scrapie

Summary

Summary: A fatal disease of the nervous system in sheep and goats, characterized by pruritus, debility, and locomotor incoordination. It is caused by proteinaceous infectious particles called PRIONS.

Top Publications

  1. 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
  2. ncbi Formation of native prions from minimal components in vitro
    Nathan R Deleault
    Department of Biochemistry, Dartmouth Medical School, Hanover, NH 03755, USA
    Proc Natl Acad Sci U S A 104:9741-6. 2007
  3. ncbi Novel proteinaceous infectious particles cause scrapie
    S B Prusiner
    Science 216:136-44. 1982
  4. ncbi Protease-sensitive scrapie prion protein in aggregates of heterogeneous sizes
    Salit Tzaban
    Department of Molecular Biology, The Hebrew University Hadassah Medical School, and Hadassah University Hospital, Jerusalem 91120, Israel
    Biochemistry 41:12868-75. 2002
  5. ncbi RNA molecules stimulate prion protein conversion
    Nathan R Deleault
    Department of Biochemistry, 7200 Vail Building, Dartmouth Medical School, Hanover, New Hampshire 03755, USA
    Nature 425:717-20. 2003
  6. ncbi Accumulation and dissemination of prion protein in experimental sheep scrapie in the natural host
    Stephen J Ryder
    Department of Pathology, Veterinary Laboratories Agency, Woodham Lane, New Haw, Addlestone, Surrey KT153NB, UK
    BMC Vet Res 5:9. 2009
  7. ncbi Sheep with scrapie and mastitis transmit infectious prions through the milk
    Ciriaco Ligios
    Istituto Zooprofilattico Sperimentale della Sardegna, Sassari, Italy
    J Virol 85:1136-9. 2011
  8. ncbi Strain-specified relative conformational stability of the scrapie prion protein
    D Peretz
    Institute for Neurodegenerative Diseases, University of California, San Francisco, California 94143, USA
    Protein Sci 10:854-63. 2001
  9. ncbi In vitro amplification of PrPSc derived from the brain and blood of sheep infected with scrapie
    Leigh Thorne
    Department of Molecular Pathogenesis and Genetics, Veterinary Laboratories Agency, New Haw, Addlestone, Surrey KT15 3NB, UK
    J Gen Virol 89:3177-84. 2008
  10. ncbi Strain typing of German transmissible spongiform encephalopathies field cases in small ruminants by biochemical methods
    A Gretzschel
    Friedrich-Loeffler-Institut (FLI, Institute for Novel and Emerging Diseases, Insel Riems, Germany
    J Vet Med B Infect Dis Vet Public Health 52:55-63. 2005

Research Grants

  1. Impact of Microparticles on Oral TSE Infections
    Judd Aiken; Fiscal Year: 2009
  2. Impact of Microparticles on Oral TSE Infections
    Joel A Pedersen; Fiscal Year: 2010
  3. Characteristics of Prion Entry and Neuroinvasion Following Nasal Cavity Infection
    Anthony E Kincaid; Fiscal Year: 2010
  4. Characterization of de novo generation of infectious prions
    Joaquin Castilla; Fiscal Year: 2009
  5. Characterization of prion protein conformational changes
    Valerie Daggett; Fiscal Year: 2007
  6. Characterization of prion protein conformational changes
    VALERIE D DAGGETT; Fiscal Year: 2010
  7. Role of Prion Protein in Manganese Neurotoxicity
    ANUMANTHA GOUNDER KANTHASAMY; Fiscal Year: 2010
  8. Key molecular mechanisms of extraneural pathogenesis and transmission of TSEs
    Mark Zabel; Fiscal Year: 2009
  9. Key molecular mechanisms of extraneural pathogenesis and transmission of TSEs
    Mark D Zabel; Fiscal Year: 2010
  10. Species Susceptibility Assay for Chronic Wasting Disease
    Surachai Supattapone; Fiscal Year: 2005

Detail Information

Publications306 found, 100 shown here

  1. 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
    ..The transmissible agent (prion) responsible for scrapie is believed to be a modified form of PrPC. Mice homozygous for disrupted PrP genes have been generated...
  2. ncbi Formation of native prions from minimal components in vitro
    Nathan R Deleault
    Department of Biochemistry, Dartmouth Medical School, Hanover, NH 03755, USA
    Proc Natl Acad Sci U S A 104:9741-6. 2007
    ..appears to play a critical role in the pathogenesis of prion diseases such as Creutzfeldt-Jakob disease and scrapie. However, the fundamental mechanism by which infectious prions are produced in neurons remains unknown...
  3. ncbi Novel proteinaceous infectious particles cause scrapie
    S B Prusiner
    Science 216:136-44. 1982
    After infection and a prolonged incubation period, the scrapie agent causes a degenerative disease of the central nervous system in sheep and goats...
  4. ncbi Protease-sensitive scrapie prion protein in aggregates of heterogeneous sizes
    Salit Tzaban
    Department of Molecular Biology, The Hebrew University Hadassah Medical School, and Hadassah University Hospital, Jerusalem 91120, Israel
    Biochemistry 41:12868-75. 2002
    ..The small PrP(Sc) aggregates described here are consistent with the previous demonstration of scrapie infectivity in brain fractions with a sedimentation coefficient as small as 40 S [Prusiner et al. (1980) J...
  5. ncbi RNA molecules stimulate prion protein conversion
    Nathan R Deleault
    Department of Biochemistry, 7200 Vail Building, Dartmouth Medical School, Hanover, New Hampshire 03755, USA
    Nature 425:717-20. 2003
    ..They also provide a practical approach to improve the sensitivity of diagnostic techniques based on PrPres amplification...
  6. ncbi Accumulation and dissemination of prion protein in experimental sheep scrapie in the natural host
    Stephen J Ryder
    Department of Pathology, Veterinary Laboratories Agency, Woodham Lane, New Haw, Addlestone, Surrey KT153NB, UK
    BMC Vet Res 5:9. 2009
    In order to study the sites of uptake and mechanisms of dissemination of scrapie prions in the natural host under controlled conditions, lambs aged 14 days and homozygous for the VRQ allele of the PrP gene were infected by the oral route...
  7. ncbi Sheep with scrapie and mastitis transmit infectious prions through the milk
    Ciriaco Ligios
    Istituto Zooprofilattico Sperimentale della Sardegna, Sassari, Italy
    J Virol 85:1136-9. 2011
    ..Here, we show that sheep with scrapie and lentiviral mastitis secrete prions into the milk and infect nearly 90% of naïve suckling lambs...
  8. ncbi Strain-specified relative conformational stability of the scrapie prion protein
    D Peretz
    Institute for Neurodegenerative Diseases, University of California, San Francisco, California 94143, USA
    Protein Sci 10:854-63. 2001
    ..Based on these results, the eight prion strains segregated into four distinct groups. Our results support the unorthodox proposal that distinct PrP(Sc) conformers encipher the biological properties of prion strains...
  9. ncbi In vitro amplification of PrPSc derived from the brain and blood of sheep infected with scrapie
    Leigh Thorne
    Department of Molecular Pathogenesis and Genetics, Veterinary Laboratories Agency, New Haw, Addlestone, Surrey KT15 3NB, UK
    J Gen Virol 89:3177-84. 2008
    b>Scrapie is a fatal, naturally transmissible, neurodegenerative prion disease that affects sheep and goats and is characterized by the accumulation of a misfolded protein, PrPSc, converted from host-encoded PrPc, in the central nervous ..
  10. ncbi Strain typing of German transmissible spongiform encephalopathies field cases in small ruminants by biochemical methods
    A Gretzschel
    Friedrich-Loeffler-Institut (FLI, Institute for Novel and Emerging Diseases, Insel Riems, Germany
    J Vet Med B Infect Dis Vet Public Health 52:55-63. 2005
    ..In this national survey, 186 scrapie-affected sheep were found which originated from 78 flocks...
  11. 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 mice are being used in experiments designed to address the role of PrP in the pathogenesis of scrapie and the replication of infectivity.
  12. ncbi Strain characterization of natural sheep scrapie and comparison with BSE
    Moira E Bruce
    Institute for Animal Health, Neuropathogenesis Unit, Ogston Building, West Mains Road, Edinburgh EH9 3JF, UK
    J Gen Virol 83:695-704. 2002
    b>Scrapie was transmitted to mice from ten sheep, collected in the UK between 1985 and 1994...
  13. ncbi Isolation and characterization of a proteinase K-sensitive PrPSc fraction
    Miguel A Pastrana
    Prion Research Unit, Department of Medicine, School of Medicine, University of Santiago de Compostela, Rue de S. Francisco s/n, Santiago de Compostela, Galiza, Spain 15782
    Biochemistry 45:15710-7. 2006
    ..PK-sensitive PrPSc isolates should prove useful for structural studies to help understand fundamental issues of the molecular biology of PrPSc and in the quest to design tests to detect preclinical prion disease...
  14. 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...
  15. ncbi Follicular dendritic cell dedifferentiation by treatment with an inhibitor of the lymphotoxin pathway dramatically reduces scrapie susceptibility
    Neil A Mabbott
    Neuropathogenesis Unit, Institute for Animal Health, Edinburgh EH9 3JF, Scotland, United Kingdom
    J Virol 77:6845-54. 2003
    ..Studies of mouse scrapie models have shown that mature follicular dendritic cells (FDCs), expressing the host prion protein (PrP(c)), are ..
  16. ncbi Sympathetic innervation of lymphoreticular organs is rate limiting for prion neuroinvasion
    M Glatzel
    Institute of Neuropathology, University Hospital Zurich, Schmelzbergstrasse 12, CH-8091, Zurich, Switzerland
    Neuron 31:25-34. 2001
    ..Following intraperitoneal prion inoculation, chemical or immunological sympathectomy delayed or prevented scrapie. Prion titers in spinal cords were drastically reduced at early time points after inoculation...
  17. ncbi Secondary structure analysis of the scrapie-associated protein PrP 27-30 in water by infrared spectroscopy
    B W Caughey
    NIAID, National Institutes of Health, Rocky Mountain Laboratories, Hamilton, Montana 59840
    Biochemistry 30:7672-80. 1991
    A protease-resistant form of the protein PrP (PrP-res) accumulates in tissues of mammals infected with scrapie, Creutzfeldt-Jakob disease, and related transmissible neurodegenerative diseases...
  18. ncbi Atypical/Nor98 scrapie infectivity in sheep peripheral tissues
    Olivier Andreoletti
    UMR INRA ENVT 1225, Interactions Hôtes Agents Pathogènes, Ecole Nationale Veterinaire de Toulouse, Toulouse, France
    PLoS Pathog 7:e1001285. 2011
    Atypical/Nor98 scrapie was first identified in 1998 in Norway...
  19. ncbi The natural atypical scrapie phenotype is preserved on experimental transmission and sub-passage in PRNP homologous sheep
    Marion M Simmons
    Department of Pathology, Veterinary Laboratories Agency Weybridge, New Haw, Addlestone KT15 3NB, UK
    BMC Vet Res 6:14. 2010
    Atypical scrapie was first identified in Norwegian sheep in 1998 and has subsequently been identified in many countries...
  20. ncbi Prion diseases are efficiently transmitted by blood transfusion in sheep
    Fiona Houston
    Neuropathogenesis Division, Roslin Institute, Compton, United Kingdom
    Blood 112:4739-45. 2008
    ..We previously reported preliminary data demonstrating the transmission of BSE and natural scrapie by blood transfusion in sheep...
  21. ncbi The physical relationship between infectivity and prion protein aggregates is strain-dependent
    Philippe Tixador
    INRA Institut National de la recherche Agronomique, UR892, Virologie Immunologie Moléculaires, Jouy en Josas, France
    PLoS Pathog 6:e1000859. 2010
    ..to a panel of natural, biologically cloned strains obtained by propagation of classical and atypical sheep scrapie and BSE infectious sources in transgenic mice expressing ovine PrP...
  22. ncbi Neuroinvasion in sheep transmissible spongiform encephalopathies: the role of the haematogenous route
    S Siso
    Department of Pathology, Veterinary Laboratories Agency, Bush Loan, Penicuik, Midlothian, UK
    Neuropathol Appl Neurobiol 35:232-46. 2009
    ....
  23. ncbi A novel method for preclinical detection of PrPSc in blood
    Richard Rubenstein
    Departments of Neurology and Physiology Pharmacology, SUNY Downstate Medical Center, 450 Clarkson Avenue, Brooklyn, NY 11203, USA
    J Gen Virol 91:1883-92. 2010
    ..disease-associated form of the prion protein (PrP(Sc)) in protease-untreated plasma from preclinical and clinical scrapie sheep, and white-tailed deer with chronic wasting disease, following natural and experimental infection...
  24. ncbi PrP gene polymorphisms in Cyprus goats and their association with resistance or susceptibility to natural scrapie
    Penelope Papasavva-Stylianou
    TSE Section, Laboratory of Animal Health, State Veterinary Laboratories, Veterinary Services, 1417 Nicosia, Cyprus
    Vet J 187:245-50. 2011
    In contrast to scrapie in sheep, the genetic basis of susceptibility to scrapie in goats is not well understood...
  25. ncbi State-of-the-art review of goat TSE in the European Union, with special emphasis on PRNP genetics and epidemiology
    Gabriele Vaccari
    Department of Food Safety and Veterinary Public Health, Unit of Transmissible Spongiform Encephalopathies and Emerging Infectious Diseases of Animals, Istituto Superiore di Sanita, Viale Regina Elena 299, 00161, Rome, Italy
    Vet Res 40:48. 2009
    b>Scrapie is a fatal, neurodegenerative disease of sheep and goats...
  26. ncbi Prion strain discrimination in cell culture: the cell panel assay
    Sukhvir P Mahal
    Department of Infectology, Scripps Florida, 5353 Parkside Drive, Jupiter, FL 33458, USA
    Proc Natl Acad Sci U S A 104:20908-13. 2007
    ..lines, N2a-PK1, N2a-R33, LD9 and CAD5, which show widely different responses to prion strains RML, 22L, 301C, and Me7, into a panel that allows their discrimination in vitro within 2 weeks, using the standard scrapie cell assay (SSCA).
  27. ncbi Transportation of prion protein across the intestinal mucosa of scrapie-susceptible and scrapie-resistant sheep
    M Jeffrey
    Veterinary Laboratories Agency VLA Lasswade, Pentlands Science Park, Midlothian, UK
    J Pathol 209:4-14. 2006
    To determine the mechanisms of intestinal transport of infection, and early pathogenesis, of sheep scrapie, isolated gut-loops were inoculated to ensure that significant concentrations of scrapie agent would come into direct contact with ..
  28. ncbi Proteinase K-sensitive disease-associated ovine prion protein revealed by conformation-dependent immunoassay
    Alana M Thackray
    Centre for Veterinary Science, Department of Veterinary Medicine, University of Cambridge, Madingley Road, Cambridge CB3 OES, UK
    Biochem J 401:475-83. 2007
    ..utilizing time-resolved fluorescence to study the conformers of disease-associated PrP in natural cases of sheep scrapie, without using PK (proteinase K) treatment to discriminate between PrPC and PrPSc...
  29. ncbi Oral prion infection requires normal numbers of Peyer's patches but not of enteric lymphocytes
    Marco Prinz
    Institute of Neuropathology, University Hospital of Zurich, Zurich, Switzerland
    Am J Pathol 162:1103-11. 2003
    ..Although susceptibility to prion infection following oral challenge correlates with the number of PPs, it is remarkably independent of the number of PP-associated lymphocytes...
  30. ncbi Disease dynamics over very different time-scales: foot-and-mouth disease and scrapie on the network of livestock movements in the UK
    Rowland R Kao
    Department of Zoology, University of Oxford, Oxford OX1 3PS, UK
    J R Soc Interface 4:907-16. 2007
    ..UK and the dynamics of two diseases: foot-and-mouth disease (FMD), which has an incubation period of days, and scrapie, which incubates over years...
  31. ncbi Faecal shedding, alimentary clearance and intestinal spread of prions in hamsters fed with scrapie
    Dominique Krüger
    Robert Koch Institute, P24 Transmissible Spongiform Encephalopathies, Nordufer 20, D 13353 Berlin, Germany
    Vet Res 40:4. 2009
    ..Here, we fed hamsters 10mg of 263K scrapie brain homogenate and examined the faecal excretion of disease-associated prion protein (PrP(TSE)) during the ..
  32. ncbi Novel polymorphisms at codons 146 and 151 in the prion protein gene of Cyprus goats, and their association with natural scrapie
    Penelope Papasavva-Stylianou
    Virology Laboratory, Veterinary Services, 1417 Nicosia, Cyprus
    Vet J 173:459-62. 2007
    ..an association exists between specific combinations of polymorphisms of the prion protein (PrP) and natural scrapie in Cyprus goats, 250 goats were examined, including 164 histologically positive cases...
  33. ncbi Transfer of scrapie prion infectivity by cell contact in culture
    Nnennaya Kanu
    Department of Biochemistry and Molecular Biology, University College London, Gower Street, WC1E 6BT, London, United Kingdom
    Curr Biol 12:523-30. 2002
    BACKGROUND: When a cell is infected with scrapie prions, newly synthesized molecules of the prion protein PrP(C) are expressed at the cell surface and may subsequently be converted to the abnormal form PrP(Sc)...
  34. ncbi Scrapie infectivity in hamster blood is not associated with platelets
    Karel Holada
    Laboratory of Cellular Hematology, Center for Biologics Evaluation and Research, Food and Drug Administration, Bethesda, Maryland 20892, USA
    J Virol 76:4649-50. 2002
    The infectivity of hamster scrapie strain 263K was measured in platelets isolated from blood pooled from six hamsters with clinical scrapie. The total number of infectious doses present in the blood pool was 220, out of which only 3...
  35. ncbi Post-mortem immunodiagnosis of scrapie and bovine spongiform encephalopathy
    C F Farquhar
    Institute for Animal Health, AFRC and MRC Neuropathogenesis Unit, Edinburgh
    J Virol Methods 24:215-21. 1989
    Two polyclonal antisera were raised in rabbits against the scrapie-associated fibril protein (PrP) prepared from sheep and mice which were terminally infected with experimental scrapie...
  36. ncbi Mice with gene targetted prion protein alterations show that Prnp, Sinc and Prni are congruent
    R C Moore
    Institute of Cell and Molecular Biology, University of Edinburgh, UK
    Nat Genet 18:118-25. 1998
    Classical genetic analysis has identified Sinc/Prni as the major gene controlling mouse scrapie incubation time. Sinc/Prni is linked to Prnp, the gene encoding the prion protein (PrP)...
  37. ncbi The kuru infectious agent is a unique geographic isolate distinct from Creutzfeldt-Jakob disease and scrapie agents
    Laura Manuelidis
    Section of Neuropathology, Yale University Medical School, 333 Cedar Street, New Haven, CT 06510, USA
    Proc Natl Acad Sci U S A 106:13529-34. 2009
    Human sporadic Creutzfeldt-Jakob disease (sCJD), endemic sheep scrapie, and epidemic bovine spongiform encephalopathy (BSE) are caused by a related group of infectious agents. The new U.K...
  38. ncbi Immunohistochemical characterisation of classical scrapie neuropathology in sheep
    E Vidal
    Priocat Laboratory, Centre de Recerca en Sanitat Animal, UAB IRTA, Campus de la Universitat Autonoma de Barcelona, 08193 Bellaterra, Barcelona, Spain
    J Comp Pathol 141:135-46. 2009
    ..was to characterize the pathogenic events occurring in the CNS of sheep with spontaneously arising classical scrapie. Brains from seven affected animals and two controls were subject to immunohistochemical and histochemical ..
  39. ncbi Prions are secreted into the oral cavity in sheep with preclinical scrapie
    Ben C Maddison
    ADAS UK, Department of Biology, University of Leicester, Leicestershire, United Kingdom
    J Infect Dis 201:1672-6. 2010
    ..We analyzed buccal swab samples obtained from preclinical scrapie-infected sheep by concentrating the collected prions on silicon dioxide, followed by amplification by serial ..
  40. ncbi Isolation of proteinase K-sensitive prions using pronase E and phosphotungstic acid
    Laura D'Castro
    MRC Prion Unit and Department of Neurodegenerative Disease, UCL Institute of Neurology, National Hospital for Neurology and Neurosurgery, London, United Kingdom
    PLoS ONE 5:e15679. 2010
    ..This procedure now allows characterization of proteinase K-sensitive prions and investigation of their clinical relevance in human and animal prion disease without being confounded by contaminating PrP(C)...
  41. ncbi Three serial passages of bovine spongiform encephalopathy in sheep do not significantly affect discriminatory test results
    Michael Stack
    Veterinary Laboratories Agency VLA, Woodham Lane, Addlestone, Surrey, UK
    J Gen Virol 90:764-8. 2009
    ..techniques, Western immunoblotting (WB) and immunohistochemistry (IHC), have been developed to distinguish scrapie from cases of experimental sheep BSE by the characteristics of their respective abnormal, disease-associated ..
  42. 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
    ..the cellular location of disease-specific co-factors using fractions derived from gradient centrifugation of a scrapie-susceptible cell line...
  43. ncbi PrPSc accumulation in fetal cotyledons of scrapie-resistant lambs is influenced by fetus location in the uterus
    Janet Alverson
    USDA, ARS, Animal Disease Research Unit, 3003 ADBF, Washington State University, Pullman, WA 99164, USA
    J Gen Virol 87:1035-41. 2006
    Placentae from scrapie-infected ewes have been shown to accumulate PrPSc when the genotype of the fetus is of a susceptible genotype (VRQ/VRQ, ARQ/VRQ or ARQ/ARQ)...
  44. ncbi Prion protein allele A136 H154Q171 is associated with high susceptibility to scrapie in purebred and crossbred German Merinoland sheep
    G Luhken
    Department of Animal Breeding and Genetics, Justus Liebig University, Giessen, Germany
    Arch Virol 149:1571-80. 2004
    Prion protein (PrP) genotypes were determined in eight sheep that have been tested positive for atypical scrapie from purebred or crossbred Merinoland sheep flocks in Germany and compared with the PrP genotypes of their flock mates...
  45. ncbi Effect of scrapie on the stability of housekeeping genes
    J Lyahyai
    Laboratorio de Genetica Bioquimica LAGENBIO, Facultad de Veterinaria, Universidad de Zaragoza, Zaragoza, Spain
    Anim Biotechnol 21:1-13. 2010
    b>Scrapie is the archetype of prion diseases, fatal neurodegenerative disorders that affect humans and animals...
  46. ncbi Highly efficient protein misfolding cyclic amplification
    Nuria Gonzalez-Montalban
    Center for Biomedical Engineering and Technology, University of Maryland, Baltimore, Maryland, United States of America
    PLoS Pathog 7:e1001277. 2011
    ..Using serial PMCAb, a 10¹²-fold dilution of scrapie brain material could be amplified to the level detectible by Western blotting in 3 rounds (72 hours)...
  47. ncbi Caprine PRNP polymorphisms at codons 171, 211, 222 and 240 in a Greek herd and their association with classical scrapie
    Ilias G Bouzalas
    Laboratory of Microbiology and Infectious Diseases, Faculty of Veterinary Medicine, Aristotle University of Thessaloniki, 54124 Thessaloniki, Greece
    J Gen Virol 91:1629-34. 2010
    The association between PRNP variation and scrapie incidence was investigated in a highly affected Greek goat herd. Four mutations were identified at codons 171Q/R, 211R/Q, 222Q/K and 240P/S...
  48. ncbi Molecular biology and pathology of scrapie and the prion diseases of humans
    S B Prusiner
    Department of Neurology, University of California, San Francisco 94143
    Brain Pathol 1:297-310. 1991
    b>Scrapie and bovine spongiform encephalopathy of animals and Creutzfeldt-Jakob and Gerstmann-Sträussler-Scheinker diseases of humans are transmissible and genetic neurodegenerative diseases caused by prions...
  49. ncbi Prion protein (PrP) with amino-proximal deletions restoring susceptibility of PrP knockout mice to scrapie
    M Fischer
    Institut für Molekularbiologie der Universität Zürich, Switzerland
    EMBO J 15:1255-64. 1996
    ..Disruption of the PrP gene in the mouse abolishes susceptibility to scrapie and prion replication...
  50. ncbi Dynamics and genetics of PrPSc placental accumulation in sheep
    C Lacroux
    UMR INRA ENVT 1225, Interactions Hôte Agent Pathogène, Ecole Nationale Veterinaire de Toulouse, 23 Chemin des Capelles, 31076 Toulouse, France
    J Gen Virol 88:1056-61. 2007
    Placentae from scrapie-affected ewes are an important source of contamination...
  51. ncbi Detection of PrPsc in blood from sheep infected with the scrapie and bovine spongiform encephalopathy agents
    L A Terry
    Department of Molecular Pathogenesis and Genetics, Veterinary Laboratories Agency, Woodham Lane, New Haw, Surrey KT15 3NB, United Kingdom
    J Virol 83:12552-8. 2009
    ..In sheep with clinical disease, PrP(sc) was detected in the blood of 55% of scrapie agent-infected animals (n = 80) and 71% of animals with bovine spongiform encephalopathy (n = 7)...
  52. ncbi PrP(Sc) accumulation in placentas of ewes exposed to natural scrapie: influence of foetal PrP genotype and effect on ewe-to-lamb transmission
    Olivier Andreoletti
    UMR 959 INRA ENVT, Physiopathologie Infectieuse et Parasitaire des Ruminants, Ecole Nationale Veterinaire, 23 Chemin des Capelles, 31076 Toulouse Cedex 3, France
    J Gen Virol 83:2607-16. 2002
    Placentas from scrapie-affected ewes are known to be infectious. Nevertheless, placenta infectivity in such ewes is not systematic...
  53. ncbi In vitro generation of infectious scrapie prions
    Joaquin Castilla
    Department of Neurology, University of Texas Medical Branch, Galveston, Texas 77555, USA
    Cell 121:195-206. 2005
    ..Inoculation of wild-type hamsters with in vitro-produced PrPres led to a scrapie disease identical to the illness produced by brain infectious material...
  54. ncbi Complement facilitates early prion pathogenesis
    M A Klein
    Institute of Neuropathology, University of Zurich, Schmelzbergstrasse, Zurich, Switzerland
    Nat Med 7:488-92. 2001
    New-variant Creutzfeldt-Jakob disease and scrapie are typically initiated by extracerebral exposure to the causative agent, and exhibit early prion replication in lymphoid organs...
  55. ncbi Pathogenesis of bovine spongiform encephalopathy in sheep
    L J M van Keulen
    Department of Bacteriology and TSE s, Central Institute for Animal Disease Control CIDC, Wageningen University and Research Centre, P O Box 2004, 8203 AA Lelystad, The Netherlands
    Arch Virol 153:445-53. 2008
    ..The pathogenesis of bovine spongiform encephalopathy (BSE) in sheep was studied by immunohistochemical detection of scrapie-associated prion protein (PrP(Sc)) in the gastrointestinal, lymphoid and neural tissues following oral ..
  56. ncbi Scrapie resistance in ARQ sheep
    W W Laegreid
    University of Illinois, Department of Pathobiology, 2001 S Lincoln Ave, Urbana, IL 61802, USA
    J Virol 82:10318-20. 2008
    Variation in the ovine prion protein amino acid sequence influences scrapie progression, with sheep homozygous for A(136)R(154)Q(171) considered susceptible...
  57. ncbi Molecular analysis of ovine prion protein identifies similarities between BSE and an experimental isolate of natural scrapie, CH1641
    J Hope
    BBSRC Institute for Animal Health, Compton Laboratory, Berkshire, UK
    J Gen Virol 80:1-4. 1999
    ..variation in contemporary and archival brain tissue from cases of experimental BSE or experimental and natural scrapie in sheep...
  58. ncbi Sensitive detection of pathological prion protein by cyclic amplification of protein misfolding
    G P Saborio
    Serono Pharmaceutical Research Institute, Geneva, Switzerland
    Nature 411:810-3. 2001
    ....
  59. ncbi Efficient in vitro amplification of a mouse-adapted scrapie prion protein
    Yuichi Murayama
    Prion Disease Research Center, National Institute of Animal Health, 3 1 5 Kannondai, Tsukuba, Ibaraki 305 0856, Japan
    Neurosci Lett 413:270-3. 2007
    Protein misfolding cyclic amplification (PMCA) is a highly sensitive technique used to detect minute amounts of scrapie prion protein (PrP(Sc)), a major protein component of the infectious agents associated with prion diseases...
  60. ncbi Role of the GALT in scrapie agent neuroinvasion from the intestine
    Bridget R Glaysher
    Neuropathogenesis Unit, Institute for Animal Health, Ogston Building, West Mains Road, Edinburgh, United Kingdom
    J Immunol 178:3757-66. 2007
    ..Mice deficient in specific GALT components were used here to determine their separate involvement in scrapie agent neuroinvasion from the intestine...
  61. ncbi Identification of seven haplotypes of the caprine PrP gene at codons 127, 142, 154, 211, 222 and 240 in French Alpine and Saanen breeds and their association with classical scrapie
    F Barillet
    INRA, UR 631, Station d Amélioration Génétique des Animaux, BP 52627, 31326 Castanet Tolosan Cedex, France
    J Gen Virol 90:769-76. 2009
    In sheep, susceptibility to scrapie is mainly influenced by polymorphisms of the PrP gene. In goats, there are to date few data related to scrapie susceptibility association with PrP gene polymorphisms...
  62. ncbi Prion protein gene polymorphisms in natural goat scrapie
    Charalambos Billinis
    Laboratory of Microbiology and Infectious Diseases, Laboratory of Pathology and Clinic of Productive Animal Medicine, Faculty of Veterinary Medicine, Aristotle University, 54006 Thessaloniki, Greece
    J Gen Virol 83:713-21. 2002
    A total of 51 goats, including seven clinical cases, from the first herd in Greece reported to have scrapie was examined to discern an association between scrapie susceptibility and polymorphisms of the gene encoding the prion protein (..
  63. ncbi Identification of an allelic variant of the goat PrP gene associated with resistance to scrapie
    Gabriele Vaccari
    Department of Food Safety and Veterinary Public Health, Istituto Superiore di Sanita, Rome, Italy
    J Gen Virol 87:1395-402. 2006
    The association between PrP gene variations and scrapie susceptibility was studied in a single herd of Ionica breed goats...
  64. ncbi Identification of prion protein gene polymorphisms in goats from Italian scrapie outbreaks
    P L Acutis
    CEA Centro di referenza per le Encefalopatie Animali, Istituto Zooprofilattico Sperimentale del Piemonte, Liguria e Valle d Aosta, Via Bologna 148, 10154 Turin, Italy
    J Gen Virol 87:1029-33. 2006
    Susceptibility to scrapie in sheep is influenced by polymorphisms of the prion protein (PrP) gene, whereas no strong association between genetics and scrapie has yet been determined in goats due to the limited number of studies on these ..
  65. ncbi Temporary depletion of complement component C3 or genetic deficiency of C1q significantly delays onset of scrapie
    N A Mabbott
    Neuropathogenesis Unit, Institute for Animal Health, Edinburgh, UK
    Nat Med 7:485-7. 2001
    ..centers in lymphoid tissues of patients with variant Creutzfeldt-Jakob disease (vCJD), sheep with natural scrapie or rodents following experimental peripheral infection with scrapie...
  66. ncbi Cytosolic PrP induces apoptosis of cell by disrupting microtubule assembly
    Xiao Li Li
    State Key Laboratory for Infectious Disease Prevention and Control, National Institute for Viral Disease Control and Prevention, Chinese Center for Disease Control and Prevention, Ying Xin Rd 100, Beijing, 100052, People s Republic of China
    J Mol Neurosci 43:316-25. 2011
    ..Moreover, the levels of endogenous tubulin in the brain tissues of scrapie-infected hamsters were significantly lower than that of normal one...
  67. ncbi Overexpression of Shadoo protein in transgenic mice does not impact the pathogenesis of scrapie
    Haiying Wang
    Department of Animal Biotechnology, College of Animal Science and Veterinary Medicine, Jilin University, Changchun 130062, China
    Neurosci Lett 496:1-4. 2011
    ..Following infection with mouse-adapted scrapie strain 22L, all transgenic mice tested exhibited characteristics of scrapie disease...
  68. ncbi Detection of classical and atypical/Nor98 scrapie by the paraffin-embedded tissue blot method
    W M Wemheuer
    Prion and Dementia Research Unit, Department of Neuropathology, University Medical Centre, Gottingen, 37075 Gottingen, Germany
    Vet Rec 164:677-81. 2009
    ..was used to investigate sections of the central nervous system and lymphatic tissues from 24 cases of classical scrapie and 25 cases of atypical/Nor98 scrapie in sheep and four healthy control sheep...
  69. ncbi No associations between PrP genotypes and reproduction traits in INRA 401 sheep
    Z G Vitezica
    Institut National de la Recherche Agronomique, Station d Amélioration Génétique des Animaux, 31326 Castanet Tolosan, France
    J Anim Sci 84:1317-22. 2006
    ..It is unlikely that selection for scrapie resistance will have an effect on the reproductive or performance traits studied in the INRA 401 breed.
  70. ncbi Similarities between forms of sheep scrapie and Creutzfeldt-Jakob disease are encoded by distinct prion types
    Wiebke M Wemheuer
    Prion and Dementia Research Unit, Department of Neuropathology, University Medical Center Goettingen, Goettingen, Germany
    Am J Pathol 175:2566-73. 2009
    Transmissible spongiform encephalopathies such as scrapie in sheep, Creutzfeldt-Jakob disease (CJD) in humans, and bovine sporadic encephalopathy in cattle are characterized by the accumulation of a misfolded protein: the pathological ..
  71. ncbi Anti-prion antibodies for prophylaxis following prion exposure in mice
    Einar M Sigurdsson
    Department of Psychiatry, New York University School of Medicine, Millhauser Laboratory, 550 First Avenue, New York, NY 10016, USA
    Neurosci Lett 336:185-7. 2003
    Prion disease is characterized by a conformational change of the normal form of the prion protein (PrP(C)) to the scrapie-associated form (PrP(Sc))...
  72. ncbi Changes in brain gene expression shared by scrapie and Alzheimer disease
    J R Duguid
    Geriatric Research, Education and Clinical Center, Edith N Rogers Memorial Veterans Hospital, Bedford, MA 01730
    Proc Natl Acad Sci U S A 86:7260-4. 1989
    We have isolated two recombinant cDNAs whose corresponding RNAs have an increased abundance in scrapie-infected hamster brain...
  73. ncbi Assessment of calcium-binding proteins (Parvalbumin and Calbindin D-28K) and perineuronal nets in normal and scrapie-affected adult sheep brains
    Enric Vidal
    Priocat Laboratory, CReSA, Veterinary Faculty, , , Barcelona, Spain
    J Virol Methods 136:137-46. 2006
    b>Scrapie is a prion disease in small ruminants that manifests itself with neurological clinical signs amongst which are ataxia and tremors. These signs can be explained partially by an imbalance in central inhibitory innervation...
  74. ncbi Sparse PrP(Sc) accumulation in the placentas of goats with naturally acquired scrapie
    Katherine I O'Rourke
    Animal Disease Research Unit, Agricultural Research Service, U S Department of Agriculture, Pullman, WA 99164, USA
    BMC Vet Res 7:7. 2011
    Domestic goats (Capra hircus) are a natural and experimental host of scrapie and bovine spongiform encephalopathy, the transmissible spongiform encephalopathies (TSE) of sheep and cattle...
  75. ncbi Changes of tau profiles in brains of the hamsters infected with scrapie strains 263 K or 139 A possibly associated with the alteration of phosphate kinases
    Gui Rong Wang
    State Key Laboratory for Infectious Disease Prevention and Control, National Institute for Viral Disease Control and Prevention, Chinese Center for Disease Control and Prevention, Ying Xin Rd 100, Beijing 100052, PR China
    BMC Infect Dis 10:86. 2010
    ..This study investigated the possible changes of tau and phosphorylated tau (p-tau, at Ser396, Ser404, and Ser202/Thr205) in scrapie experimental animals.
  76. ncbi Insight into the PrPC-->PrPSc conversion from the structures of antibody-bound ovine prion scrapie-susceptibility variants
    Frederic Eghiaian
    Laboratoire d Enzymologie et de Biochimie Structurales, Centre National de la Recherche Scientifique, 91198 Gif sur Yvette, France
    Proc Natl Acad Sci U S A 101:10254-9. 2004
    ..We report here the 2.5-A-resolution crystal structures of three scrapie-susceptibility ovine PrP variants complexed with an antibody that binds to PrPC and to PrPSc; they identify two ..
  77. ncbi Distinct spatial activation of intrinsic and extrinsic apoptosis pathways in natural scrapie: association with prion-related lesions
    Carmen Serrano
    Laboratorio de Genetica Bioquimica LAGENBIO, Facultad de Veterinaria, Universidad de Zaragoza, Miguel Servet 177, 50013 Zaragoza, Spain
    Vet Res 40:42. 2009
    ..in the intrinsic and extrinsic apoptotic pathways in the central nervous systems of sheep naturally infected with scrapie. Expression changes obtained by real-time RT-PCR were also compared with the extent of classical scrapie lesions, ..
  78. ncbi Characterization of atypical scrapie cases from Great Britain in transgenic ovine PrP mice
    Peter C Griffiths
    Molecular Pathogenesis and Genetics Department, Veterinary Laboratories Agency, Addlestone, Surrey KT15 3NB, UK
    J Gen Virol 91:2132-8. 2010
    Twenty-four atypical scrapie cases from sheep with different prion protein genotypes from Great Britain were transmitted to transgenic tg338 and/or TgshpXI mice expressing sheep PrP alleles, but failed to transmit to wild-type mice...
  79. ncbi Acute cellular uptake of abnormal prion protein is cell type and scrapie-strain independent
    Christopher S Greil
    Rocky Mountain Laboratories, Laboratory of Persistent Viral Diseases, NIAID, NIH, 903 S 4th Street, Hamilton, Montana 59840, USA
    Virology 379:284-93. 2008
    ..neurodegenerative diseases that include Creutzfeldt-Jakob disease, bovine spongiform encephalopathy and sheep scrapie. Although one of the earliest events during TSE infection is the cellular uptake of protease resistant prion ..
  80. 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
    ..these issues we have taken a combination of pharmacological and genetic approaches using persistently infected scrapie mouse brain (SMB) cells...
  81. ncbi Polymorphisms of the prion protein gene in sheep of Inner Mongolia, China
    Yiqin Wang
    National Animal Transmissible Spongiform Encephalopathies Laboratory, College of Veterinary Medicine, China Agricultural University, Beijing 100094, China
    Virus Genes 37:128-30. 2008
    ..residue alterations at codons 136, 154, and 174, in sheep have been found to be associated with susceptibility to scrapie disease. We investigated Prnp polymorphisms in three local sheep breeds in Inner Mongolia, China...
  82. ncbi Hyperefficient PrP Sc amplification of mouse-adapted BSE and scrapie strain by protein misfolding cyclic amplification technique
    Aiko Fujihara
    Department of Molecular Microbiology and Immunology, Nagasaki University Graduate School of Biomedical Sciences, Japan
    FEBS J 276:2841-8. 2009
    ..encephalopathy models infected with either mouse-adapted bovine spongiform encephalopathy or mouse-adapted scrapie, Chandler strain...
  83. ncbi Transmissibility of atypical scrapie in ovine transgenic mice: major effects of host prion protein expression and donor prion genotype
    Jean Noel Arsac
    Agence Francaise de Securite Sanitaire des Aliments, Lyon, France
    PLoS ONE 4:e7300. 2009
    Atypical scrapie or Nor98 has been identified as a transmissible spongiform encephalopathy (TSE) that is clearly distinguishable from classical scrapie and BSE, notably regarding the biochemical features of the protease-resistant prion ..
  84. ncbi Abnormal synaptic protein expression and cell death in murine scrapie
    S Siso
    Priocat Laboratory, Centre of Animal Health Research CReSA, Department of Medicine and Animal Surgery, Autonomous University of Barcelona, Bellaterra, Spain
    Acta Neuropathol 103:615-26. 2002
    ..and beta-synuclein, matching the distribution of spongiform degeneration, was found in the neurological phase of scrapie-infected mice...
  85. ncbi Novel polymorphisms in the caprine PrP gene: a codon 142 mutation associated with scrapie incubation period
    W Goldmann
    Institute for Animal Health, BBSRC and MRC Neuropathogenesis Unit, Edinburgh, UK
    J Gen Virol 77:2885-91. 1996
    ..at codon 142 (Ile --> Met) appeared to be associated with differing disease incubation periods in goats experimentally infected with isolates of bovine spongiform encephalopathy, sheep scrapie CH1641 or sheep-passaged ME7 scrapie.
  86. ncbi Histidine at codon 154 of the prion protein gene is a risk factor for Nor98 scrapie in goats
    Silvia Colussi
    Istituto Zooprofilattico Sperimentale del Piemonte, Liguria e Valle d Aosta, Via Bologna 148, Torino, Italy
    J Gen Virol 89:3173-6. 2008
    Prion protein gene (PRNP) polymorphisms are involved in modulating the appearance of atypical/Nor98 scrapie in sheep, with the alleles AHQ and AF141RQ strongly associated with occurrence of the disease...
  87. ncbi PrP genetics in sheep and the applications for scrapie and BSE
    N Hunter
    Institute for Animal Health, BBSRC MRC Neuropathogenesis Unit, Edinburgh, UK
    Trends Microbiol 5:331-4. 1997
    The strong links between PrP genotype and the occurrence of scrapie in sheep strengthen evidence supporting the central importance of the PrP protein in the development of transmissible spongiform encephalopathies, despite the fact that ..
  88. ncbi [Immunity against prions?]
    F L Heppner
    Institut für Neuropathologie, Universitatsspital Zurich, Switzerland
    Dtsch Med Wochenschr 127:328-30. 2002
  89. ncbi [Review of Creutzfeldt-Jakob disease and other prion diseases]
    S Zivkovic
    Department of Neurology, University of Pittsburgh School of Medicine, Pennsylvania, USA
    Rev Neurol 31:1171-9. 2000
    ..CONCLUSIONS: There is still no treatment for CJD. Further studies of the physiopathological mechanisms of prion diseases may help in the development of treatment to delay the progress of this disease...
  90. ncbi [Scrapie of sheep and Creutzfeldt-Jakob disease in Iceland]
    Gudmundur Georgsson
    Laeknabladid 94:541-8. 2008
    b>Scrapie of sheep and Creutzfeldt-Jakob disease (CJD) are both classified as prion diseases. The infectious agents of both diseases are closely related...
  91. ncbi Reference materials for the evaluation of pre-mortem variant Creutzfeldt-Jakob disease diagnostic assays
    J K Cooper
    CJD Resource Centre, National Institute for Biological Standards and Control, Blanche Lane, South Mimms, Potters Bar EN6 3QG, UK
    Vox Sang 92:302-10. 2007
    ..A standard panel of materials is needed for the evaluation of assays being developed for the diagnosis of variant Creutzfeldt-Jakob disease...
  92. ncbi Chicken antibody against a restrictive epitope of prion protein distinguishes normal and abnormal prion proteins
    Kazuyoshi Miyamoto
    Laboratory of Immunobiology, Department of Molecular and Applied Bioscience, Graduate School of Biosphere Science, Hiroshima University, 1 4 4 Kagamiyama, Higashi Hiroshima, Hiroshima, Japan
    Biologicals 35:303-8. 2007
    ..immunoprecipitation, sphAb3-15 efficiently bound to PrP from normal brain homogenates, but weakly bound PrP from scrapie-infected brain homogenates...
  93. ncbi Astrocytes accumulate 4-hydroxynonenal adducts in murine scrapie and human Creutzfeldt-Jakob disease
    Olivier Andreoletti
    UMR INRA-ENVT, Physiopathologie Infectieuse et Parasitaire des Ruminants, , 31076, Toulouse, France
    Neurobiol Dis 11:386-93. 2002
    b>Scrapie-infected mice are considered a model for study in prion diseases, which are characterized by the progressive accumulation in the brain of an abnormal isoform (PrPsc) of the normal cellular prion protein (PrPc)...
  94. ncbi [Comments on present-day spread and epidemiology of BSE and prion diseases]
    W Bodemer
    Deutsches Primatenzentrum Göttingen, Abt Infektionspathologie
    Gesundheitswesen 66:S21-5. 2004
    ..The diseases have been well known for decades. Scrapie was first described around 1750, a BSE case was reported in the 1850-ties most likely a misdiagnosis, and in 1920/..
  95. ncbi Sheep scrapie susceptibility-linked polymorphisms do not modulate the initial binding of cellular to disease-associated prion protein prior to conversion
    Alan Rigter
    Central Institute for Animal Disease Control, Department of Bacteriology and TSEs, PO Box 2004, 8203 AA Lelystad, The Netherlands
    J Gen Virol 86:2627-34. 2005
    Conversion of the host-encoded protease-sensitive cellular prion protein (PrPC) into the scrapie-associated protease-resistant isoform (PrPSc) of prion protein (PrP) is the central event in transmissible spongiform encephalopathies or ..
  96. ncbi Prion infection of epithelial Rov cells is a polarized event
    Sophie Paquet
    , Institut National de la Recherche Agronomique, 78350 Jouy-en-Josas, France
    J Virol 78:7148-52. 2004
    ..These data raise the possibility that apically expressed PrP may be involved in this polarized process of infection. This would add further support for a crucial role of PrP at the cell surface in prion infection of target cells...
  97. ncbi Tissue plasminogen activator in brain tissues infected with transmissible spongiform encephalopathies
    K Xanthopoulos
    Prion Disease Research Group, Laboratory of Pharmacology, Department of Pharmaceutical Sciences, School of Health Sciences, Aristotle University of Thessaloniki, 54124 Thessaloniki, Greece
    Neurobiol Dis 20:519-27. 2005
    ..In addition, it was observed that TSE symptoms and subsequent death of plasminogen-deficient and tPA-deficient scrapie challenged mice preceded that of wild-type controls...
  98. ncbi Atypical prion protein in sheep brain collected during the British scrapie-surveillance programme
    S J Everest
    Department of TSE Molecular Biology, Veterinary Laboratories Agency, New Haw, Addlestone, Surrey KT15 3NB, UK
    J Gen Virol 87:471-7. 2006
    b>Scrapie of sheep and goats is the most common prion disease (or transmissible spongiform encephalopathy, TSE) of mammals and aggregates of abnormal, proteinase-resistant prion protein (PrP(Sc)) are found in all naturally occurring prion ..
  99. ncbi Anchors away--of plaques and pathology in prion disease
    Glenn Telling
    Department of Microbiology, Immunology and Molecular Genetics, University of Kentucky, Lexington, USA
    N Engl J Med 353:1177-9. 2005
  100. ncbi Soluble dimeric prion protein binds PrP(Sc) in vivo and antagonizes prion disease
    Philipp Meier
    Institute of Neuropathology, Schmelzbergstrasse, University Hospital of Zurich, Zurich, Switzerland
    Cell 113:49-60. 2003
    ..Accordingly, mice expressing PrP-Fc(2) but lacking endogenous PrP(C) are resistant to scrapie, do not accumulate PrP-Fc(2)(Sc), and do not transmit disease to others...
  101. ncbi Primary isolation of the bovine spongiform encephalopathy agent in mice: agent definition based on a review of 150 transmissions
    R Green
    Department of Pathology, Veterinary Laboratories Agency (VLA)-Weybridge, New Haw, Addlestone, Surrey KT15 3NB, UK
    J Comp Pathol 132:117-31. 2005
    ..These features, together with the conventional lesion profile, will be of use in distinguishing the agents of BSE and scrapie in sheep.

Research Grants76

  1. Impact of Microparticles on Oral TSE Infections
    Judd Aiken; Fiscal Year: 2009
    ..Oral transmission is the primary route of infection for BSE, scrapie and TME and is the suspected route of transmission for vCJD and CWD...
  2. Impact of Microparticles on Oral TSE Infections
    Joel A Pedersen; Fiscal Year: 2010
    ..Oral transmission is the primary route of infection for BSE, scrapie and TME and is the suspected route of transmission for vCJD and CWD...
  3. Characteristics of Prion Entry and Neuroinvasion Following Nasal Cavity Infection
    Anthony E Kincaid; Fiscal Year: 2010
    ..to the horizontal spread of prion disease between animals, such as chronic wasting disease in deer and elk, and scrapie in sheep and goats...
  4. Characterization of de novo generation of infectious prions
    Joaquin Castilla; Fiscal Year: 2009
    ..e. Bovine spongiform encephalopathy (BSE) in US cattle and Nor98 (atypical scrapie) in New Zealand sheep, provide evidence that this infrequent spontaneous phenomenon may occur naturally...
  5. Characterization of prion protein conformational changes
    Valerie Daggett; Fiscal Year: 2007
    ..TSEs, or prion diseases, include Creutzfeldt-Jakob disease, fatal familial insomnia and Kuru in humans, scrapie in sheep, bovine spongiform encephalopathy in cattle, and chronic wasting disease in deer and elk...
  6. Characterization of prion protein conformational changes
    VALERIE D DAGGETT; Fiscal Year: 2010
    ..TSEs, or prion diseases, include Creutzfeldt-Jakob disease, fatal familial insomnia and Kuru in humans, scrapie in sheep, bovine spongiform encephalopathy in cattle, and chronic wasting disease in deer and elk...
  7. Role of Prion Protein in Manganese Neurotoxicity
    ANUMANTHA GOUNDER KANTHASAMY; Fiscal Year: 2010
    ..protein, iii) to determine whether chronic manganese exposure accelerates the accumulation and aggregation of the scrapie form of prion protein (PrPSc) and causes increased neuronal damage in a mouse model of prion disease, iv) to ..
  8. Key molecular mechanisms of extraneural pathogenesis and transmission of TSEs
    Mark Zabel; Fiscal Year: 2009
    ..These include chronic wasting disease (CWD) of deer and elk, scrapie in sheep, bovine spongiform encephalopathy (BSE) in cattle and Creutzfeldt-Jacob disease and kuru in humans...
  9. Key molecular mechanisms of extraneural pathogenesis and transmission of TSEs
    Mark D Zabel; Fiscal Year: 2010
    ..These include chronic wasting disease (CWD) of deer and elk, scrapie in sheep, bovine spongiform encephalopathy (BSE) in cattle and Creutzfeldt-Jacob disease and kuru in humans...
  10. Species Susceptibility Assay for Chronic Wasting Disease
    Surachai Supattapone; Fiscal Year: 2005
    ..amplification to measure the potential susceptibility of a range of potential host species to CWD as well as scrapie. Species to be tested include humans, cows, pigs, sheep, goats, chickens, dogs, cats, coyotes, foxes, raccoons, ..
  11. PrP-scrapie transport across intestinal & BBB
    Neena Singh; Fiscal Year: 2007
    ..Despite these concerns, surprisingly little is known about the mechanism(s) by which the infectious prion or PrP-scrapie (PrPsc), a protein of 27-30kDa, is transported from the intestine or peripheral blood to the central nervous ..
  12. Emergence of drug resistance in prion populations
    Charles Weissmann; Fiscal Year: 2010
    ..innovation making this investigation possible is the Cell Panel Assay (CPA), which is based on the Standard Scrapie Cell Assay (SSCA), both of which were developed in our laboratories and which allow the rapid discrimination ..
  13. Intercellular transfer of prion in prion disease
    Man Sun Sy; Fiscal Year: 2006
    ..the same pathogenic mechanism based on the conversion of the normal cellular prion (PrP c) into the infectious scrapie prion (prpSc)...
  14. SYNTHESES AND CELLULAR STUDIES OF NEW PHOTOSENSITIZERS FOR MEDICAL APPLICATIONS
    Kevin Smith; Fiscal Year: 2009
    ..blood, bone marrow purging, and for inhibition of transmissible spongiform encephalopathies [bovine (BSE), sheep (scrapie) and human forms (Creutzfeld-Jacob disease)]...
  15. SYNTHESES AND CELLULAR STUDIES OF NEW PHOTOSENSITIZERS FOR MEDICAL APPLICATIONS
    Kevin M Smith; Fiscal Year: 2011
    ..blood, bone marrow purging, and for inhibition of transmissible spongiform encephalopathies [bovine (BSE), sheep (scrapie) and human forms (Creutzfeld-Jacob disease)]...
  16. SYNTHESES AND CELLULAR STUDIES OF NEW PHOTOSENSITIZERS FOR MEDICAL APPLICATIONS
    Kevin M Smith; Fiscal Year: 2010
    ..blood, bone marrow purging, and for inhibition of transmissible spongiform encephalopathies [bovine (BSE), sheep (scrapie) and human forms (Creutzfeld-Jacob disease)]...
  17. A Proteomic Approach to Biomarker Discovery in Prion Disease
    Lingjun Li; Fiscal Year: 2007
    ..impact upon human and animal health are Creutzfeldt-Jakob disease (CJD), bovine spongiform encephalopathy (BSE), scrapie and chronic wasting disease (CWD)...
  18. Therapeutic Approaches for Prion Disease
    Thomas Wisniewski; Fiscal Year: 2009
    ..Mabs will be tested for therapeutic efficacy in tissue culture models of scrapie and human sporadic CJD prion infection and 3) Passive immunization studies using existing Mabs and antibodies ..
  19. Therapeutic Approaches for Prion Disease
    Thomas M Wisniewski; Fiscal Year: 2010
    ..Mabs will be tested for therapeutic efficacy in tissue culture models of scrapie and human sporadic CJD prion infection and 3) Passive immunization studies using existing Mabs and antibodies ..
  20. Structural Investigations of the Prion Protein het-s
    Roland Riek; Fiscal Year: 2006
    The "protein-only hypothesis" states that prion diseases such as scrapie in sheep, bovine spongiform encephalopathy (BSE) and Creutzfeldt-Jakob disease in human are distinct from infectious diseases caused by bacteria, viruses, or ..
  21. Mechanism of Prion Neurotropism
    Surachai Supattapone; Fiscal Year: 2010
    ..in cattle, chronic wasting disease (CWD) in deer and elk, transmissible mink encephalopathy (TME) in mink, and scrapie in sheep. The infectious agents of TSEs are unconventional, proteinaceous entities, which have been termed prions...
  22. Germinal Center Function in Prion Disease
    Alan Young; Fiscal Year: 2007
    ..clearly demonstrated a disruption in the differentiation and phenotype of these two B cell subsets in response to scrapie infection...
  23. Transmissibility of Neurodegenerative Diseases
    DMITRY Y GOLDGABER; Fiscal Year: 2010
    ..to animals were obtained with a notable exception of prion diseases such as Creitzfeldt-Jakob disease in man and scrapie and mad cow disease in animals...
  24. Transmissibility of Neurodegenerative Diseases
    Dmitry Goldgaber; Fiscal Year: 2009
    ..to animals were obtained with a notable exception of prion diseases such as Creitzfeldt-Jakob disease in man and scrapie and mad cow disease in animals...
  25. An improved expression vector to create transgenic mice for prion research.
    GULTEKIN TAMGUNEY; Fiscal Year: 2007
    ..agents responsible for fatal neurodegenerative diseases such as Creutzfeldt-Jakob disease (CJD) in humans, scrapie in sheep, and bovine spongiform encephalopathy (BSE) in cattle...
  26. Preventing Human Prion Diseases - Inactivation of Prions
    STANLEY PRUSINER; Fiscal Year: 2009
    ..we plan to study three strains of prions passaged in mice: RML and 301V, which originated from sheep with scrapie and cattle with BSE, respectively, plus a novel synthetic strain MoSP1, which is the most stable strain reported ..
  27. Preventing Human Prion Diseases - Inactivation of Prions
    STANLEY PRUSINER; Fiscal Year: 2007
    ..we plan to study three strains of prions passaged in mice: RML and 301V, which originated from sheep with scrapie and cattle with BSE, respectively, plus a novel synthetic strain MoSP1, which is the most stable strain reported ..
  28. CWD: ROLE OF THE LYMPHOID TISSUE PHASE IN PRION DISEASE
    Christina Sigurdson; Fiscal Year: 2004
    ..topical, and important feature of several TSEs, including variant Creutzfeldt-Jakob disease (vCJD) in humans, scrapie in sheep, and chronic wasting disease (CWD) in deer...