Prnp

Summary

Gene Symbol: Prnp
Description: prion protein
Alias: AA960666, AI325101, CD230, PrP, PrP, PrPC, PrPSc, Prn-i, Prn-p, Sinc, prP27-30, prP33-35C, major prion protein
Species: mouse

Top Publications

  1. ncbi Novel proteinaceous infectious particles cause scrapie
    S B Prusiner
    Science 216:136-44. 1982
  2. ncbi Normal development and behaviour of mice lacking the neuronal cell-surface PrP protein
    H Bueler
    Institut für Molekularbiologie I, Universitat Zurich, Switzerland
    Nature 356:577-82. 1992
  3. ncbi 129/Ola mice carrying a null mutation in PrP that abolishes mRNA production are developmentally normal
    J C Manson
    Institute for Animal Health, Edinburgh
    Mol Neurobiol 8:121-7. 1994
  4. ncbi The cellular prion protein identifies bipotential cardiomyogenic progenitors
    Kyoko Hidaka
    Department of Bioscience, National Cardiovascular Center Research Institute, Suita, Osaka, Japan
    Circ Res 106:111-9. 2010
  5. ncbi Low density subcellular fractions enhance disease-specific prion protein misfolding
    James F Graham
    Neuropathogenesis Division, The Roslin Institute and Royal Dick School of Veterinary Studies, University of Edinburgh, Alexander Robertson Building, Easter Bush Veterinary Centre, Roslin, Midlothian EH25 9RG, UK
    J Biol Chem 285:9868-80. 2010
  6. ncbi In vitro conversion of full-length mammalian prion protein produces amyloid form with physical properties of PrP(Sc)
    Olga V Bocharova
    Medical Biotechnology Center, University of Maryland Biotechnology Institute, Baltimore, MD 21201, USA
    J Mol Biol 346:645-59. 2005
  7. ncbi Packaging of prions into exosomes is associated with a novel pathway of PrP processing
    L J Vella
    Department of Biochemistry and Molecular Biology, The University of Melbourne, Parkville, Victoria 3010, Australia
    J Pathol 211:582-90. 2007
  8. ncbi Prion protein attenuates excitotoxicity by inhibiting NMDA receptors
    Houman Khosravani
    Department of Physiology and Biophysics, Hotchkiss Brain Institute, University of Calgary, Calgary T2N4N1, Canada
    J Cell Biol 181:551-65. 2008
  9. ncbi The prion or the related Shadoo protein is required for early mouse embryogenesis
    Rachel Young
    INRA UMR1313, Génétique Animale et Biologie Intégrative, Institut National de la Recherche Agronomique, 78350 Jouy en Josas, France
    FEBS Lett 583:3296-300. 2009
  10. ncbi Expression and knockdown of cellular prion protein (PrPC) in differentiating mouse embryonic stem cells
    Oscar A Peralta
    Department of Large Animal Clinical Sciences, Virginia Maryland Regional College of Veterinary Medicine, Virginia Tech, Blacksburg, VA 24061 0442, USA
    Differentiation 81:68-77. 2011

Research Grants

  1. Reconstitution of Prion Infectivity
    Ilia Baskakov; Fiscal Year: 2005
  2. Reconstitution of Prion Transmission in Cells
    Ilia Baskakov; Fiscal Year: 2003
  3. Pathology of dysmyelination and demyelination
    Jack Rosenbluth; Fiscal Year: 2005
  4. Pathology of dysmyelination and demyelination
    Jack Rosenbluth; Fiscal Year: 2007
  5. Experimental Creutzfeldt Jacob Disease
    Laura Manuelidis; Fiscal Year: 2007
  6. Targeting the apoE/ABeta Interaction as a Novel AD Therapy
    Martin Sadowski; Fiscal Year: 2009
  7. Pathology of dysmyelination and demyelination
    Jack Rosenbluth; Fiscal Year: 2009
  8. Key molecular mechanisms of extraneural pathogenesis and transmission of TSEs
    Mark Zabel; Fiscal Year: 2009
  9. Targeting the apoE/ABeta Interaction as a Novel AD Therapy
    MARTIN JOSEPH SADOWSKI; Fiscal Year: 2010
  10. Key molecular mechanisms of extraneural pathogenesis and transmission of TSEs
    Mark D Zabel; Fiscal Year: 2010

Detail Information

Publications143 found, 100 shown here

  1. ncbi Novel proteinaceous infectious particles cause scrapie
    S B Prusiner
    Science 216:136-44. 1982
    ..Knowledge of the scrapie agent structure may have significance for understanding the causes of several degenerative diseases...
  2. ncbi Normal development and behaviour of mice lacking the neuronal cell-surface PrP protein
    H Bueler
    Institut für Molekularbiologie I, Universitat Zurich, Switzerland
    Nature 356:577-82. 1992
    b>PrPC is a host protein anchored to the outer surface of neurons and to a lesser extent of lymphocytes and other cells. The transmissible agent (prion) responsible for scrapie is believed to be a modified form of PrPC...
  3. ncbi 129/Ola mice carrying a null mutation in PrP that abolishes mRNA production are developmentally normal
    J C Manson
    Institute for Animal Health, Edinburgh
    Mol Neurobiol 8:121-7. 1994
    The neural membrane glycoprotein PrP is implicated in the pathogenesis of the transmissible spongiform encephalopathies; however, the normal function of PrP and its precise role in disease are not understood...
  4. ncbi The cellular prion protein identifies bipotential cardiomyogenic progenitors
    Kyoko Hidaka
    Department of Bioscience, National Cardiovascular Center Research Institute, Suita, Osaka, Japan
    Circ Res 106:111-9. 2010
    ..Identification of relevant surface markers may also enhance our understanding of the mechanisms underlying differentiation...
  5. ncbi Low density subcellular fractions enhance disease-specific prion protein misfolding
    James F Graham
    Neuropathogenesis Division, The Roslin Institute and Royal Dick School of Veterinary Studies, University of Edinburgh, Alexander Robertson Building, Easter Bush Veterinary Centre, Roslin, Midlothian EH25 9RG, UK
    J Biol Chem 285:9868-80. 2010
    ..with or without exogenous seed typically results in infectivity titers less than those associated with PrP(Sc) isolated ex vivo and highlights the potential role of co-factors that can catalyze disease-specific prion ..
  6. ncbi In vitro conversion of full-length mammalian prion protein produces amyloid form with physical properties of PrP(Sc)
    Olga V Bocharova
    Medical Biotechnology Center, University of Maryland Biotechnology Institute, Baltimore, MD 21201, USA
    J Mol Biol 346:645-59. 2005
    The "protein only" hypothesis postulates that the infectious agent of prion diseases, PrP(Sc), is composed of the prion protein (PrP) converted into an amyloid-specific conformation...
  7. ncbi Packaging of prions into exosomes is associated with a novel pathway of PrP processing
    L J Vella
    Department of Biochemistry and Molecular Biology, The University of Melbourne, Parkville, Victoria 3010, Australia
    J Pathol 211:582-90. 2007
    ..are fatal, transmissible neurodegenerative disorders associated with conversion of the host-encoded prion protein (PrP(C)) into an abnormal pathogenic isoform (PrP(Sc))...
  8. ncbi Prion protein attenuates excitotoxicity by inhibiting NMDA receptors
    Houman Khosravani
    Department of Physiology and Biophysics, Hotchkiss Brain Institute, University of Calgary, Calgary T2N4N1, Canada
    J Cell Biol 181:551-65. 2008
    It is well established that misfolded forms of cellular prion protein (PrP [PrP(C)]) are crucial in the genesis and progression of transmissible spongiform encephalitis, whereas the function of native PrP(C) remains incompletely ..
  9. ncbi The prion or the related Shadoo protein is required for early mouse embryogenesis
    Rachel Young
    INRA UMR1313, Génétique Animale et Biologie Intégrative, Institut National de la Recherche Agronomique, 78350 Jouy en Josas, France
    FEBS Lett 583:3296-300. 2009
    The prion protein PrP has a key role in transmissible spongiform encephalopathies but its biological function remains largely unknown. Recently, a related protein, Shadoo, was discovered...
  10. ncbi Expression and knockdown of cellular prion protein (PrPC) in differentiating mouse embryonic stem cells
    Oscar A Peralta
    Department of Large Animal Clinical Sciences, Virginia Maryland Regional College of Veterinary Medicine, Virginia Tech, Blacksburg, VA 24061 0442, USA
    Differentiation 81:68-77. 2011
    The mammalian cellular prion protein (PrP(C)) is a highly conserved glycoprotein that may undergo conversion into a conformationally altered isoform (scrapie prion protein or PrP(Sc)), widely believed to be the pathogenic agent of ..
  11. ncbi Developmental expression of prion protein and its ligands stress-inducible protein 1 and vitronectin
    Glaucia N M Hajj
    Ludwig Institute for Cancer Research, São Paulo 01323 903, Brazil
    J Comp Neurol 517:371-84. 2009
    Prion protein (PrP(C)) is the normal isoform of PrP(Sc), a protein involved in neurodegenerative disorders...
  12. ncbi Mutant prion protein expression causes motor and memory deficits and abnormal sleep patterns in a transgenic mouse model
    Sara Dossena
    Dulbecco Telethon Institute, 20156 Milan, Italy Department of Neuroscience, Mario Negri Institute for Pharmacological Research, 20156 Milan, Italy
    Neuron 60:598-609. 2008
    A familial form of Creutzfeldt-Jakob disease (CJD) is linked to the D178N/V129 prion protein (PrP) mutation...
  13. ncbi Early detection of PrPres in BSE-infected bovine PrP transgenic mice
    J Castilla
    Centro de Investigacion en Sanidad Animal, INIA, Madrid, Spain
    Arch Virol 148:677-91. 2003
    ..Typical CNS spongiform degeneration was observed by histopathology and presence of PrP(res) could be detected both by Western blot and immunohistochemistry (IHC) assays, confirming for this model the ..
  14. ncbi Targeting cellular prion protein reverses early cognitive deficits and neurophysiological dysfunction in prion-infected mice
    Giovanna R Mallucci
    MRC Prion Unit, Department of Neurodegenerative Disease, Institute of Neurology, Queen Square, London WC1N 3BG, United Kingdom
    Neuron 53:325-35. 2007
    ..However, we previously showed that targeting endogenous neuronal prion protein (PrP(C)) (the precursor of its disease-associated isoform, PrP(Sc)) in mice with early prion infection reversed ..
  15. ncbi Expression of Prnp mRNA (prion protein gene) in mouse spermatogenic cells
    Masahiko Fujisawa
    Department of Veterinary Anatomy, Graduate School of Agricultural and Life Sciences, The University of Tokyo, Yayoi, Bunkyo ku, Japan
    J Reprod Dev 50:565-70. 2004
    The Prnp (prion protein) gene, which encodes a soluble protein anchored to the cell surface by glycosylphosphatidylinositol (GPI), might be involved in cell-to-cell interaction...
  16. ncbi Identification of two prion protein regions that modify scrapie incubation time
    S Supattapone
    Institute for Neurodegenerative Diseases, University of California, San Francisco, California 94143, USA
    J Virol 75:1408-13. 2001
    ..with Rocky Mountain Laboratory (RML) murine prions, scrapie incubation times for Tg(MoPrP)4053, Tg(MHM2)294/Prnp(0/0), and Tg(MoPrP, Delta23-88)9949/Prnp(0/0) mice were approximately 50, 120, and 160 days, respectively...
  17. ncbi Brain transcriptional stability upon prion protein-encoding gene invalidation in zygotic or adult mouse
    Sead Chadi
    INRA, UMR1313, Génétique Animale et Biologie Intégrative, F 78350, Jouy en Josas, France
    BMC Genomics 11:448. 2010
    ..To potentially assess this conundrum, we performed a comparative transcriptomic analysis of the brain of wild-type mice with that of transgenic mice invalidated at this locus either at the zygotic or at the adult stages...
  18. ncbi Identifying key components of the PrPC-PrPSc replicative interface
    Gil C Abalos
    Department of Immunology, The Scripps Research Institute, La Jolla, California 92037, USA
    J Biol Chem 283:34021-8. 2008
    In prion disease, direct interaction between the cellular prion protein (PrP(C)) and its misfolded disease-associated conformer PrP(Sc) is a crucial, although poorly understood step promoting the formation of nascent PrP(Sc) and prion ..
  19. ncbi Overexpression of cellular prion protein alters postischemic Erk1/2 phosphorylation but not Akt phosphorylation and protects against focal cerebral ischemia
    Jens Weise
    Department of Neurology, University of Jena Medical School, 07747 Jena, Germany
    Restor Neurol Neurosci 26:57-64. 2008
    The physiological function of the cellular prion protein (PrPC) is still unclear. A growing body of evidence suggests that PrPC has neuroprotective properties and that its deletion increases susceptibility to focal cerebral ischemia...
  20. ncbi Involvement of cellular prion protein in the nociceptive response in mice
    Flavia Carla Meotti
    Departamento de Quimica, Universidade Federal de Santa Maria, Santa Maria, RS, 97110 000, Brasil
    Brain Res 1151:84-90. 2007
    ..Here we investigated the putative involvement of the PrP(c) function on the nociceptive response using PrP(c) null (Prnp(0/0)) and wild-type (Prnp(+/+)) mice submitted to thermal and chemical models of nociception...
  21. ncbi Developmental influence of the cellular prion protein on the gene expression profile in mouse hippocampus
    Stefano Benvegnù
    Laboratory of Prion Biology, Scuola Internazionale Superiore di Studi Avanzati SISSA, Trieste
    Physiol Genomics 43:711-25. 2011
    ..5-day-old) and adult (3-mo-old) mice, both from wild-type and Prnp(0/0) animals...
  22. ncbi New insights into cellular prion protein (PrPc) functions: the "ying and yang" of a relevant protein
    Oriol Nicolas
    Molecular and Cellular Neurobiotechnology laboratory, Catalonian Bioengineering Institute IBEC, Baldiri Reixac 15 21, and Department of Cell Biology, University of Barcelona, Barcelona, Spain
    Brain Res Rev 61:170-84. 2009
    The conversion of cellular prion protein (PrP(c)), a GPI-anchored protein, into a protease-K-resistant and infective form (generally termed PrP(sc)) is mainly responsible for Transmissible Spongiform Encephalopathies (TSEs), ..
  23. ncbi Threshold for epileptiform activity is elevated in prion knockout mice
    S Ratte
    Neuronal Networks Group, School of Clinical and Experimental Medicine, University of Birmingham, Edgbaston, Birmingham B15 2TT, UK
    Neuroscience 179:56-61. 2011
    Prion protein (PrP) is abundant in the nervous system, but its role remains uncertain. Prion diseases depend on an aggregation of the protein that is likely to interfere with its normal function...
  24. ncbi Prion protein misfolding affects calcium homeostasis and sensitizes cells to endoplasmic reticulum stress
    Mauricio Torres
    Center for Molecular Studies of the Cell, Institute of Biomedical Sciences, Faculty of Medicine, University of Chile, Santiago, Chile
    PLoS ONE 5:e15658. 2010
    ..well as the accumulation of an abnormally folded and protease resistant form of the cellular prion protein, termed PrP(RES)...
  25. ncbi Cellular prion protein localizes to the nucleus of endocrine and neuronal cells and interacts with structural chromatin components
    Alexander Strom
    Chronic Disease Program, Ottawa Hospital Research Institute, Ottawa, Ontario, Canada
    Eur J Cell Biol 90:414-9. 2011
    Several physiological processes have been purported for cellular prion protein (PrP(C)). However, the physiological function of PrP(C) is still unclear and the cellular localization of PrP(C) remains a subject of debate...
  26. ncbi Cellular prion protein promotes regeneration of adult muscle tissue
    Roberto Stella
    University of Padua, Department of Biological Chemistry, Padua, Italy
    Mol Cell Biol 30:4864-76. 2010
    It is now well established that the conversion of the cellular prion protein, PrP(C), into its anomalous conformer, PrP(Sc), is central to the onset of prion disease...
  27. ncbi Localization of the human gene allowing infection by gibbon ape leukemia virus to human chromosome region 2q11-q14 and to the homologous region on mouse chromosome 2
    M Kaelbling
    Department of Molecular Genetics, Albert Einstein College of Medicine, Bronx, New York 10461
    J Virol 65:1743-7. 1991
    ..This mouse chromosome carries Rec-2, the likely receptor for M813, a retrovirus derived from a feral Asian mouse. These data raise the interesting possibility that Rec-2 and Glvr-1 are structurally related...
  28. ncbi Normal host prion protein necessary for scrapie-induced neurotoxicity
    S Brandner
    Department of Pathology, University Hospital, Zurich, Switzerland
    Nature 379:339-43. 1996
    Accumulation of the prion protein PrPSc, a pathological and protease-resistant isoform of the normal host protein PrPC, is a feature of prion disease such as scrapie...
  29. ncbi Prions prevent neuronal cell-line death
    C Kuwahara
    Nature 400:225-6. 1999
  30. ncbi Loss of cerebellar Purkinje cells in aged mice homozygous for a disrupted PrP gene
    S Sakaguchi
    Department of Bacteriology, Nagasaki University School of Medicine, Nagasaki, Japan
    Nature 380:528-31. 1996
    Prion protein (PrP) is a glycoprotein constitutively expressed on the neuronal cell surface. A protease-resistant isoform of prion protein is implicated in the pathogenesis of a series of transmissible spongiform encephalopathies...
  31. ncbi Cellular prion protein mediates impairment of synaptic plasticity by amyloid-beta oligomers
    Juha Lauren
    Cellular Neuroscience, Neurodegeneration and Repair Program, Yale University School of Medicine, New Haven, Connecticut 06536, USA
    Nature 457:1128-32. 2009
    ..Here we identify the cellular prion protein (PrP(C)) as an amyloid-beta-oligomer receptor by expression cloning...
  32. ncbi The prion protein requires cholesterol for cell surface localization
    Sabine Gilch
    Institute of Virology, Technical University of Munich, Biedersteiner Str 29, D 80802 Munich, Germany
    Mol Cell Neurosci 31:346-53. 2006
    The cellular prion protein PrP(c) is attached to the plasma membrane by a glycosyl-phosphatidyl-inositol (GPI-) anchor and is localized in lipid rafts, membrane microdomains characterized by a high content of sphingolipids and ..
  33. ncbi The prion protein gene: a role in mouse embryogenesis?
    J Manson
    Institute for Animal Health, AFRC, Edinburgh, UK
    Development 115:117-22. 1992
    The neural membrane glycoprotein PrP (prion protein) has a key role in the development of scrapie and related neurodegenerative diseases...
  34. ncbi Single treatment with RNAi against prion protein rescues early neuronal dysfunction and prolongs survival in mice with prion disease
    Melanie D White
    Department of Neurodegenerative Disease, Medical Research Council, Prion Unit Institute of Neurology, University College London, London, United Kingdom
    Proc Natl Acad Sci U S A 105:10238-43. 2008
    ..Prion propagation involves the conversion of cellular prion protein, PrP(C), to its conformational isomer, PrP(Sc), which accumulates in disease...
  35. ncbi Prion protein is necessary for normal synaptic function
    J Collinge
    Department of Biochemistry and Molecular Genetics, St Mary s Hospital Medical School, Imperial College, London, UK
    Nature 370:295-7. 1994
    ..consists principally of a post-translationally modified form of a host-encoded glycoprotein (PrPC), designated PrPSc (ref. 1); the normal cellular function of PrPC is, however, unknown...
  36. ncbi Altered circadian activity rhythms and sleep in mice devoid of prion protein
    I Tobler
    Institute of Pharmacology, University of Zurich, Switzerland
    Nature 380:639-42. 1996
    There is a wealth of data supporting a central role for the prion protein (PrP) in the neurodegenerative prion diseases of both humans and other species, yet the normal function of PrP, which is expressed at the cell surface of neurons ..
  37. ncbi Accumulation of proteinase K-resistant prion protein (PrP) is restricted by the expression level of normal PrP in mice inoculated with a mouse-adapted strain of the Creutzfeldt-Jakob disease agent
    S Sakaguchi
    Department of Bacteriology, Nagasaki University School of Medicine, Japan
    J Virol 69:7586-92. 1995
    ..To determine whether there was an involvement of the host-encoded prion protein (PrPc) in CJD development and prion propagation, mice heterozygous (PrP+/-) or homozygous (PrP-/-) for a disrupted PrP ..
  38. ncbi Host PrP glycosylation: a major factor determining the outcome of prion infection
    Nadia L Tuzi
    Neuropathogenesis Unit, Roslin Institute, Edinburgh, United Kingdom
    PLoS Biol 6:e100. 2008
    The expression of the prion protein (PrP) is essential for transmissible spongiform encephalopathy (TSE) or prion diseases to occur, but the underlying mechanism of infection remains unresolved...
  39. ncbi Immunologically induced, complement-dependent up-regulation of the prion protein in the mouse spleen: follicular dendritic cells versus capsule and trabeculae
    Marius Lötscher
    Institutes of Experimental Immunology and Neuropathology, University Hospital of Zurich, Zurich, Switzerland
    J Immunol 170:6040-7. 2003
    The expression of the prion protein (PrP) in the follicular dendritic cell network of germinal centers in the spleen is critical for the splenic propagation of the causative agent of prion diseases...
  40. ncbi Reduced erythroid cell and erythropoietin production in response to acute anemia in prion protein-deficient (Prnp-/-) mice
    Jan H Zivny
    1st School of Medicine, Charles University, Prague, Czech Republic
    Blood Cells Mol Dis 40:302-7. 2008
    ..Induction of equivalent anemia in wild-type (WT) and Prnp-/- mice resulted in a higher number of circulating reticulocytes, hematocrits and spleen weights in WT mice than in ..
  41. ncbi Prions prevent brain damage after experimental brain injury: a preliminary report
    S Hoshino
    Departament of Neurosurgery, Nippon Medical School, Tokyo, Japan
    Acta Neurochir Suppl 86:297-9. 2003
    ..In the current study we used prion protein gene knock-out mice (Prnp-/-) to assess the role of PrPC in traumatic brain injury...
  42. ncbi Conditions of endoplasmic reticulum stress favor the accumulation of cytosolic prion protein
    Andrea Orsi
    Universita Vita Salute San Raffaele, DiBiT Istituto Scientifico San Raffaele, Via Olgettina 58, 20132 Milano, Italy
    J Biol Chem 281:30431-8. 2006
    After signal sequence-dependent targeting to the endoplasmic reticulum (ER), prion protein (PrP) undergoes several post-translational modifications, including glycosylation, disulfide bond formation, and the addition of a ..
  43. ncbi Anchorless prion protein results in infectious amyloid disease without clinical scrapie
    Bruce Chesebro
    Laboratory of Persistent Viral Diseases, Rocky Mountain Laboratories, National Institute of Allergy and Infectious Diseases, Hamilton, MT 59840, USA
    Science 308:1435-9. 2005
    ..In scrapie-infected transgenic mice expressing prion protein (PrP) lacking the glycosylphosphatidylinositol (GPI) membrane anchor, abnormal protease-resistant PrPres was deposited ..
  44. ncbi Neonatal lethality in transgenic mice expressing prion protein with a deletion of residues 105-125
    Aimin Li
    Department of Cell Biology and Physiology, Washington University School of Medicine, St Louis, MO 63110, USA
    EMBO J 26:548-58. 2007
    To identify sequence domains important for the neurotoxic and neuroprotective activities of the prion protein (PrP), we have engineered transgenic mice that express a form of murine PrP deleted for a conserved block of 21 amino acids (..
  45. ncbi Prion protein recruits its neuronal receptor NCAM to lipid rafts to activate p59fyn and to enhance neurite outgrowth
    Antonella Santuccione
    Zentrum für Molekulare Neurobiologie, Universitat Hamburg, Germany
    J Cell Biol 169:341-54. 2005
    In spite of advances in understanding the role of the cellular prion protein (PrP) in neural cell interactions, the mechanisms of PrP function remain poorly characterized...
  46. ncbi Hippocampal synaptic plasticity in mice devoid of cellular prion protein
    Laura E Maglio
    Departamento de Farmacologia, Facultad de Ciencias Quimicas, Universidad Nacional de Cordoba, Haya de la Torre y Medina Allende, 5000 Cordoba, Argentina
    Brain Res Mol Brain Res 131:58-64. 2004
    ..The higher expression of specific N-methyl-d-aspartate receptor subunits may account for these effects...
  47. ncbi Doppel-induced Purkinje cell death is stoichiometrically abrogated by prion protein
    Naohiro Yamaguchi
    Department of Molecular Microbiology and Immunology, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki 852 8523, Japan
    Biochem Biophys Res Commun 319:1247-52. 2004
    Mice devoid of prion protein (PrP) exhibiting ataxia and Purkinje cell degeneration, such as Ngsk Prnp(-/-) mice, ectopically express PrP-like protein, Dpl, in neurons including Purkinje cells...
  48. ncbi Alpha- and beta- cleavages of the amino-terminus of the cellular prion protein
    Alain Mangé
    Institut de Genetique Humaine, CNRS U P R 1142, 141, rue de la Cardonille, 34396 Montpellier Cedex 5, France
    Biol Cell 96:125-32. 2004
    It is commonly assumed that the physiological isoform of prion protein, PrP(C), is cleaved during its normal processing between residues 111/112, whereas the pathogenic isoform, PrP(Sc), is cleaved at an alternate site in the octapeptide ..
  49. ncbi A cellular gene encodes scrapie PrP 27-30 protein
    B Oesch
    Cell 40:735-46. 1985
    A clone encoding PrP 27-30, the major protein in purified preparations of scrapie agent, was selected from a scrapie-infected hamster brain cDNA library by oligonucleotide probes corresponding to the N terminus of the protein...
  50. ncbi Mice devoid of PrP are resistant to scrapie
    H Bueler
    Institut für Molekularbiologie I, Universitat Zurich, Switzerland
    Cell 73:1339-47. 1993
    S.B. Prusiner proposed that the infectious agent of scraple, the prion, is PrPSc, a modified form of the normal host protein PrPC. Prn-p0/0 mice devoid of PrPC showed normal development and behavior...
  51. ncbi Stimulation of PrP(C) retrograde transport toward the endoplasmic reticulum increases accumulation of PrP(Sc) in prion-infected cells
    Florence Beranger
    Institut de Genetique Humaine, UPR CNRS1142, 141 rue de la Cardonille, 34396, Montpellier Cedex 5, France
    J Biol Chem 277:38972-7. 2002
    ..disorders characterized by the accumulation of an abnormally folded isoform of the cellular prion protein (PrP(C)) denoted PrP(Sc)...
  52. ncbi A deleted prion protein that is neurotoxic in vivo is localized normally in cultured cells
    Heather M Christensen
    Department of Cell Biology and Physiology, Washington University School of Medicine, St Louis, Missouri 63110, USA
    J Neurochem 108:44-56. 2009
    The prion protein (PrP) possesses sequence-specific domains that endow the molecule with neuroprotective and neurotoxic activities, and that may contribute to the pathogenesis of prion diseases...
  53. ncbi Accumulation of protease-resistant prion protein (PrP) and apoptosis of cerebellar granule cells in transgenic mice expressing a PrP insertional mutation
    R Chiesa
    Department of Cell Biology and Physiology, Washington University School of Medicine, St Louis, MO 63110, USA
    Proc Natl Acad Sci U S A 97:5574-9. 2000
    We have generated lines of transgenic mice that express a mutant prion protein (PrP) containing 14 octapeptide repeats whose human homologue is associated with an inherited prion dementia...
  54. ncbi Expression of amino-terminally truncated PrP in the mouse leading to ataxia and specific cerebellar lesions
    D Shmerling
    Institut fur Molekularbiologie, Abteilung I, Universitat Zurich, Switzerland
    Cell 93:203-14. 1998
    The physiological role of prion protein (PrP) remains unknown. Mice devoid of PrP develop normally but are resistant to scrapie; introduction of a PrP transgene restores susceptibility to the disease...
  55. ncbi Delimiting the location of the scrapie prion incubation time gene on chromosome 2 of the mouse
    G A Carlson
    McLaughlin Research Institute, Great Falls, Montana 59401
    Genetics 133:979-88. 1993
    ..influenced by an incubation time gene (Prn-i) that is linked to Prn-p, the structural gene for prion protein (PrP)...
  56. ncbi Evidence for protein X binding to a discontinuous epitope on the cellular prion protein during scrapie prion propagation
    K Kaneko
    Department of Neurology, University of California, San Francisco, CA 94143, USA
    Proc Natl Acad Sci U S A 94:10069-74. 1997
    ..provisionally designated protein X participates in the formation of nascent scrapie isoform of prion protein (PrPSc)...
  57. ncbi NMR structure of the mouse prion protein domain PrP(121-231)
    R Riek
    Institut fur Molekularbiologie und Biophysik, Eidgenossische Technische Hochschule Honggerberg, Zurich, Switzerland
    Nature 382:180-2. 1996
    ..are thought to exist in two different conformations: the 'benign' PrPcform, and the infectious 'scrapie form', PrPsc. Knowledge of the three-dimensional structure of PrPc is essential for understanding the transition to PrPsc...
  58. ncbi A mouse prion protein transgene rescues mice deficient for the prion protein gene from purkinje cell degeneration and demyelination
    N Nishida
    Department of Bacteriology, Nagasaki University School of Medicine, Japan
    Lab Invest 79:689-97. 1999
    Disruption of both alleles of the prion protein gene, Prnp, renders mice resistant to prions; in a Prnp o/o line reported by some of us, mice progressively developed ataxia and Purkinje cell loss...
  59. ncbi Reduced translocation of nascent prion protein during ER stress contributes to neurodegeneration
    Neena S Rane
    Cell Biology and Metabolism Program, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA
    Dev Cell 15:359-70. 2008
    During acute stress in the endoplasmic reticulum (ER), mammalian prion protein (PrP) is temporarily prevented from translocation into the ER and instead routed directly for cytosolic degradation...
  60. ncbi Conversion of raft associated prion protein to the protease-resistant state requires insertion of PrP-res (PrP(Sc)) into contiguous membranes
    Gerald S Baron
    Laboratory of Persistent Viral Diseases, NIAID, NIH, Rocky Mountain Laboratories, 903 South 4th Street, Hamilton, MT 59840, USA
    EMBO J 21:1031-40. 2002
    Prion protein (PrP) is usually attached to membranes by a glycosylphosphatidylinositol-anchor that associates with detergent-resistant membranes (DRMs), or rafts...
  61. ncbi Stress-inducible protein 1 is a cell surface ligand for cellular prion that triggers neuroprotection
    Silvio M Zanata
    Ludwig Institute for Cancer Research, Sao Paulo Branch, Rua Prof Antônio Prudente 109 4A, São Paulo 01509010, Brasil
    EMBO J 21:3307-16. 2002
    Prions are composed of an isoform of a normal sialoglycoprotein called PrP(c), whose physiological role has been under investigation, with focus on the screening for ligands...
  62. ncbi Prion protein-deficient neurons reveal lower glutathione reductase activity and increased susceptibility to hydrogen peroxide toxicity
    A R White
    Department of Pathology, University of Melbourne, Melbourne, Australia
    Am J Pathol 155:1723-30. 1999
    The prion protein (PrP) has a central role in the pathogenesis of transmissible spongiform encephalopathies (TSE)...
  63. ncbi Analysis of protein levels of 24 cytokines in scrapie agent-infected brain and glial cell cultures from mice differing in prion protein expression levels
    Déborah Tribouillard-Tanvier
    Laboratory of Persistent Viral Diseases, Rocky Mountain Laboratories, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Hamilton, MT 59840, USA
    J Virol 83:11244-53. 2009
    ..Scrapie agent-infected wild-type mice and transgenic mice expressing anchorless prion protein (PrP) had similar cytokine responses in spite of extensive differences in neuropathology...
  64. ncbi Amino acid conditions near the GPI anchor attachment site of prion protein for the conversion and the GPI anchoring
    Masaki Hizume
    Division of CJD Science and Technology, Department of Prion Research, Tohoku University Graduate School of Medicine, Sendai 980 8575, Japan
    Biochem Biophys Res Commun 391:1681-6. 2010
    Prion protein (PrP) is a glycosylphosphatidylinositol (GPI)-anchored protein, and the C-terminal GPI anchor signal sequence (GPI-SS) of PrP is cleaved before GPI anchoring...
  65. ncbi Prion protein expression by mouse dendritic cells is restricted to the nonplasmacytoid subsets and correlates with the maturation state
    Gloria Martinez del Hoyo
    Department of Immunology and Oncology, Centro Nacional de Biotecnología Consejo Superior de Investigaciones Científicas, Universidad Autonoma, Madrid, Spain
    J Immunol 177:6137-42. 2006
    Expression of the physiological cellular prion protein (PrP(C)) is remarkably regulated during differentiation and activation of cells of the immune system...
  66. ncbi Cellular prion protein modulates defensive attention and innate fear-induced behaviour evoked in transgenic mice submitted to an agonistic encounter with the tropical coral snake Oxyrhopus guibei
    Bruno Lobão-Soares
    Laboratory of Neuroanatomy and Neuropsychobiology, Department of Pharmacology, School of Medicine of Ribeirão Preto of the University of São Paulo USP, Av dos Bandeirantes 3900, Ribeirao Preto, Sao Paulo SP, 14049 900, Brazil
    Behav Brain Res 194:129-37. 2008
    ..involvement of the PrP(C) in the innate fear-induced behavioural reactions in wild-type (WT), PrP(C) knockout (Prnp(0/0)) and the PrP(C) overexpressing Tg-20 mice evoked in a prey versus predator paradigm...
  67. ncbi Live cell fluorescence resonance energy transfer predicts an altered molecular association of heterologous PrPSc with PrPC
    Suparna Mallik
    Department of Neurology, The University of Chicago, Chicago, Illinois 60637, USA
    J Biol Chem 285:8967-75. 2010
    Prion diseases result from the accumulation of a misfolded isoform (PrP(Sc)) of the normal host prion protein (PrP(C)). PrP(Sc) propagates by templating its conformation onto resident PrP(C) to generate new PrP(Sc)...
  68. ncbi Structural insights into the interaction between prion protein and nucleic acid
    Luis Mauricio T R Lima
    Faculdade de Farmacia, Universidade Federal do Rio de Janeiro, 21941 590 Rio de Janeiro, RJ Brazil
    Biochemistry 45:9180-7. 2006
    ..of transmissible spongiform encephalopathies (TSE) is believed to comprise, at least in part, the prion protein (PrP)...
  69. ncbi Amyloid fibrils of mammalian prion protein are highly toxic to cultured cells and primary neurons
    Vera Novitskaya
    Medical Biotechnology Center, University of Maryland Biotechnology Institute, Baltimore, Maryland 21201, USA
    J Biol Chem 281:13828-36. 2006
    ..The cell death caused by rPrP fibrils or beta-oligomers was substantially reduced when expression of endogenous PrP(C) was down-regulated by small interfering RNAs...
  70. ncbi Overexpression of cellular prion protein induces an antioxidant environment altering T cell development in the thymus
    Evelyne Jouvin-Marche
    Laboratoire d Immunochimie, Commissariat a l Energie Atomique, Institut National de la Santé et de la Recherche Médicale Unité 548, Universite J Fourier, 17 rue des Martyrs, 38054 Grenoble, France
    J Immunol 176:3490-7. 2006
    Cellular prion protein (PrP(C)) is an ubiquitously expressed glycoprotein whose roles are still widely discussed, particularly in the field of immunology...
  71. ncbi Prion protein regulates glutamate-dependent lactate transport of astrocytes
    Ralf Kleene
    Zentrum für Molekulare Neurobiologie, Universitat Hamburg, 20246 Hamburg, Germany
    J Neurosci 27:12331-40. 2007
    Prion-related protein (PrP) is a neural cell adhesion molecule involved in neurite outgrowth, neuronal survival, and synaptic function...
  72. ncbi Reciprocal remodeling upon binding of the prion protein to its signaling partner hop/STI1
    Sebastian A Romano
    Instituto de Biofisica da UFRJ, CCS, Bloco G, Cidade Universitaria, RJ 21941 902, Brazil
    FASEB J 23:4308-16. 2009
    The glycosylphosphatidylinositol (GPI)-anchored prion protein (PrP(C)), usually associated with neurodegenerative diseases, modulates various cellular responses and may scaffold multiprotein cell surface signaling complexes...
  73. ncbi Hydrogen/deuterium exchange mass spectrometry identifies two highly protected regions in recombinant full-length prion protein amyloid fibrils
    Alexis Nazabal
    Department of Chemistry and Applied Biosciences, ETH Zurich, Wolfgang Pauli Strasse 10, 8093 Zurich, Switzerland
    J Mass Spectrom 44:965-77. 2009
    ..form with physicochemical properties reminiscent to those of the disease-related isoform of the prion protein, PrPSc. To study the tertiary and quaternary structural organization within recombinant amyloid fibrils from mouse, mPrP(..
  74. ncbi Dynamics and local ordering of spin-labeled prion protein: an ESR simulation study of a highly PH-sensitive site
    Yun Wei Chiang
    Department of Chemistry, National Tsing Hua University, Hsinchu 30013, Taiwan
    J Biomol Struct Dyn 26:355-66. 2008
    ..It suggests a more mobile and disordered dynamic molecular structure for mouse PrPC, which is very likely correlated with increased beta-sheet content at low pH, as the environment changes from ..
  75. ncbi Cellular prion protein co-localizes with nAChR beta4 subunit in brain and gastrointestinal tract
    S Petrakis
    Prion Disease Research Group, Laboratory of Pharmacology, School of Pharmaceutical Sciences, Aristotle University of Thessaloniki, 54124 Thessaloniki, Greece
    Eur J Neurosci 27:612-20. 2008
    b>PrP(C), the cellular isoform of prion protein, is widely expressed in most tissues, including brain, muscle and gastrointestinal tract. Despite its involvement in several bioprocesses, PrP has still no apparent physiological role...
  76. ncbi Deconvoluting the Cu2+ binding modes of full-length prion protein
    Mark Klewpatinond
    School of Biological and Chemical Sciences, Queen Mary, University of London, Mile End Road, London, E1 4NS, United Kingdom
    J Biol Chem 283:1870-81. 2008
    The prion protein (PrP) is a cell-surface Cu(2+)-binding glycoprotein that when misfolded is responsible for a number of transmissible spongiform encephalopathies...
  77. ncbi PrP N-terminal domain triggers PrP(Sc)-like aggregation of Dpl
    Paul Erlich
    Laboratoire Adaptation et Pathogénie des Micro Organismes, Universite Joseph Fourier, BP 170, 38042 Grenoble Cedex 9, France
    Biochem Biophys Res Commun 365:478-83. 2008
    ..thought to be transmitted by self-perpetuating conformational conversion of a neuronal membrane glycoprotein (PrP(C), for "cellular prion protein") into an abnormal state (PrP(Sc), for "scrapie prion protein")...
  78. ncbi Octapeptide repeat region of prion protein (PrP) is required at an early stage for production of abnormal prion protein in PrP-deficient neuronal cell line
    Akikazu Sakudo
    Department of Virology, Center for Infectious Disease Control, Research Institute for Microbial Diseases, Osaka University, Yamadaoka, Suita, Osaka 565 0871, Japan
    Biochem Biophys Res Commun 365:164-9. 2008
    An abnormal isoform of prion protein (PrP(Sc)), which is composed of the same amino acids as cellular PrP (PrP(C)) and has proteinase K (PK)-resistance, hypothetically converts PrP(C) into PrP(Sc)...
  79. ncbi Identification of adjacent binding sites for the YY1 and E4BP4 transcription factors in the ovine PrP (Prion) gene promoter
    Stewart T G Burgess
    Roslin Institute and R D SVS, Neuropathogenesis Division, University of Edinburgh, Roslin, Midlothian EH25 9PS, United Kingdom
    J Biol Chem 284:6716-24. 2009
    The PrP gene encodes the cellular isoform of the prion protein (PrP(c)) which has been shown to be crucial to the development of transmissible spongiform encephalopathies (TSEs)...
  80. ncbi Dual polarisation interferometry analysis of copper binding to the prion protein: evidence for two folding states
    Andrew R Thompsett
    Department of Biology and Biochemistry, University of Bath, Claverton Down, Bath, BA2 7AY, UK
    Biochim Biophys Acta 1774:920-7. 2007
    ..This technique could be important not only for the study of metal-protein interactions but also small structural differences that could define prion strains...
  81. ncbi Unexpected tolerance of alpha-cleavage of the prion protein to sequence variations
    José B Oliveira-Martins
    Institute of Neuropathology, University Hospital of Zurich, Zurich, Switzerland
    PLoS ONE 5:e9107. 2010
    The cellular form of the prion protein, PrP(C), undergoes extensive proteolysis at the alpha site (109K [see text]H110)...
  82. ncbi Contributions of neuronal prion protein on sleep recovery and stress response following sleep deprivation
    Manuel Sanchez-Alavez
    Molecular and Integrative Neuroscience Department, The Scripps Research Institute, 10550 North Torrey Pines Road, La Jolla, CA 92037, USA
    Brain Res 1158:71-80. 2007
    In order to gain insights on the function of the cellular prion protein (PrP(C)) sleep and the levels of the stress hormones corticosterone (CORT) and the adrenocorticotropic hormone (ACTH) before and after sleep deprivation (SD) were ..
  83. ncbi Reduced susceptibility to bovine spongiform encephalopathy prions in transgenic mice expressing a bovine PrP with five octapeptide repeats
    Alejandro Brun
    Centro de Investigacion en Sanidad Animal, INIA, Valdeolmos, 28130 Madrid, Spain
    J Gen Virol 88:1842-9. 2007
    ..These results raise the possibility of selecting cattle breeds bearing the 5OR Prnp allele that are less susceptible to prion infection.
  84. ncbi Copper binding sites in the C-terminal domain of mouse prion protein: A hybrid (QM/MM) molecular dynamics study
    Maria Carola Colombo
    Laboratory of Computational Chemistry and Biochemistry, Institute of Chemical Sciences and Engineering, EPFL, CH 1015 Lausanne, Switzerland
    Proteins 70:1084-98. 2008
    ..Moreover, a reinterpretation of the experimental proton hyperfine couplings was possible in the light of the present computational findings...
  85. ncbi Altered glycosylated PrP proteins can have different neuronal trafficking in brain but do not acquire scrapie-like properties
    Enrico Cancellotti
    Neuropathogenesis Unit, Institute for Animal Health, Ogston Building, West Mains Road, Edinburgh EH9 3JF, United Kingdom
    J Biol Chem 280:42909-18. 2005
    ..Most importantly, we found that, in vivo, unglycosylated PrP does not acquire the characteristics of the aberrant pathogenic form (PrPSc), as was previously reported using in vitro models.
  86. ncbi cDNA cloning of turtle prion protein
    T Simonic
    Istituto di Fisiologia Veterinaria e Biochimica, Universita di Milano, Via Celoria 10, 20133, Milan, Italy
    FEBS Lett 469:33-8. 2000
    ..The common structural features of the known prion proteins are all conserved in turtle PrP, whose identity degree to mammalian and avian proteins is about 40 and 58%, respectively...
  87. ncbi The spectrum of transmissible spongiform encephalopathies
    T Weber
    Neurologische Klinik, Marienkrankenhaus Hamburg, Deutschland
    Intervirology 40:198-212. 1997
    ..The gene for the physiological isoform of this protein (prion related protein or PrPc)-is encoded on the short arm of chromosome 20 in humans. The role of the physiological isoform of PrPc is unknown...
  88. ncbi Calpain-dependent endoproteolytic cleavage of PrPSc modulates scrapie prion propagation
    Rajgopal Yadavalli
    Department of Microbiology, University of Kentucky, Lexington, Kentucky 40536, USA
    J Biol Chem 279:21948-56. 2004
    Previous studies using post-mortem human brain extracts demonstrated that PrP in Creutzfeldt-Jakob disease (CJD) brains is cleaved by a cellular protease to generate a C-terminal fragment, referred to as C2, which has the same molecular ..
  89. ncbi Autocatalytic conversion of recombinant prion proteins displays a species barrier
    Ilia V Baskakov
    Medical Biotechnology Center, University of Maryland Biotechnology Institute, Baltimore, Maryland 21201, USA
    J Biol Chem 279:7671-7. 2004
    ..unorthodox feature of the prion disease is the existence of an abnormal infectious isoform of the prion protein, PrP(Sc)...
  90. ncbi The C-terminal globular domain of the prion protein is necessary and sufficient for import into the endoplasmic reticulum
    Johanna Heske
    Department of Cellular Biochemistry, Max Planck Institut fur Biochemie, Am Klopferspitz 18, D 82152 Martinsried, Germany
    J Biol Chem 279:5435-43. 2004
    The mammalian prion protein (PrP) is composed of an unstructured flexible N-terminal region and a C-terminal globular domain...
  91. ncbi Amino terminal interaction in the prion protein identified using fusion to green fluorescent protein
    Yongxiu Yao
    School of Animal and Microbial Sciences, The University of Reading, Reading RG6 6AJ, UK
    J Neurochem 87:1057-65. 2003
    ..This previously unreported interaction may be significant for understanding prion bioactivity and for structural studies aimed at the complete prion structure...
  92. ncbi Depleting neuronal PrP in prion infection prevents disease and reverses spongiosis
    Giovanna Mallucci
    Medical Research Council Prion Unit and Department of Neurodegenerative Disease, Institute of Neurology, Queen Square, London WC1, UK
    Science 302:871-4. 2003
    The mechanisms involved in prion neurotoxicity are unclear, and therapies preventing accumulation of PrPSc, the disease-associated form of prion protein (PrP), do not significantly prolong survival in mice with central nervous system ..
  93. ncbi Unexpected high testis-specific transcriptional activity of the cyclin T1 promoter in transgenic mice
    X Mata
    Laboratoire de Génétique Biochimique et de Cytogénétique, INRA, 78352 Cedex, Jouy en Josas, France
    FEBS Lett 549:163-6. 2003
    ..To assess if its promoter could target ubiquitous expression of the bovine Prnp in transgenic mice, two constructs carrying either 1 or 30 kb of cyclin T1 5'-flanking sequences were built and ..
  94. ncbi Characterization of a prion protein (PrP) gene from rabbit; a species with apparent resistance to infection by prions
    B Loftus
    Zoology Department, University College, Dublin, Ireland
    Gene 184:215-9. 1997
    The prion protein gene (PrP) encodes a cellular protein of unknown function. A conformational isoform of this protein is involved in the neurodegenerative prion diseases...
  95. ncbi Identification of genetic loci affecting mouse-adapted bovine spongiform encephalopathy incubation time in mice
    Sarah E Lloyd
    MRC Prion Unit, Department of Neurodegenerative Diseases, Institute of Neurology, University College, London, W21N 3BG, UK
    Neurogenetics 4:77-81. 2002
    ..This provides hope that it may be possible to identify human quantitative trait loci for prion incubation time using mouse models that may allow identification of at-risk individuals and the discovery of novel therapeutic targets...
  96. ncbi Changes in the glycosylation pattern of prion protein in murine scrapie. Implications for the mechanism of neurodegeneration in prion diseases
    Milene Russelakis-Carneiro
    Serono Pharmaceutical Research Institute, 14, Chemin des Aulx, 1228 Geneva, Switzerland
    J Biol Chem 277:36872-7. 2002
    In prion diseases, the normal prion protein (PrP(c)) undergoes a conformational change that results in the abnormal form, named scrapie prion protein (PrP(sc))...
  97. ncbi Ablation of cellular prion protein expression affects mitochondrial numbers and morphology
    Gino Miele
    Department of Gene Expression and Development, Roslin Institute, Roslin, Midlothian, Scotland, United Kingdom
    Biochem Biophys Res Commun 291:372-7. 2002
    The cellular prion protein (PrP(C)), predominantly expressed in the central nervous system, is required for pathogenesis of prion neurodegenerative diseases and its conversion into a pathogenic isoform (PrP(Sc)) is a common feature of ..
  98. ncbi Molecular studies of prion diseases
    J Safar
    Department of Neurology, University of California, San Francisco, USA
    Prog Brain Res 117:421-34. 1998
  99. ncbi Cloning, central nervous system expression and chromosomal mapping of the mouse PAK-1 and PAK-3 genes
    P D Burbelo
    Lombardi Cancer Center, Georgetown University Medical Center, Washington, DC 20007, USA
    Gene 232:209-15. 1999
    ..The high level of PAK-1 and PAK-3 kinase expression in the mouse brain and spinal cord suggests a potentially important role for these kinases in the control of the cellular architecture and/or signaling in the central nervous system...
  100. ncbi Copper converts the cellular prion protein into a protease-resistant species that is distinct from the scrapie isoform
    E Quaglio
    Department of Cell Biology and Physiology, Washington University School of Medicine, St Louis, Missouri 63110, USA
    J Biol Chem 276:11432-8. 2001
    Several lines of evidence have suggested that copper ions play a role in the biology of both PrP(C) and PrP(Sc), the normal and pathologic forms of the prion protein...
  101. ncbi Prion protein peptide neurotoxicity can be mediated by astrocytes
    D R Brown
    Department of Biochemistry, Cambridge University, England
    J Neurochem 73:1105-13. 1999
    ..However, PrP106-126 was toxic to cerebellar cells derived from Prnp(0/0) mice (deficient in PrPc expression) when those cerebellar cells were cocultured with astrocytes...

Research Grants37

  1. Reconstitution of Prion Infectivity
    Ilia Baskakov; Fiscal Year: 2005
    ..The "protein only hypothesis" postulates that an abnormal isoform of PrP (PrPSc) acts as an infectious agent and propagates its pathological conformation using normal isoform (PrPC) as a ..
  2. Reconstitution of Prion Transmission in Cells
    Ilia Baskakov; Fiscal Year: 2003
    ..The ultimate direct proof of the "protein only hypothesis', the reconstitution of prpSc in vitro from noninfectious PrP, has not been achieved yet...
  3. Pathology of dysmyelination and demyelination
    Jack Rosenbluth; Fiscal Year: 2005
    ..We will also use the in vivo model of focal antibody-mediated dysmyelination and demyelination, which we have been working with, to determine whether equivalent abnormalities can be identified in this model of multiple sclerosis. ..
  4. Pathology of dysmyelination and demyelination
    Jack Rosenbluth; Fiscal Year: 2007
    ..These studies may clarify which spcific defects are responsible for functional impairment in human myelin diseases and may provide a basis for treatment or prevention. ..
  5. Experimental Creutzfeldt Jacob Disease
    Laura Manuelidis; Fiscal Year: 2007
    ..with host molecules to provoke pathological changes, and we think this is the origin of host prion protein (PrP) amyloid...
  6. Targeting the apoE/ABeta Interaction as a Novel AD Therapy
    Martin Sadowski; Fiscal Year: 2009
    ..The overall goal of this proposal is to bring this novel therapeutic approach closer to clinical studies and to identify a lead peptidomimetic compound, which could be further developed for safe, long-term application in humans. ..
  7. Pathology of dysmyelination and demyelination
    Jack Rosenbluth; Fiscal Year: 2009
    ..These studies may clarify which spcific defects are responsible for functional impairment in human myelin diseases and may provide a basis for treatment or prevention. ..
  8. Key molecular mechanisms of extraneural pathogenesis and transmission of TSEs
    Mark Zabel; Fiscal Year: 2009
    ..in CD21-expressing lymphoid tissue from deer and 3) our current data that shows murine CD21/35 interacts with PrPSc without its endogenous ligands C3 and C4...
  9. Targeting the apoE/ABeta Interaction as a Novel AD Therapy
    MARTIN JOSEPH SADOWSKI; Fiscal Year: 2010
    ..The overall goal of this proposal is to bring this novel therapeutic approach closer to clinical studies and to identify a lead peptidomimetic compound, which could be further developed for safe, long-term application in humans. ..
  10. Key molecular mechanisms of extraneural pathogenesis and transmission of TSEs
    Mark D Zabel; Fiscal Year: 2010
    ..in CD21-expressing lymphoid tissue from deer and 3) our current data that shows murine CD21/35 interacts with PrPSc without its endogenous ligands C3 and C4...
  11. Experimental Creutzfeldt Jacob Disease
    Laura Manuelidis; Fiscal Year: 2010
    ..at very early asymptomatic stages, and only later does one find secondary patholgocial changes in prion protein (PrP)...
  12. Therapeutic Monoclonal Antibodies for Human Prion Diseases
    MARTIN JOSEPH SADOWSKI; Fiscal Year: 2010
    ..with a conformational transformation of cellular prion protein PrPC into a toxic, infectious, and self-replicating PrPSC conformer for which no effective treatment is currently available...
  13. EXPERIMENTAL CREUTZFELDT-JAKOB DISEASE
    Laura Manuelidis; Fiscal Year: 1999
    ..These are currently being analyzed. If the method is as robust as it appears to be, it can be applied for the discovery of other unsuspected or new RNA viruses that participate in late-onset dementias. ..
  14. EXPERIMENTAL CREUTZFELDT-JAKOB DISEASE
    Laura Manuelidis; Fiscal Year: 1993
    ..We will also investigate minor protein species using 2-D electrophoresis and other methods with high sensitivity in order to identify CJD-specific proteins that are recovered under several experimental conditions...
  15. ABNORMALITIES IN CNS MYELINATED FIBERS
    Jack Rosenbluth; Fiscal Year: 2000
    ....
  16. Targeting the apoE/ABeta Interaction as a Novel AD Therapy
    MARTIN JOSEPH SADOWSKI; Fiscal Year: 2010
    ..The overall goal of this proposal is to bring this novel therapeutic approach closer to clinical studies and to identify a lead peptidomimetic compound, which could be further developed for safe, long-term application in humans. ..