghavalu
    National Centre for Biological Sciences, TIFR, UAS GKVK Campus, Bangalore 560065, India
    J Virol 79:7889-98. 2005
    ..b>Jagged1, in contrast to Delta1, is preferentially upregulated in human cervical tumors...
  45. ncbi Different modes of action of high-dose immunoglobulins in rheumatoid arthritis
    M Delire
    Clinical research Immunology, Behringwerke AG, Marburg, Germany
    Acta Univ Carol Med (Praha) 40:95-9. 1994
    ..I.v.i.g. preparations contain specific Abs which bind some of these super Ags, and impair their adequate presentation to T helper-cells. 4...
  46. pmc The regulation of histidine decarboxylase gene expression
    M Hocker
    Gastrointestinal Unit, Massachusetts General Hospital, Boston 02114, USA
    Yale J Biol Med 69:21-33. 1996
    ..studies, we established a suitable cell system by stable transfection of a human gastric adenocarcinoma cell line (AGS) with the CCK-B/gastrin receptor...
  47. ncbi [Clinical and bacteriological studies on hospital outbreak of Salmonella enteritidis food poisoning]
    Y Dohtsu
    Department of Medicine, Nagasaki Municipal Hospital
    Kansenshogaku Zasshi 75:110-5. 2001
    ..In vitro, S. Enteritidis were sensitive to OFLX, TFLX, FOM, most of PCs, CEPs, AGs but resistant to MPIPC, CAM, CLDM, VCM...
  48. ncbi Relief of intractable pruritus in Alagille syndrome by partial external biliary diversion
    Peter Mattei
    Department of General, Thoracic, and Fetal Surgery, Children s Hospital of Philadelphia, Philadelphia, PA 19104, USA
    J Pediatr Surg 41:104-7; discussion 104-7. 2006
    Patients with Alagille syndrome (AGS) may develop pruritus, skin hypertrophy, and xanthomas because of chronic cholestasis and hypercholesterolemia...
  49. ncbi Lack of hepatocellular CD10 along bile canaliculi is physiologic in early childhood and persistent in Alagille syndrome
    Jane A Byrne
    Division of Gene and Cell Based Therapy, King s College London School of Medicine, London, UK
    Lab Invest 87:1138-48. 2007
    ..NEA) has been recommended as a marker of cholestasis in adults but not in children with Alagille syndrome (AGS). We investigated ontogenic and disease-related differences in the expression of CD10...
  50. ncbi Leptin mediates a proliferative response in human gastric mucosa cells with functional receptor
    R Schneider
    Medical Department I, University Hospital Carl Gustav Carus, Technical University, Dresden, Germany
    Horm Metab Res 33:1-6. 2001
    ..METHODS: RT-PCR was employed to detect leptin mRNA in the human stomach and the human gastric carcinoma cell line AGS while immunogold staining and electron microscopy were used to detect leptin protein...
  51. pmc Jagged1 (JAG1) mutations in patients with tetralogy of Fallot or pulmonic stenosis
    Robert C Bauer
    Department of Pediatrics, The Children s Hospital of Philadelphia, 34th Street and Civic Center Boulevard, Philadelphia, PA 19104, USA
    Hum Mutat 31:594-601. 2010
    Mutations in the Notch pathway ligand Jagged1 (JAG1) cause Alagille syndrome (AGS), as well as cardiac defects in seemingly nonsyndromic individuals...
  52. ncbi Partial external biliary diversion for intractable pruritus and xanthomas in Alagille syndrome
    Karan M Emerick
    Department of Pediatrics, Children s Memorial Hospital, Northwestern University Medical School, Chicago, IL 60614, USA
    Hepatology 35:1501-6. 2002
    Alagille syndrome (AGS) causes intractable pruritus and disfiguring xanthomas because of retained bile acids and cholesterol...
  53. ncbi A mouse model of Alagille syndrome: Notch2 as a genetic modifier of Jag1 haploinsufficiency
    Brent McCright
    The Jackson Laboratory, 600 Main Street, Bar Harbor, Maine 04609, USA
    Development 129:1075-82. 2002
    ....
  54. ncbi Supernumerary digital flexion creases: an additional clinical manifestation of Alagille syndrome
    Binita M Kamath
    Division of Gastroenterology and Nutrition, The Children s Hospital of Philadelphia and University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104, USA
    Am J Med Genet 112:171-5. 2002
    ..b>Jagged1 has been identified as the AGS disease gene...
  55. ncbi Peripheral bile duct paucity and cholestasis in the liver of a patient with Alagille syndrome: further evidence supporting a lack of postnatal bile duct branching and elongation
    Louis Libbrecht
    Department of Pathology, University Hospitals of Leuven, Minderbroedersstraat 12, 3000 Leuven, Belgium
    Am J Surg Pathol 29:820-6. 2005
    Alagille syndrome (AGS) is a developmental, multiorgan disease caused by mutations of the Jagged1 gene...
  56. ncbi Bile duct proliferation in liver-specific Jag1 conditional knockout mice: effects of gene dosage
    Kathleen M Loomes
    Division of Gastroenterology, Hepatology and Nutrition, Children s Hospital of Philadelphia, Philadelphia, PA 19104, USA
    Hepatology 45:323-30. 2007
    ..When Jag1 conditional mice were crossed with mice carrying the Jag1 null allele, the adult progeny exhibited striking bile duct proliferation...
  57. ncbi Ileal exclusion for refractory symptomatic cholestasis in Alagille syndrome
    Biren P Modi
    Department of Surgery, Children s Hospital Boston and Harvard Medical School, Boston, MA 02115, USA
    J Pediatr Surg 42:800-5. 2007
    Alagille syndrome (AGS) can result in pruritic self-mutilation and disabling or disfiguring xanthomas...
  58. pmc Expression of Notch-1 and its ligand Jagged-1 in rat liver during liver regeneration
    Christoph Kohler
    Department of Internal Medicine, University of Halle, Halle, Germany
    Hepatology 39:1056-65. 2004
    ..In conclusion, Notch/Jagged signaling pathway is activated during liver regeneration and is potentially contributing to signals affecting cell growth and differentiation...
  59. pmc Jagged1 in the portal vein mesenchyme regulates intrahepatic bile duct development: insights into Alagille syndrome
    Jennifer J Hofmann
    Molecular Biology Institute, University of California Los Angeles, Los Angeles, CA 90095, USA
    Development 137:4061-72. 2010
    Mutations in the human Notch ligand jagged 1 (JAG1) result in a multi-system disorder called Alagille syndrome (AGS)...
  60. ncbi An autosomal recessive form of Alagille-like syndrome that is not linked to JAG1
    Sarah Dyack
    Department of Pediatrics, Dalhousie University, Halifax, Nova Scotia, Canada
    Genet Med 9:544-50. 2007
    ..Our objective was to characterize the disorder clinically and determine whether affected individuals had inherited a mutation in JAG1...
  61. ncbi [Potentiation of metaphit-induced audiogenic epilepsy with N-methyl-D-aspartate in rats]
    O Stanojlovic
    Department of Physiology, School of Medicine, University of Belgrade, Belgrade
    Srp Arh Celok Lek 128:316-21. 2000
    Audiogenic seizures (AGS) are induced by high intensity sound stimulation in genetically susceptible rats or in animals subjected to chemical or electrical manipulation...
  62. ncbi Alagille syndrome associated with a paracentric inversion 20p12.2p13 disrupting the JAG1 gene
    P Stankiewicz
    Institute of Human Genetics and Medical Biology, University Halle Wittenberg, Halle S, Germany
    Am J Med Genet 103:166-71. 2001
    Mutations in the human gene Jagged1 (JAG1) localized in 20p12 have been recently identified as causal for the anomalies found in patients with Alagille syndrome (AGS)...
  63. pmc A longitudinal study to identify laboratory predictors of liver disease outcome in Alagille syndrome
    Binita M Kamath
    Division of Gastroenterology, Children s Hospital of Philadelphia, USA
    J Pediatr Gastroenterol Nutr 50:526-30. 2010
    Liver disease in Alagille syndrome (AGS) is highly variable, ranging from biochemical abnormalities only to end-stage disease...
  64. doi Fluctuation of lipoprotein metabolism linked with bile acid-activated liver nuclear receptors in Alagille syndrome
    Hironori Nagasaka
    Division of Metabolism, Chiba Children s Hospital, Chiba 266 0007, Japan
    Atherosclerosis 198:434-40. 2008
    Alagille syndrome (AGS) is a rare hereditary disorder exhibiting fluctuating cholestasis and dyslipidemia...
  65. doi Orthotopic liver transplantation for children with Alagille syndrome
    Ronen Arnon
    Pediatrics, Mount Sinai Medical Center, New York, NY 10029, USA
    Pediatr Transplant 14:622-8. 2010
    b>AGS is an inherited disorder involving the liver, heart, eyes, face, and skeleton.
  66. ncbi Embryonic lethality and vascular defects in mice lacking the Notch ligand Jagged1
    Y Xue
    The Jackson Laboratory, 600 Main Street, Bar Harbor, ME 04609 1500, USA
    Hum Mol Genet 8:723-30. 1999
    ..Mutations in the human JAGGED1 ( JAG1 ) gene, which encodes a ligand for the Notch family of transmembrane receptors, cause the autosomal ..
  67. ncbi The expression of Jagged1 in the developing mammalian heart correlates with cardiovascular disease in Alagille syndrome
    K M Loomes
    Division of Human Genetics, The Children s Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia 19104, USA
    Hum Mol Genet 8:2443-9. 1999
    ..Recently, mutations in Jagged1 ( JAG1 ), a conserved gene of the Notch intercellular signaling pathway, have been found to cause AGS...
  68. ncbi Jagged1 mutations in patients ascertained with isolated congenital heart defects
    I D Krantz
    Division of Human Genetics, Children s Hospital of Philadelphia and The University of Pennsylvania School of Medicine, 19104, USA
    Am J Med Genet 84:56-60. 1999
    Mutations in Jagged1 cause Alagille syndrome (AGS), a pleiotropic disorder with involvement of the liver, heart, skeleton, eyes, and facial structures. Cardiac defects are seen in more than 95% of AGS patients...
  69. ncbi The role of Notch receptor expression in bile duct development and disease
    Diana M Flynn
    Liver Research Laboratories, Queen Elizabeth Hospital, Edgbaston, UK
    J Pathol 204:55-64. 2004
    Mutations in the Jagged1 gene, a ligand for the Notch signalling pathway, have been implicated in the pathogenesis of Alagille syndrome (AGS), resulting in bile duct paucity...
  70. ncbi Applications of a copper microparticle-modified carbon fiber microdisk array electrode for the simultaneous determination of aminoglycoside antibiotics by capillary electrophoresis
    W C Yang
    Department of Chemistry, State Key Laboratory of Coordination Chemistry, Nanjing University, China
    J Chromatogr A 905:309-18. 2001
    ..employed in capillary electrophoresis for the simultaneous determination of the five aminoglycoside antibiotics (AGs) including netilmicin, tobramycin, lincomycin, kanamycin and amikacin...
  71. ncbi Slow wave sleep elevations after body heating: proximity to sleep and effects of aspirin
    J A Horne
    Department of Human Sciences, Loughborough University, Leicestershire, England
    Sleep 10:383-92. 1987
    On three different occasions, six healthy young adult subjects ahd their body temperatures raised by an average of 2.0 degrees C for 30 min while sitting in baths of warm water...
  72. pmc Isolation of tube precipitin antibody-reactive fractions of Coccidioides immitis
    D Kruse
    Department of Botany, University of Texas, Austin 78713 7640
    Infect Immun 58:169-78. 1990
    ..In this study we isolated two TP antibody-reactive antigens (TP-Ags) from an alkali-soluble, water-soluble fraction of the inner conidial wall and a culture filtrate plus toluene ..
  73. ncbi Quantitative structure-retention relationships in the examination of the topography of the binding site of antihistamine drugs on alpha 1-acid glycoprotein
    R Kaliszan
    Department of Biopharmaceutics and Pharmacodynamics, , Poland
    J Chromatogr A 722:25-32. 1996
    Quantitative relationships between the structure of antihistamine drugs (AHD) and their retention on an alpha 1-acid glycoprotein (AGP) HPLC column (QSRR) were studied in order to identify characteristic structural features of the ..
  74. ncbi Reduced diversity of CTLs specific for multiple minor histocompatibility antigens relative to allograft rejection in vivo
    W K Nevala
    Department of Surgery, The Mayo Foundation, Rochester, MN 55905, USA
    J Immunol 158:1102-7. 1997
    ..generated by the in vivo priming and in vitro boosting of mice with spleen cells incompatible for multiple minor H Ags are specific for a limited number of dominant Ags (peptides)...
  75. ncbi [Expression changes of Notch-related genes during the differentiation of human mesenchymal stem cells into neurons]
    Ying Xing
    The Stem Cells Research Center, China
    Sheng Li Xue Bao 59:267-72. 2007
    ..Based on these findings, we propose that low level of Notch signaling activation may contribute to neural cell differentiation...
  76. ncbi Demonstration of amyloid and immune complex deposits in renal and hepatic parenchyma of Alaskan alveolar hydatid disease patients
    Z Ali Khan
    Department of Microbiology and Immunology, School of Medicine, McGill University, Montreal, Quebec, Canada
    Ann Trop Med Parasitol 81:381-92. 1987
    ..in six liver (autopsy or biopsy) and two kidney (autopsy) samples from seven Alaskan alveolar hydatid disease (AHD) patients. Both the kidney samples showed evidence of severe nephropathy and glomerular IC deposits...
  77. ncbi Compression garments as countermeasures to orthostatic intolerance
    Steven H Platts
    NASA Johnson Space Center, Human Adaptations and Countermeasures Division, Houston, TX 77058, USA
    Aviat Space Environ Med 80:437-42. 2009
    ..of two compression garments, the National Aeronautics and Space Administration's inflatable antigravity suit (AGS) and the Russian Federal Space Agency's non-inflatable compression garment (Kentavr), to prevent hypovolemia-..
  78. pmc In vitro infection of Cryptosporidium parvum to four different cell lines
    J R Yu
    Department of Parasitology, College of Medicine, Konkuk University, Chungju, Korea
    Korean J Parasitol 38:59-64. 2000
    To determine a suitable condition for in vitro infection model of Cryptosporidium parvum, four different cell lines, AGS, MDCK, HCT-8 and Caco-2, were used as host cell lines which were cultured at various concentrations of added ..
  79. doi Induction of G2/M arrest and apoptosis by water extract of Strychni Semen in human gastric carcinoma AGS cells
    Sang Man Lee
    Department of Pathology, Dongeui University College of Oriental Medicine, Busan 614 052, South Korea
    Phytother Res 22:752-8. 2008
    ..The study tested whether and how the water extract of Strychni Semen (ESS) treatment would affect the growth of AGS human gastric carcinoma cells. ESS was found to inhibit the growth of AGS cells in a concentration-dependent manner...
  80. ncbi The covalent interaction of C3 with IgG immune complexes
    F Vivanco
    Department of Immunology, Fundacion Jimenez Diaz, Madrid, Spain
    Mol Immunol 36:843-52. 1999
    Antigens (Ags) are converted into immune complexes (antigen-antibody complexes, IC) as soon as they encounter their specific antibodies (Abs)...
  81. ncbi Effect of long term feeding of ammoniated wheat straw treated with or without HCl on blood biochemical parameters in growing male buffalo (Bubalus bubalis) calves
    Usha Rani Mehra
    Nuclear Research Laboratory, Indian Veterinary Research Institute, Izatnagar 243 122 UP, India
    Reprod Nutr Dev 45:163-73. 2005
    ..weight (BW) in a completely randomised design to study the effect of long term feeding of ammoniated wheat straw (AWS) and hydrochloric acid treated ammoniated wheat straw (HCl-AWS) on blood biochemical changes...
  82. ncbi Argyrophilic grains are not always argyrophilic--distinction from neurofibrillary tangles of diffuse neurofibrillary tangles with calcification revealed by comparison between Gallyas and Campbell-Switzer methods
    Toshiki Uchihara
    Department of Neuropathology, Tokyo Metropolitan Institute for Neuroscience, 2 6 Musashi dai, Fuchu, 183 8526, Tokyo, Japan
    Acta Neuropathol 110:158-64. 2005
    Silver staining profiles of argyrophilic grains (AGs) and of neurofibrillary tangles (NFTs) of diffuse neurofibrillary tangles with calcification (DNTC, collectively as DNTC-NFTs) were examined for their relation to tau- and ubiquitin-..
  83. pmc [18F]2-fluoro-2-deoxy-D-glucose incorporation by AGS gastric adenocarcinoma cells in vitro during response to epirubicin, cisplatin and 5-fluorouracil
    S A Suttie
    Department of Surgery, School of Medical Sciences, University of Aberdeen, Foresterhill, Aberdeen AB25 2ZN, UK
    Br J Cancer 97:902-9. 2007
    ..Here human gastric adenocarcinoma (AGS) cells were treated with lethal dose 10 and 50 (LD(10) and LD(50)), determined by using the MTT assay, of the three ..
  84. ncbi Characterization and strain distribution pattern of the murine Ah receptor specified by the Ahd and Ahb-3 alleles
    A Poland
    McArdle Laboratory for Cancer Research, University of Wisconsin, Madison 53706
    Mol Pharmacol 38:306-12. 1990
    ..used to characterize two further murine variants and the strains that carry them: 1) the low affinity Ah receptor (Ahd allele) in strains that are nonresponsive to aromatic hydrocarbons and 2) a newly identified, high affinity variant ..
  85. doi Suppressive effect of bacterial polysaccharides on BAFF system is responsible for their poor immunogenicity
    Sunita Kanswal
    Laboratory of Bacterial Polysaccharides, Division of Bacterial Parasitic and Allergenic Products, Center for Biologics Evaluation and Research, US Food and Drug Administration, Bethesda, MD 20852, USA
    J Immunol 186:2430-43. 2011
    Capsular polysaccharides of encapsulated bacteria are weakly immunogenic T cell-independent type 2 (TI-2) Ags. Recent findings suggest that BAFF system molecules have a critical role in the development of Ab responses against TI-2 Ags...
  86. ncbi Oral vaccination with salmonella simultaneously expressing Yersinia pestis F1 and V antigens protects against bubonic and pneumonic plague
    Xinghong Yang
    Veterinary Molecular Biology, Montana State University, Bozeman, MT 59717, USA
    J Immunol 178:1059-67. 2007
    ..To test whether the protective Ags to Yersinia pestis can be orally delivered, the Y...
  87. doi Correlation of Device landing zone calcification and acute procedural success in patients undergoing transcatheter aortic valve implantations with the self-expanding CoreValve prosthesis
    Daniel John
    Department of Cardiology Angiology, HELIOS Heart Center Siegburg, Siegburg, Germany
    JACC Cardiovasc Interv 3:233-43. 2010
    ....
  88. doi Tracheal intubation with the PENTAX-AWS (airway scope) reduces changes of hemodynamic responses and bispectral index scores compared with the Macintosh laryngoscope
    Koichi Nishikawa
    Department of Anesthesiology, Gunma University Graduate School of Medicine, Showa machi, Maebashi City 371 8511, Japan
    J Neurosurg Anesthesiol 21:292-6. 2009
    The PENTAX-AWS, a novel video laryngoscope, allows indirect visualization of the vocal cords on a color monitor display and enables tracheal intubation without upward lifting force required to expose the glottis...
  89. ncbi Trehalose dimycolate elicits eosinophilic skin hypersensitivity in mycobacteria-infected guinea pigs
    Atsushi Otsuka
    Laboratory of Cell Regulation, Institute for Virus Research, Kyoto University, Kyoto, Japan
    J Immunol 181:8528-33. 2008
    ..Besides protein Ags, recent evidence has suggested that a substantial immunity directed against glycolipid Ags is also elicited in ..
  90. ncbi Transactivation of the EGFR by AP-1 is induced by Helicobacter pylori in gastric cancer
    Hassan Ashktorab
    Department of Medicine and Cancer Center, Howard University, College of Medicine, Washington, District of Columbia, USA
    Am J Gastroenterol 102:2135-46. 2007
    ..In this study, we investigated the effects of H. pylori on EGFR- and AP-1-mediated signal transduction pathways in the AGS gastric epithelial cell line and gastric tissue from humans.
  91. ncbi Enzymatic conversion of retinaldehyde to retinoic acid by cloned murine cytosolic and mitochondrial aldehyde dehydrogenases
    M Chen
    Department of Pharmacology, Cornell University Medical College, New York, New York 10021
    Mol Pharmacol 46:88-96. 1994
    ..previously been reported that retinaldehyde can be converted to retinoic acid by cytosolic aldehyde dehydrogenase (AHD-2) in liver extracts [Biochem. Pharmacol. 42: 1279-1285 (1991)]...
  92. ncbi Evaluation of Schistosoma haematobium 27-29 kDa antigen for immunodiagnosis of schistosomiasis haematobium
    Azza El Amir
    Department of Zoology, Faculty of Science, Cairo University, Egypt
    J Egypt Soc Parasitol 38:435-51. 2008
    ..haematobium adult worm antigens (Ags) (SAWA), excretory/secretory Ags (E/S) and cysteine proteinase Ag (27-29 kDa) for diagnosis of schistosomiasis...
  93. pmc Mechanisms regulating monocyte CXCL8 secretion in neurocysticercosis and the effect of antiparasitic therapy
    Jasim Uddin
    Department of Infectious Diseases and Immunity, Wellcome Trust Centre for Clinical Tropical Medicine, Imperial College London, London, United Kingdom
    J Immunol 185:4478-84. 2010
    ..Stimulation of human monocytes by cyst Ags from NCC-infected pigs showed that scolex and membrane Ags drive CXCL8 and CCL2 secretion...
  94. ncbi Gamma-hydroxybutyrate (GHB) for treatment of alcohol withdrawal and prevention of relapses
    Maurizio A Leone
    SCDU Neurologia, , C Mazzini 18, 28100 Novara, Italy
    Cochrane Database Syst Rev 2:CD006266. 2010
    BACKGROUND: Chronic excessive alcohol consumption may lead to dependence, and to alcohol withdrawal syndrome (AWS) in case of abrupt drinking cessation...
  95. ncbi High-performance liquid chromatographic analysis of aminoglycoside antibiotics
    R Tawa
    Kyoto Pharmaceutical University, Japan
    J Chromatogr A 812:141-50. 1998
    A simple precolumn derivatisation method for the determination of aminoglycoside antibiotics (AGs) is described...
  96. ncbi Decreased GABA and increased glutamate receptor-mediated activity on inferior colliculus neurons in vitro are associated with susceptibility to ethanol withdrawal seizures
    C Faingold
    Departments of Pharmacology, Southern Illinois University School of Medicine, P O Box 19629, Springfield, IL 62794 9629, USA
    Brain Res 868:287-95. 2000
    ..in ethanol-dependent rats results in an ethanol withdrawal (ETX) syndrome, including audiogenic seizures (AGS)...
  97. ncbi Correlation analysis between anticonvulsant ED50 values of antiepileptic drugs in mice and rats and their therapeutic doses and plasma levels
    Meir Bialer
    Department of Pharmaceutics, School of Pharmacy, Faculty of Medicine, Hebrew University of Jerusalem, Jerusalem, Israel
    Epilepsy Behav 5:866-72. 2004
    ..Consequently, the aim of this work was to perform a correlation analysis between therapeutic daily doses (D) and average steady-state plasma concentrations (Css,av) of AEDs and their activity in common anticonvulsant animal models...
  98. ncbi [Effect of Abl-interacting protein 1 overexpression upon human gastric cancer cell proliferation in vitro]
    Mei Hua Cui
    Department of Gastroenterlogy, Peking University People s Hospital, Beijing 100044, China
    Zhonghua Yi Xue Za Zhi 89:3111-5. 2009
    ..the expression of Abl-interacting protein 1 (ABI1) in normal gastric mucosal cell line GES-1 and gastric cancer cell line AGS, and the effects of ABI1 gene overexpression upon the proliferation of human gastric cancer cell AGS in vitro.
  99. ncbi Baclofen in the treatment of alcohol withdrawal syndrome: a comparative study vs diazepam
    Giovanni Addolorato
    Institute of Internal Medicine, Catholic University of Rome, Rome, Italy
    Am J Med 119:276.e13-8. 2006
    Benzodiazepines are the drugs of choice in the treatment of alcohol withdrawal syndrome (AWS). Recent data have shown that baclofen may reduce AWS symptoms...
  100. doi Evaluation of the Pentax-AWS(®) and the Macintosh laryngoscope in difficult intubation: a manikin study
    T Kariya
    Department of Anesthesiology and Critical Care Medicine, Yokohama City University School of Medicine, Japan
    Acta Anaesthesiol Scand 55:223-7. 2011
    The Pentax-AWS (AWS(®)), a new video laryngoscope, has been shown to be useful in cases of difficult intubation...
  101. ncbi Secretory leukoprotease inhibitor in mucosal lymph node dendritic cells regulates the threshold for mucosal tolerance
    Janneke N Samsom
    Department of Molecular Cell Biology and Immunology, Vrije Universiteit University Medical Center, Amsterdam, The Netherlands
    J Immunol 179:6588-95. 2007
    The notion that the mucosal immune system maintains a tolerogenic response to harmless Ags while continually being challenged with microbial products seems an enigma...

Research Grants78

  1. MHC Class I Antigen Presentation in Viral Infections
    Luis J Sigal; Fiscal Year: 2011
    ..The current model, mostly inferred from experiments with non-infectious antigens (Ags), proposes that dendritic cells carry Ags from infected tissues to secondary lymphoid organs (SLO) where CD8+ T ..
  2. Progesterone &Estrogen Differentially Regulate DC Function in Lupus Autoimmunity
    Grant Hughes; Fiscal Year: 2012
    ..In SLE, however, immune complexes (ICs) comprised of autoantibodies (autoAbs) and nuclear antigens (Ags), through TLRs 7 and 9, activate myeloid DCs (mDCs) and stimulate plasmacytoid DCs (pDCs) to make large amounts of ..
  3. Regulation of Type I IFNs in Tolerance and Autoimmunity
    Stefania Gallucci; Fiscal Year: 2013
    ..Abnormal activation of DCs may shift the presentation of self-Ags from tolerance to autoimmunity and, indeed, excessive responses to IFN-a have been proposed to be pathogenic in ..
  4. Lymphatic endothelial cells as inducers of systemic peripheral tolerance
    Victor H Engelhard; Fiscal Year: 2013
    ..These subpopulations each express distinct subsets of many peripheral tissue Ags, suggesting that they represent a major, previously unrecognized mechanism of systemic intrinsic peripheral ..
  5. BACTERIAL AND LIPOSOMAL ANTIGEN PROCESSING
    Clifford V Harding; Fiscal Year: 2012
    DESCRIPTION (provided by applicant): Class I MHC Ag cross processing allows exogenous or vacuolar Ags to be processed for presentation to CD8+ T cells...
  6. Restriction of HIV infection by SAMHD1 protein
    Jacek Skowronski; Fiscal Year: 2013
    ..is to a large extent mediated by cellular SAMHD1 protein, mutations in which lead to Aicardi Goutieres Syndrome (AGS), an autoimmune disease associated with activation of the innate immune response thought to be provoked by ..
  7. ADENOVIRUS FLT3 LIGAND INDUCES NALT DCs FOR SALIVARY GLAND S-lgA Ab RESPONSES
    Kohtaro Fujihashi; Fiscal Year: 2010
    ..cells was important in the induction of Ag-specific salivary S-lgA Ab responses even for immune responses to Tl Ags. We hypothesize that targeting NALT with FL-based adjuvants induce migration of NALT DCs to the SMGs that interact ..
  8. CLASS II ANTIGEN PRESENTATION VIA CHAPERONE MEDIATED AUTOPHAGY
    Janice S Blum; Fiscal Year: 2013
    ..regulating class II cytoplasmic Ag presentation, and to identify new means to modulate immunity to self and tumor Ags. With subversion of class I presentation by tumors and viruses, exploiting novel pathways for cytoplasmic Ag ..
  9. Role of Peripheral Dendritic Cells in Central and Oral Tolerance
    Husein Hadeiba; Fiscal Year: 2013
    ..tolerance by induction of regulatory T cells (Tregs), but also efficiently transport innocuous peripheral antigens (Ags) to the thymus where they mediate central tolerance through clonal deletion...
  10. Establishing and characterizing BAFF RFP reporter and BAFF knockin mice
    Edward A Clark; Fiscal Year: 2013
    ..BAFF is essential for mature B cells to develop and to respond to antigens (Ags)...
  11. Disruption of HLA Class II-Mediated Immune Recognition of B-Cell Lymphomas
    Azizul Haque; Fiscal Year: 2013
    ..Several groups have shown that BL can not present antigens (Ags) by class I molecules which contribute to their escaping immune recognition from tumor-specific CD8+ T cells...
  12. Impact of Highly Active T Lymphocytes from Induced Pluripotent Stem Cells
    Jianxun Song; Fiscal Year: 2012
    ..The long-term goal is to elicit efficient immune responses to tumor Ags and facilitate the development and application of vaccines and immunotherapy that can be used to successfully ..
  13. T CELLS ON THE FUNCTIONAL LIFE-SPAN OF DENTRITIC CELLS
    Stefania Gallucci; Fiscal Year: 2004
    ..How long DCs can survive after activation and efficiently present Ags to T and B lymphocytes is still unclear...
  14. Immune Mechanism of Rejection in Human Lung Allografts
    Thalachallour Mohanakumar; Fiscal Year: 2013
    ..Recently, we have obtained evidence for an obligatory role for immune responses to lung associated self-antigens (Ags) and cross talk between immune responses to self-Ags and allo-Ags in pre-disposing for the development of BOS ..
  15. Genetic Modifiers of Liver Disease Severity in Alagille Syndrome
    Nancy Bettina Spinner; Fiscal Year: 2013
    ..Mutations in Jagged1 (JAG1) are found in 95% of patients and mutations in Notch2 in less than 1%...
  16. Mechanisms of Peripheral Induction of T-Cell Tolerance
    MATTHEW FRANKLIN MESCHER; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Mechanisms that enforce T tolerance to self-antigens (self-Ags) in the secondary lymphoid organs (periphery) are critical for preventing development of autoimmune diseases, but can also limit the ..
  17. Advancing our Understanding of Rare Pediatric Liver Diseases
    KATHLEEN MARY LOOMES; Fiscal Year: 2013
    ..the national collaborative process focusing on biliary atresia (BA), hepatic mitochondropathies, Alagille syndrome (AGS), alpha-one antitrypsin deficiency, progressive familial intrahepatic cholestasis, bile acid synthetic disorders, ..
  18. Jagged-Notch and Fgf signaling: patterning the vertebrate upper face
    ELIZABETH ZUNIGA-SANCHEZ; Fiscal Year: 2012
    ....
  19. Bioactive Peptides for Mitigation of AGS and Acute Radiation Injury
    Lurong Zhang; Fiscal Year: 2010
    ..In acute radiation injury (ARI), the major causes of death are the acute gastrointestinal syndrome (AGS) and BMS (bone marrow syndrome)...
  20. Immune Tolerance in Non-clonal Immune Systems
    David Nemazee; Fiscal Year: 2013
    ..These combining sites were expressed as chimeric membrane proteins, called macroself antigens (Ags), in transgenic mice...
  21. The central role of c-Met in H. pylori pathogenesis.
    Sarah E Keates; Fiscal Year: 2010
    ..pylori-infected AGS gastric epithelial cells;2) determine the mechanism and role of c-Met ectodomain shedding in response to H...
  22. Establichment of CHLA's ChilDREN Clinical Center
    KASPER SAONUN WANG; Fiscal Year: 2013
    ..idiopathic neonatal hepatitis, studied under the Biliary Atresia Research Consortium, along with Alagille syndrome (AGS), alpha-1-antitrypsin deficiency, progressive intrahepatic cholestasis, bile acid synthesis defects, mitochondrial ..
  23. Multivalent Plague, Anthrax Vaccines Using Bacteriophage T4 Display
    Venigalla B Rao; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): We hypothesize that in vitro assembly of antigens (Ags) on bacteriophage T4 can be developed as a common platform for construction of next generation multivalent biodefense vaccines...
  24. REGULATION OF PROTECTIVE HUMORAL IMMUNITY TO STREPTOCOCCUS PNEUMONIAE BY PD-1
    Karen M Haas; Fiscal Year: 2012
    ..and that blocking PD-1 from interacting with its ligands significantly increases IgG responses against TI-2 Ags, including PPS-3...
  25. Molecular Biology of Cardiac Valvuloseptal Morphogenesis
    DAVID GEENAN; Fiscal Year: 2006
    ..abstract_text> ..
  26. Molecular mechanisms of Bile Duct Proliferation in Mice with Glycosylation Defect
    MATTHEW JOSEPH RYAN; Fiscal Year: 2012
    ....
  27. Cell-Cell Interactions In The Retinal Vasculature
    PATRICIA ANN D'AMORE; Fiscal Year: 2013
    ..The proposed studies, which will examine the molecules involved in signaling between EC and pericytes, are critical to our understanding of the pathogenic process and to the development of effective means for prevention and treatment. ..
  28. A Novel Mitigation Agent for Gastrointestinal Syndrome of Acute Radiation Injury
    Lurong Zhang; Fiscal Year: 2009
    ..are no available agents to offer to victims after radiation exposure to mitigate acute gastrointestinal syndrome (AGS). FGF-P is a powerful radiation mitigator, which relieves AGS when given after irradiation...
  29. TYPE II Enterotoxins as Mucosal Immunomodulators
    Terry D Connell; Fiscal Year: 2011
    ..or bypasses those regulatory systems to dramatically enhance mucosal and systemic immune responses to foreign Ags. Upon intranasal administration, LT-IIb exerts immunostimulatory effects using immune pathways and cellular ..
  30. Epithelial-mesenchymal interactions in facial patterning
    GAGE D CRUMP; Fiscal Year: 2013
    ..The completion of these Aims will inform how changes in bone and cartilage differentiation underlie the facial defects of Axenfeld-Rieger Syndrome. ..
  31. Nontoxic Strategies to Establish Allochimerism in Mice
    Bruce R Blazar; Fiscal Year: 2012
    ..Although much is known about Ab response to foreign antigens (Ags), the mechanisms responsible for alloAb generation have not been fully elucidated because of an inability to track ..
  32. CXCL10/CXCR3 Signaling Regulates TLR4-Mediated Liver Inflamation and Injury
    Yuan Zhai; Fiscal Year: 2013
    ..Although exogenous Ags are not required for the pathogenesis of liver IRI, adaptive immune component CD4 T cells are indispensible, which ..
  33. Understanding the role of TREX1 in the innate immunity and autoimmune diseases
    Nan Yan; Fiscal Year: 2012
    ..Other TREX1 mutants have also been identified to associate with Aicardi-Goutieres syndrome (AGS) and retinal vasculopathy and cerebral leukodystrophy (RVCL)...
  34. AGS Guiding Principles for the Care of Older Adults with Multimorbidity: A Mobile
    Nancy Lundebjerg; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): The American Geriatrics Society (AGS) is requesting support from the Agency for Healthcare Research and Quality to develop and disseminate the Multimorbidity Guiding Principles App (MGP app) that is ..
  35. Molecular Analysis of Alagille Syndrome
    NANCY SPINNER; Fiscal Year: 2006
    ..The expressivity of this disorder is highly variable, both within and between families. AGS is caused by mutations in Jaggedi (JAGI), a member of the evolutionarily conserved Notch signaling pathway...
  36. Mitochondrial Acetylation Modification in Regulating Drug-Induced Liver
    Zhongping Lu; Fiscal Year: 2013
    ..We will also investigate the acetylation status of AlF.ATPa ahd'PiSPIO in regulating acetaminpp|pn*hepatbt^^ We believe that this study willunmask hovel funGliohs 0 pfotein ..
  37. Nonsense-mediated decay array analysis of atypical Werner syndrome
    Junko Oshima; Fiscal Year: 2010
    ..Cases without WRN mutations are operationally categorized as "atypical WS" (AWS). In 2003, we identified LMNA mutations among a small subset of atypical WS cases by a candidate gene approach...
  38. The role of Plasminogen activator Inhibitor-1 in Helicobacter pylori infection.
    Sarah E Keates; Fiscal Year: 2010
    ..Aim 1 examines how H. pylori upregulates PAI-1 in AGS (GEC's)...
  39. Liver Antigen - Presenting Cells in Hepatic Injury and Transplantation
    Angus W Thomson; Fiscal Year: 2010
    ..underlying the role of specific liver APC, that generally dampen systemic immune responses to gut-derived Ags, in regulation of liver injury and hepatic immunity relevant to transplant outcome...
  40. Notch signaling and bone fracture healing
    MICHAEL IRVING DISHOWITZ; Fiscal Year: 2012
    ..The purpose of this study is to 1) investigate the role(s) of the Notch signaling pathway in craniofacial bone healing, and 2) show that Notch manipulation can clinically enhance craniofacial bone regeneration. ..
  41. Notch signaling in Nos2 activation and steatohepatitis by obesity and alcohol
    Jun Xu; Fiscal Year: 2013
    ....
  42. G Proteins and Opiate Receptor Functions
    Ping Yee Law; Fiscal Year: 2010
    ..By recruiting different proteins to the receptor vicinity, e.g., beta-arrestin, Src, RGS and AGS, the magnitude and duration of signals could be modulated...
  43. HDV spread during chronic infection as a novel target for antiviral intervention
    Severin O Gudima; Fiscal Year: 2013
    ..due to mutations in ?Ag ORF, and whether ongoing spread selects against the genomes encoding non-functional ?Ags. Overall, this study will greatly advance our understanding of the mechanism of chronic HBV/HDV infection, and will ..
  44. Regulation of Notch Signaling by ErbB-2: Novel Therapeutic Strategy
    Clodia Osipo; Fiscal Year: 2013
    ....
  45. Dendritic Cell-Associated C-Type Lectins
    Edward A Clark; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Surprisingly little is known about how antigens (Ags) enter the immune system and induce B cells to produce sustained levels of neutralizing antibodies (Abs), which protect us against deadly viruses ..
  46. Molecular Mechanisms in Joint Development
    Anna Spagnoli; Fiscal Year: 2013
    ....
  47. Role of TLR2 engagement on CD4+ T cells in immunity to M. tuberculosis
    Roxana E Rojas; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): In addition to recognizing antigens (Ags) via the T cell receptor (TCR), CD4+T cells can recognize microbial patterns through pattern recognition receptors (PRRs)...
  48. Genetic Suceptibility for Occupational Asthma
    David I Bernstein; Fiscal Year: 2013
    ..will be tested for associations with confirmed DA (n=150) versus two comparator groups of 150 asymptomatic workers (AWs) exposed to methylene diphenyl diisocyanate (MDI) or hexamethylene diisocyanate (HDI)...
  49. Structure and dynamics of G protein coupled receptor-G protein complexes
    Brian K Kobilka; Fiscal Year: 2013
    ..flexible link between the two domains that make up G?s: the Ras-like GTPase domain and the alpha helical domain (AHD)...
  50. COMBINED ULTRASOUND/MOLECULAR ANALYSIS OF TUMOR RESPONSE TO VEGF BLOCKADE
    Jessica J Kandel; Fiscal Year: 2010
    ..We hypothesize that molecular changes in WT angiogenesis can be correlated with vascular changes revealed by noninvasive ultrasound. ..
  51. STATUS EPILEPTICUS REORGANIZES CANNABINOID RECEPTORS
    ROBERT JOHN DELORENZO; Fiscal Year: 2010
    ..SE) in the pilocarpine model of SE produces a long-term reorganization in the expression of the CB1 receptor ahd that these changes in CB1 expression persist for essentially the life of the animal...
  52. Evaluating Innovative Weight Reduction Strategies for College Students
    Melissa Nelson Laska; Fiscal Year: 2013
    ..They have dietary, activity, sleep ahd stress management patterns that put them at greater risk for overweight. (End of Abstract)
  53. Implantable Microdevice for the Treatment of Hydrocephalus
    HONGSEOK NOH; Fiscal Year: 2009
    ..When the AGs do not function properly this dynamic equilibrium is altered and the pathologic condition of hydrocephalus ensues...
  54. Regulatory Cell Signals in the Bone Marrow
    Laura M Calvi; Fiscal Year: 2013
    ..We demonstrated that 1) PTH or activation of its receptor stimulate the Notch ligand Jagged1 (Jag1) in osteoblastic cells;2) in mice with constitutively active PTH receptors in osteoblastic cells, HSCs have ..
  55. Intracellular nucleic acid detection in autoimmunity
    Daniel B Stetson; Fiscal Year: 2013
    ..Remarkably, loss of function mutations in the human Trex1 gene cause Aicardi-Goutieres Syndrome (AGS), a rare and severe disease that resembles congenitally acquired viral infection...
  56. Neuroimaging and Neurochemical Biomarkers in ADHD
    Sandra K Loo; Fiscal Year: 2010
    ..tools, Sibling Pairs (30 pairs concordant and 30 pairs discordant for ADHD) selected from the ADHD Genetic Study (AGS) (Loo PI) at UCLA, and a sample of healthy control children will be examined...
  57. Mechanisms of Alcohol Withdrawal Seizures: Role of L-type Ca2+ Channels
    PROSPER N'GOUEMO; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Alcohol withdrawal seizures (AWS) are one of the most common medical emergencies, but their underlying mechanisms are poorly understood...
  58. Cockayne syndrome: role of the innate immune response in neurodegeneration
    Alan M Weiner; Fiscal Year: 2013
    ..with myelin defects and brain calcifications resembling those seen in Aicardi- Gouti[unreadable]res syndrome (AGS), a childhood neurodevelopmental leukodystrophy caused by constitutive activation of an innate antiviral interferon ..
  59. Sensory Organ Formation in the Inner Ear
    AMY ELIZABETH KIERNAN; Fiscal Year: 2013
    ..pathway during sensory organ formation in the mouse inner ear, focusing on the role of one of the Notch ligands, Jagged1 (JAG1)...
  60. Pharmacotheraphy Evaluation Tools for Improving Breast Cancer Outcomes in Rural A
    Rajesh Balkrishnan; Fiscal Year: 2013
    ..Medical Association (AMA) Master file for information on physician and practitioners, American Hospital Dictionary (AHD) for healthcare facility characteristics...
  61. Genes regulating M cell differentiation
    David D Lo; Fiscal Year: 2013
    ..Our studies will provide important details on both the positive and negative regulators of this process. ..
  62. MECHANISMS OF ANTIGEN INDUCED TOLERANCE IN THE LUNG
    Anuradha Ray; Fiscal Year: 2013
    ..an important immunological outcome that inhibits deleterious immune responses to both self and non-self antigens (Ags). Impaired immune tolerance can manifest as either allergic or autoimmune disease...
  63. Mechanisms of Murine Turmor Eradication by Immunotherapy
    Philip D Greenberg; Fiscal Year: 2013
    ..This has reflected in part difficulties identifying immunogenic tumor antigens (Ags) that can be safely and effectively targeted, the low avidity of T cells for the identified tumor Ags that are ..
  64. International Registry of Werner Syndrome
    Junko Oshima; Fiscal Year: 2013
    ..WRN gene responsible for classical WS as well as novel LMNA mutations among patients with atypical Werner syndrome (AWS). As requested by the Japanese Werner Consortium (P.I...
  65. Comparative effectiveness in older cancer patients: age versus health status
    LINDA KROGH HAROOTYAN; Fiscal Year: 2010
    ..The sessions and webinars described in this proposal are co-sponsored by the International Society of Geriatric-Oncology (SIOG) and the American Geriatrics Society (AGS).
  66. Amygdala systems in associative learning
    Peter C Holland; Fiscal Year: 2013
    ..In the next project period, we will focus on the interface of learning, memory ahd attention by investigating how violations of learned outcome expectancies can enhance attention to the events ..
  67. Development of anti-Salmonella Single-chain Abs as serum diagnostics
    Michael D Gunn; Fiscal Year: 2013
    ..Accomplishing these aims will result in the development of a prototype lateral flow assay capable of detecting S. enterica, S. typhi, and S. paratyphi Ags in less than 100 [unreadable]l of whole blood.
  68. Relationships Among Interpersonal Stress, Affect Regulation, and Alcohol Lapse
    Stephen A Maisto; Fiscal Year: 2010
    ..Additionalvariables related to alco~oluse ahd relat~d functioning wHl be reassessed at6 and 12 weeks. . . . In the proposed two-ye...
  69. The Depression Hypertension COACH Study
    Yeates Conwell; Fiscal Year: 2013
    ..b>AWs participate with the PCP in developing multi-disciplinary care plans for their shared patients, reinforce treatment ..
  70. COBRE in Stem Cell Biology and Regenerative Medicine
    Don Wojchowski; Fiscal Year: 2012
    ..Overall efforts will forge a nationally competitive Center in this exciting discipline. ..
  71. THE ROLE OF NOTCH SIGNALING IN BILE DUCT DEVELOPMENT
    Kathleen Loomes; Fiscal Year: 2004
    ..The recent identification of Jag1 as the disease gene for Alagille syndrome (AGS), a multisystem developmental disorder involving bile duct paucity as a cardinal feature, implicates the Notch ..
  72. Conference Series in Geriatrics: From Bedside to Bench
    George A Kuchel; Fiscal Year: 2013
    ..and leaders from within and outside the aging research community, ample time for discussion, dissemination through AGS Annual Meeting Symposia and publication in JAGS or other high impact professional journals...
  73. Kidney development Cell fate and precursors of disease in the young and adult
    ROBERTO ARIEL GOMEZ; Fiscal Year: 2013
    ....
  74. The Role of the Notch Pathway in Bile Duct Development
    KATHLEEN MARY LOOMES; Fiscal Year: 2010
    ..Identification of Jag1 as the disease gene for Alagille syndrome (AGS), an autosomal dominant disorder characterized by bile duct paucity along with anomalies in other organ systems, ..
  75. CD4+ T CELLS RESPONDING TO SALMONELLA INFECTION
    Brad Cookson; Fiscal Year: 2005
    ..However, the specific bacterial antigens (Ags) recognized by Salmonella-immune hosts are largely unknown...
  76. Immunological memory to malaria liver stage antigens
    James Kazura; Fiscal Year: 2006
    ..falciparum (Pf) will test the hypothesis that a stable pool of CD8+ memory T-cells to liver-stage antigens (Ags) develops by adulthood in residents of malaria endemic areas and that these cells evolve from a genetically diverse ..
  77. Heat Shock Protein, Citrullinated Antigen and Dendritic Cell Interactions
    Frances Santiago Schwarz; Fiscal Year: 2009
    ..The recognition that cit-proteins (especially cit-fibrinogen) are key auto-antigens (Ags) initiating and perpetuating immunopathology in RA may represent a huge step toward achieving more effective ..
  78. Farnesol Analogues as Novel Treatment of Alcoholism
    Jean Baptiste Roullet; Fiscal Year: 2005
    ..persons, cessation of drinking leads to craving for alcohol and symptoms of alcohol withdrawal syndrome (AWS)...