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 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
  2. ncbi Novel proteinaceous infectious particles cause scrapie
    S B Prusiner
    Science 216:136-44. 1982
  3. 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
  4. 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
  5. 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
  6. pmc 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
  7. pmc 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
  8. pmc 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 Reciprocal interference between specific CJD and scrapie agents in neural cell cultures
    Noriuki Nishida
    Yale Medical School, New Haven, CT 06510, USA
    Science 310:493-6. 2005
  10. pmc 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

Detail Information

Publications336 found, 100 shown here

  1. 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...
  2. 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...
  3. 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
    ....
  4. 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...
  5. 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...
  6. pmc 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)...
  7. pmc 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...
  8. pmc 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 Reciprocal interference between specific CJD and scrapie agents in neural cell cultures
    Noriuki Nishida
    Yale Medical School, New Haven, CT 06510, USA
    Science 310:493-6. 2005
    ..In addition, SY-CJD prevented superinfection by sheep-derived Chandler (Ch) and 22L scrapie agents...
  10. pmc 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...
  11. doi 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 ..
  12. 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...
  13. 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...
  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 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...
  16. pmc 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 ..
  17. ncbi Conformational transitions, dissociation, and unfolding of scrapie amyloid (prion) protein
    J Safar
    Laboratory of Central Nervous System Studies, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland 20892
    J Biol Chem 268:20276-84. 1993
    The infectious form of the scrapie amyloid (prion) precursor, PrPSc, is a host-derived protein and a component of the infectious agent causing scrapie...
  18. 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.
  19. 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...
  20. 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 ..
  21. 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...
  22. pmc 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...
  23. pmc Microglial cell line established from prion protein-overexpressing mice is susceptible to various murine prion strains
    Yoshifumi Iwamaru
    Research Center for Prion Diseases, National Institute of Animal Health, Kannondai 3 1 5, Tsukuba, Ibaraki 305 8602, Japan
    J Virol 81:1524-7. 2007
    ..After exposure to Chandler scrapie, we observed the replication and accumulation of disease-associated forms of the prion protein in MG20 cells up ..
  24. 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...
  25. pmc Prion disease detection, PMCA kinetics, and IgG in urine from sheep naturally/experimentally infected with scrapie and deer with preclinical/clinical chronic wasting disease
    Richard Rubenstein
    SUNY Downstate Medical Center, Department of Neurology, Box 1213, 450 Clarkson Avenue, Brooklyn, NY 11203, USA
    J Virol 85:9031-8. 2011
    ..prion disease-associated seeding activity (PASA) in urine from naturally and orally infected sheep with clinical scrapie agent and orally infected preclinical and infected white-tailed deer with clinical chronic wasting disease (CWD)...
  26. doi Emergence of multiple prion strains from single isolates of ovine scrapie
    Alana M Thackray
    University of Cambridge, Department of Veterinary Medicine, Cambridge, UK
    J Gen Virol 92:1482-91. 2011
    The infectious agent associated with prion diseases such as ovine scrapie shows strain diversity. Ovine prion strains have typically been identified by their transmission properties in wild-type mice...
  27. doi Propagation of ovine prions from "poor" transmitter scrapie isolates in ovine PrP transgenic mice
    Alana M Thackray
    University of Cambridge, Department of Veterinary Medicine, Madingley Road, Cambridge, CB3 OES, UK
    Exp Mol Pathol 92:167-74. 2012
    ..The existence of scrapie isolates that do not propagate in wild type mice, defined here as "poor" transmitters, are problematic for ..
  28. doi Marked influence of the route of infection on prion strain apparent phenotype in a scrapie transgenic mouse model
    Christelle Langevin
    INRA Institut National de la recherche Agronomique, UR892, Virologie Immunologie Moléculaires, F 78350 Jouy en Josas, France
    Neurobiol Dis 41:219-25. 2011
    ..this possibility, we studied prion pathogenesis in a prototypal short-incubation disease model consisting of 127S scrapie strain propagated in tg338 transgenic mice expressing the VRQ allele of ovine PrP...
  29. doi 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...
  30. doi 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
    ....
  31. pmc 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...
  32. pmc 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...
  33. pmc 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...
  34. doi 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...
  35. pmc 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...
  36. ncbi Chronic lymphocytic inflammation specifies the organ tropism of prions
    Mathias Heikenwalder
    Institute of Neuropathology, University Hospital of Zurich, CH 8091 Zurich, Switzerland
    Science 307:1107-10. 2005
    ..By expanding the tissue distribution of prions, chronic inflammatory conditions may act as modifiers of natural and iatrogenic prion transmission...
  37. pmc 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).
  38. pmc Lesion profiling at primary isolation in RIII mice is insufficient in distinguishing BSE from classical scrapie
    Katy E Beck
    Veterinary Laboratories Agency Weybridge, New Haw, Addlestone, Surrey KT15 3NB, UK
    Brain Pathol 20:313-22. 2010
    ..in RIII mice generates a lesion profile believed to be reproducible and distinct from that produced by classical scrapie. This profile, which is characterized by peaks at gray matter areas 1, 4 and 7 (dorsal medulla, hypothalamus and ..
  39. doi Ovine PrP genotype is linked with lesion profile and immunohistochemistry patterns after primary transmission of classical scrapie to wild-type mice
    Katy E Beck
    Neuropathology Unit, Veterinary Laboratories Agency Weybridge, New Haw, Addlestone, Surrey, United Kingdom
    J Neuropathol Exp Neurol 69:483-97. 2010
    It is currently believed that primary transmission of classical scrapie to wild-type mice is inefficient and characterized by low attack rates and variable incubation periods and lesion profiles...
  40. pmc Fatal transmissible amyloid encephalopathy: a new type of prion disease associated with lack of prion protein membrane anchoring
    Bruce Chesebro
    Laboratory of Persistent Viral Diseases, Rocky Mountain Laboratories, National Institute of Allergy and Infectious Diseases, Hamilton, Montana, United States of America
    PLoS Pathog 6:e1000800. 2010
    ..only anchorless PrP, we found that PrP anchoring to the cell membrane was required for typical clinical scrapie. However, in the present experiments, using homozygous transgenic mice expressing two-fold more anchorless PrP, ..
  41. pmc 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...
  42. pmc 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 ..
  43. pmc 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...
  44. 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...
  45. pmc Prionemia and leukocyte-platelet-associated infectivity in sheep transmissible spongiform encephalopathy models
    Caroline Lacroux
    UMR INRA ENVT 1225, Interactions Hôtes Agents Pathogènes, Ecole Nationale Veterinaire de Toulouse, Toulouse, France
    J Virol 86:2056-66. 2012
    ..Using two distinct sheep scrapie models and blood transfusion, this work demonstrates the occurrence of a very early and persistent prionemia...
  46. pmc Assessment of the genetic susceptibility of sheep to scrapie by protein misfolding cyclic amplification and comparison with experimental scrapie transmission studies
    Cecilia Bucalossi
    Department of Veterinary Public Health and Food Safety, Istituto Superiore di Sanita, Viale Regina Elena 299, 00161 Rome, Italy
    J Virol 85:8386-92. 2011
    The susceptibility of sheep to scrapie is influenced mainly by the prion protein polymorphisms A136V, R154H, and Q171R/H...
  47. pmc Use of murine bioassay to resolve ovine transmissible spongiform encephalopathy cases showing a bovine spongiform encephalopathy molecular profile
    Katy E Beck
    Animal Health and Veterinary Laboratories Agency, Addlestone, Surrey, UK
    Brain Pathol 22:265-79. 2012
    ..on the same farm in ARQ/ARQ PrP sheep showing attributes of both bovine spongiform encephalopathy (BSE) and scrapie. These cases, UK-1 and UK-2, were investigated further by transmissions to wild-type and ovine transgenic mice...
  48. doi Nuclease resistant circular DNAs copurify with infectivity in scrapie and CJD
    Laura Manuelidis
    Yale University Medical School, 333 Cedar Street, New Haven, CT 06510, USA
    J Neurovirol 17:131-45. 2011
    ..The current results also raise the intriguing possibility that mammals may incorporate more of the prokaryotic world in their cytoplasm than previously recognized...
  49. ncbi Ultrastructures and strain comparison of under-glycosylated scrapie prion fibrils
    Valerie L Sim
    Laboratory of Persistent Viral Diseases, Rocky Mountain Laboratories, National Institute of Allergy and Infectious Diseases, National Institutes of Health, 903 South 4th Street, Hamilton, MT 59840, United States
    Neurobiol Aging 30:2031-42. 2009
    ..visualization of their fibrillar cores, so we purified severely under-glycosylated prion protein fibrils from scrapie-infected transgenic mice expressing anchorless prion protein...
  50. ncbi Specific inhibition of pathological prion protein accumulation by small interfering RNAs
    Nathalie Daude
    Institut de Pharmacologie Moleculaire et Cellulaire, Unité Mixte de Recherche 6097, Centre National de la Recherche Scientifique 660, route des Lucioles, 06560 Valbonne, France
    J Cell Sci 116:2775-9. 2003
    ..the present report, we show that small interfering RNA (siRNA) duplexes trigger specific Prnp gene silencing in scrapie-infected neuroblastoma cells...
  51. pmc Quantitative detection and biological propagation of scrapie seeding activity in vitro facilitate use of prions as model pathogens for disinfection
    Sandra Pritzkow
    Transmissible Spongiform Encephalopathies, Project Group Immune Defense and Pathogenesis, Robert Koch Institut, Berlin, Germany
    PLoS ONE 6:e20384. 2011
    ..Here we report on the development of a methodological platform that further facilitates the use of scrapie prions as model pathogens for disinfection...
  52. pmc PK-sensitive PrP is infectious and shares basic structural features with PK-resistant PrP
    Gustavo Sajnani
    Department of Medicine, School of Medicine, University of Santiago de Compostela, Santiago de Compostela, Galiza, Spain
    PLoS Pathog 8:e1002547. 2012
    ..Although the sPrP(Sc) and rPrP(Sc) fractions have different resistance to PK-digestion, and have previously been shown to sediment differently, and have a different distribution of multimers, they share a common structure and phenotype...
  53. doi Infectious titres of sheep scrapie and bovine spongiform encephalopathy agents cannot be accurately predicted from quantitative laboratory test results
    Lorenzo Gonzalez
    Animal Health and Veterinary Laboratories Agency AHVLA, AHVLA Lasswade, Pentlands Science Park, Penicuick, Midlothian EH26 0PZ, UK
    J Gen Virol 93:2518-27. 2012
    ..To address this discrepancy, 11 brain samples of sheep affected with natural scrapie or experimental bovine spongiform encephalopathy were selected on the basis of the magnitude and predominant ..
  54. pmc Propagation of RML prions in mice expressing PrP devoid of GPI anchor leads to formation of a novel, stable prion strain
    Sukhvir Paul Mahal
    Department of Infectology, Scripps Florida, Jupiter, Florida, United States of America
    PLoS Pathog 8:e1002746. 2012
    ..Because both RML PrP(Sc) and SFL PrP(Sc) are stably propagated in wild-type mice we propose that the two conformations are separated by a high activation energy barrier which is abrogated in tgGPI⁻ mice...
  55. 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)...
  56. pmc 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...
  57. doi 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 ..
  58. pmc 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)...
  59. pmc 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...
  60. 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...
  61. doi 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 ..
  62. pmc 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...
  63. 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...
  64. 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)...
  65. 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...
  66. doi 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...
  67. 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...
  68. 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
    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)...
  69. 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...
  70. pmc 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 ..
  71. pmc Lentivector-mediated RNAi efficiently suppresses prion protein and prolongs survival of scrapie-infected mice
    Alexander Pfeifer
    Institute of Pharmacology and Toxicology, University of Bonn, Bonn, Germany
    J Clin Invest 116:3204-10. 2006
    ..Treatment of scrapie-infected neuronal cells with these lentivectors resulted in an efficient and stable suppression of PrP(Sc) ..
  72. pmc 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...
  73. 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...
  74. pmc 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
    ..Neuro2A cells chronically infected with scrapie prions showed decreased ER-calcium content that correlated with a stronger upregulation of UPR-inducible ..
  75. ncbi Comparative study of the effects of several chemical and physical treatments on the activity of protease resistance and infectivity of scrapie strain 263K
    H L Yao
    National Institute for Viral Disease Control and Prevention, Chinese Center for Disease Control and Prevention, Ying Xin Rd 100, Beijing 100052
    J Vet Med B Infect Dis Vet Public Health 52:437-43. 2005
    ..of chemical and physical factors on the protease resistant activity in vitro and the infectivity in vivo of scrapie strain 263K, PrPSc from the hamsters infected intracerebrally with scrapie strain 263K were treated with several ..
  76. doi 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 ..
  77. pmc Molecular and transmission characteristics of primary-passaged ovine scrapie isolates in conventional and ovine PrP transgenic mice
    Alana M Thackray
    Department of Veterinary Medicine, University of Cambridge, Madingley Road, Cambridge CB3 OES, United Kingdom
    J Virol 82:11197-207. 2008
    ..Here, we have compared the molecular and transmission characteristics of ovine ARQ/ARQ and VRQ/VRQ scrapie isolates following primary passage in tg338 (VRQ) and tg59 (ARQ) ovine PrP transgenic mice and the conventional ..
  78. pmc Cells expressing anchorless prion protein are resistant to scrapie infection
    Kristin L McNally
    Laboratory of Persistent Viral Diseases, Rocky Mountain Laboratories, Hamilton, Montana 59840, USA
    J Virol 83:4469-75. 2009
    ..cells expressing GPI-anchored PrP-sen, anchorless PrP-sen, or both forms of PrP-sen were exposed to the mouse scrapie strain 22L. Cells expressing anchored PrP-sen produced PrP-res after exposure to 22L...
  79. pmc 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)...
  80. 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...
  81. 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...
  82. 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...
  83. 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...
  84. pmc 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...
  85. pmc 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 ..
  86. 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...
  87. pmc Species specificity in the cell-free conversion of prion protein to protease-resistant forms: a model for the scrapie species barrier
    D A Kocisko
    Laboratory of Persistent Viral Diseases, Rocky Mountain Laboratories, National Institute of Allergies and Infectious Diseases, Hamilton, MT 59840, USA
    Proc Natl Acad Sci U S A 92:3923-7. 1995
    b>Scrapie is a transmissible neurodegenerative disease that appears to result from an accumulation in the brain of an abnormal protease-resistant isoform of prion protein (PrP) called PrPsc...
  88. pmc Inhibition of protease-resistant prion protein accumulation in vitro by curcumin
    Byron Caughey
    Laboratory of Persistent Viral Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Rocky Mountain Laboratories, Hamilton, Montana 59840, USA
    J Virol 77:5499-502. 2003
    ..curcumin (diferoylmethane), a major component of the spice turmeric, potently inhibits PrP-res accumulation in scrapie agent-infected neuroblastoma cells (50% inhibitory concentration, approximately 10 nM) and partially inhibits the ..
  89. pmc Normal host prion protein (PrPC) is required for scrapie spread within the central nervous system
    S Brandner
    Institute of Neuropathology, University Hospital, Zurich, Switzerland
    Proc Natl Acad Sci U S A 93:13148-51. 1996
    Mice devoid of PrPC (Prnp%) are resistant to scrapie and do not allow propagation of the infectious agent (prion)...
  90. pmc Temporary blockade of the tumor necrosis factor receptor signaling pathway impedes the spread of scrapie to the brain
    Neil A Mabbott
    Neuropathogenesis Unit, Institute for Animal Health, Edinburgh EH9 3JF, Scotland, United Kingdom
    J Virol 76:5131-9. 2002
    ..Studies of a mouse scrapie model have shown that mature follicular dendritic cells (FDCs), which express the host prion protein (PrP(c)), ..
  91. ncbi Role of gut macrophages in mice orally contaminated with scrapie or BSE
    Thomas Maignien
    CEA, Service de Neurovirologie, CRSSA, B P 6, Fontenay aux Roses Cedex, France
    Int J Pharm 298:293-304. 2005
    ..Mice were subsequently infected with scrapie or BSE by the oral route...
  92. pmc 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...
  93. 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...
  94. 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...
  95. 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 (..
  96. doi 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...
  97. 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 ..
  98. pmc Selective amplification of classical and atypical prions using modified protein misfolding cyclic amplification
    Natallia Makarava
    Center for Biomedical Engineering and Technology and Department of Anatomy and Neurobiology, University of Maryland School of Medicine, Baltimore, Maryland 21201, USA
    J Biol Chem 288:33-41. 2013
    ....
  99. doi Factors influencing temporal variation of scrapie incidence within a closed Suffolk sheep flock
    Lorenzo Gonzalez
    Animal Health and Veterinary Laboratories Agency AHVLA, Pentlands Science Park, Bush Loan, Midlothian EH26 0PZ, UK
    J Gen Virol 93:203-11. 2012
    Several studies have shown that transmission of natural scrapie can occur vertically and horizontally, and that variations in scrapie incidence between and within infected flocks are mostly due to differences in the proportion of sheep ..
  100. ncbi Oxidative stress in the brain at early preclinical stages of mouse scrapie
    Seong Wook Yun
    Clinical Neurochemistry and NPF Center of Excellence Research Laboratories, Clinic of Psychiatry and Psychotherapy, University of Wurzburg, 97080 Wurzburg, Germany
    Exp Neurol 201:90-8. 2006
    ..Using a mouse scrapie model, we assessed oxidative stress in the brain at various stages of the disease progression and observed ..
  101. pmc Rapid disease development in scrapie-infected mice deficient for CD40 ligand
    Michael Burwinkel
    Project Neurodegenerative Diseases, Robert Koch Institute, Nordufer 20, 13353 Berlin, Germany
    EMBO Rep 5:527-31. 2004
    ..We studied the scrapie infection of CD40L-deficient (CD40L(-/-)) mice to see whether ablation of the CD40L gene would be beneficial or ..

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 Kincaid; Fiscal Year: 2009
    ..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. 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...
  5. Red Blood Cell Mimics
    Joseph M DeSimone; Fiscal Year: 2010
    ..infectious prion proteins - the agents associated with variant Creutzfeldt- Jakob disease, mad cow disease and scrapie. A biomimetic approach will be taken to design red blood cells mimics with the same evolutionarily designed ..
  6. MOLECULAR STUDIES OF HUMAN AND ANIMAL PRION PROTEINS
    Shu Chen; Fiscal Year: 2002
    ..disease (CJD), Gerstmann-Straussler-Scheinker syndrome, and fatal familial insomnia in humans, and scrapie, chronic wasting disease (CWD) and bovine spongiform encephalopathy (BSE) in animals...
  7. Biomimetic Approach to the Fabrication of Red Blood Cell Mimics
    Joseph M DeSimone; Fiscal Year: 2010
    ..and infectious prion proteins the agents associated with variant Creutzfeldt-Jakob disease, mad cow disease, and scrapie. Military uses of blood supplies are also clear...
  8. Biomimetic Approach to the Fabrication of Red Blood Cell Mimics
    Joseph DeSimone; Fiscal Year: 2009
    ..and infectious prion proteins the agents associated with variant Creutzfeldt-Jakob disease, mad cow disease, and scrapie. Military uses of blood supplies are also clear...
  9. 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...
  10. Characterization of prion protein conformational changes
    Valerie Daggett; Fiscal Year: 2009
    ..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...
  11. 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...
  12. 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...
  13. 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 ..
  14. 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...
  15. 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...
  16. Characteristics of Prion Entry and Neuroinvasion Following Nasal Cavity Infection
    Anthony E Kincaid; Fiscal Year: 2011
    ..to the horizontal spread of prion disease between animals, such as chronic wasting disease in deer and elk, and scrapie in sheep and goats...
  17. Species Susceptibility Assay for Chronic Wasting Disease
    Surachai Supattapone; Fiscal Year: 2004
    ..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, ..
  18. STUDIES OF PRPSC STRUCTURE BY FIBER DIFFRACTION
    Gerald Stubbs; Fiscal Year: 2009
    ..The animal prion diseases include bovine spongiform encephalopathy ("mad cow disease"), scrapie in sheep and chronic wasting disease in deer...
  19. 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, ..
  20. PrP-scrapie transport across intestinal & BBB
    Neena Singh; Fiscal Year: 2004
    ..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 ..
  21. PrP-scrapie transport across intestinal & BBB
    Neena Singh; Fiscal Year: 2006
    ..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 ..
  22. PrP-scrapie transport across intestinal & BBB
    Neena Singh; Fiscal Year: 2005
    ..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 ..
  23. 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 ..
  24. Intercellular transfer of prion in prion disease
    Man Sun Sy; Fiscal Year: 2004
    ..the same pathogenic mechanism based on the conversion of the normal cellular prion (PrP c) into the infectious scrapie prion (prpSc)...
  25. 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 ..
  26. 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)...
  27. 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)]...
  28. Intercellular transfer of prion in prion disease
    Man Sun Sy; Fiscal Year: 2005
    ..the same pathogenic mechanism based on the conversion of the normal cellular prion (PrP c) into the infectious scrapie prion (prpSc)...
  29. 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)]...
  30. Intercellular transfer of prion in prion disease
    Man Sun Sy; Fiscal Year: 2003
    ..the same pathogenic mechanism based on the conversion of the normal cellular prion (PrP c) into the infectious scrapie prion (prpSc)...
  31. 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)]...
  32. 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)...
  33. A Proteomic Approach to Biomarker Discovery in Prion Disease
    Lingjun Li; Fiscal Year: 2009
    ..impact upon human and animal health are Creutzfeldt-Jakob disease (CJD), bovine spongiform encephalopathy (BSE), scrapie and chronic wasting disease (CWD)...
  34. 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 ..
  35. 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 ..
  36. CNS DEGENERATION CAUSED BY NOVEL PATHOGENS
    STANLEY PRUSINER; Fiscal Year: 1992
    Prions cause transmissible and genetic neurodegenerative diseases including scrapie and bovine spongiform encephalopathy of animals and Creutzfeldt- Jakob disease and Gerstmann-Straussler-Scheinker syndrome (GSS) of humans...
  37. Preventing Human Prion Diseases - Inactivation of Prions
    STANLEY PRUSINER; Fiscal Year: 2006
    ..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 ..
  38. MEMBRANE HYPOTHESIS OF NEURONAL DEGENERATION IN PRION DISEASES
    Stephen De Armond; Fiscal Year: 1999
    ..PrPSc and the accumulation of the latter in the brain are the cause of nerve cell dysfunction and degeneration in scrapie. More recently, we obtained cell physiological evidence that PrPSc accumulates in the plasma membrane of scrapie-..
  39. MEMBRANE HYPOTHESIS OF NEURONAL DEGENERATION IN PRION DISEASES
    Stephen De Armond; Fiscal Year: 2002
    ..PrPSc and the accumulation of the latter in the brain are the cause of nerve cell dysfunction and degeneration in scrapie. More recently, we obtained cell physiological evidence that PrPSc accumulates in the plasma membrane of scrapie-..
  40. TRANSMISSIBLE AND GENETIC NEURODENGENERATIVE DISEASES
    STANLEY PRUSINER; Fiscal Year: 1999
    ..Convergence of many lines of experimental evidence asserts that the scrapie isoform of the prion protein (PrP/Sc) is a necessary component of the infectious scrapie prion yet both PrP/Sc ..
  41. TRANSGENIC MOUSE MODELS FOR ASSESSING TREATMENTS OF PRION DISEASES
    STANLEY PRUSINER; Fiscal Year: 2007
    ..Besides studies in rodents, mouse neuroblastoma (ScN2a) cells chronically infected with scrapie prions have been used to identify several candidate antiprion drugs but none of these drugs has been shown to be ..
  42. Structural Investigations of the Prion Protein het-s
    Roland Riek; Fiscal Year: 2004
    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 ..
  43. Structural Investigations of the Prion Protein het-s
    Roland Riek; Fiscal Year: 2005
    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 ..
  44. Structural Investigations of the Prion Protein het-s
    Roland Riek; Fiscal Year: 2006
    ..unreadable] DESCRIPTION (provided by applicant): 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 ..
  45. Structural Investigations of the Prion Protein het-s
    Roland Riek; Fiscal Year: 2003
    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 ..
  46. Aging Disease--Prion/Transgenic/Immunologic Studies
    Michael Oldstone; Fiscal Year: 2007
    ..encephalopathies (TSE) are degenerative brain diseases that occur in primates and ruminants, and include scrapie in sheep, bovine spongiform encephalopathy (BSE) and several human diseases such as Creutzfeldt-Jakob disease, ..
  47. Mechanism of Prion Neurotropism
    Surachai Supattapone; Fiscal Year: 2009
    ..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...
  48. Aging Disease--Prion/Transgenic/Immunologic Studies
    Michael Oldstone; Fiscal Year: 2006
    ..encephalopathies (TSE) are degenerative brain diseases that occur in primates and ruminants, and include scrapie in sheep, bovine spongiform encephalopathy (BSE) and several human diseases such as Creutzfeldt-Jakob disease, ..
  49. Aging Disease--Prion/Transgenic/Immunologic Studies
    Michael Oldstone; Fiscal Year: 2005
    ..encephalopathies (TSE) are degenerative brain diseases that occur in primates and ruminants, and include scrapie in sheep, bovine spongiform encephalopathy (BSE) and several human diseases such as Creutzfeldt-Jakob disease, ..
  50. Aging Disease--Prion/Transgenic/Immunologic Studies
    Michael Oldstone; Fiscal Year: 2005
    ..encephalopathies (TSE) are degenerative brain diseases that occur in primates and ruminants, and include scrapie in sheep, bovine spongiform encephalopathy (BSE) and several human diseases such as Creutzfeldt-Jakob disease, ..
  51. 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...
  52. TRANSGENIC MOUSE MODELS FOR PRION DISEASE TREATMENT
    STANLEY PRUSINER; Fiscal Year: 2003
    ..Besides studies in rodents, mouse neuroblastoma (ScN2a) cells chronically infected with scrapie prions have been used to identify several candidate antiprion drugs but none of these drugs has been shown to be ..
  53. Aging Disease--Prion/Transgenic/Immunologic Studies
    Michael Oldstone; Fiscal Year: 2004
    ..encephalopathies (TSE) are degenerative brain diseases that occur in primates and ruminants, and include scrapie in sheep, bovine spongiform encephalopathy (BSE) and several human diseases such as Creutzfeldt-Jakob disease, ..
  54. PRP-SCRAPIE TRANSPORT--INTESTINAL & BLOOD BRAIN BARRIER
    Neena Singh; Fiscal Year: 2002
    ..principal infectious agent of BSE, vCJD, and other prion disorders is a conformationally transformed protein, the scrapie prion protein (PrP-Sc), which is responsible for both transmission and pathogenesis of these disorders...
  55. 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...
  56. MOLECULAR PATHOGENESIS OF AGE-DEPENDENT CNS DEGENERATION
    STANLEY PRUSINER; Fiscal Year: 1999
    ..elucidating the role of molecular chaperones in the conversion of the cellular prion protein (PrPC) into the scrapie isoform (PrPSc) are proposed (Project 1)...
  57. TRANSGENIC MOUSE MODELS FOR ALZHEIMER'S DISEASE
    George Carlson; Fiscal Year: 1991
    ..Similar experiments using neurotropic retrovirus or scrapie prions as the neurodegenerative agents are also proposed...
  58. Functional Genetics of Susceptibility to Prions
    George Carlson; Fiscal Year: 2001
    ..Diseases caused by prions include scrapie, bovine spongiform encephalopathy (BSE), and Creutzfeldt-Jakob disease (CJD) in humans...
  59. 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...
  60. Functional Genetics of Susceptibility to Prions
    George Carlson; Fiscal Year: 2002
    ..Diseases caused by prions include scrapie, bovine spongiform encephalopathy (BSE), and Creutzfeldt-Jakob disease (CJD) in humans...
  61. 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...
  62. Functional Genetics of Susceptibility to Prions
    George Carlson; Fiscal Year: 2003
    ..Diseases caused by prions include scrapie, bovine spongiform encephalopathy (BSE), and Creutzfeldt-Jakob disease (CJD) in humans...
  63. Functional Genetics of Susceptibility to Prions
    George Carlson; Fiscal Year: 2005
    ..Diseases caused by prions include scrapie, bovine spongiform encephalopathy (BSE), and Creutzfeldt-Jakob disease (CJD) in humans...
  64. Functional Genetics of Susceptibility to Prions
    George Carlson; Fiscal Year: 2004
    ..Diseases caused by prions include scrapie, bovine spongiform encephalopathy (BSE), and Creutzfeldt-Jakob disease (CJD) in humans...
  65. MEMBRANE HYPOTHESIS OF NEURONAL DEGENERATION IN PRION DISEASES
    Stephen De Armond; Fiscal Year: 2000
    ..PrPSc and the accumulation of the latter in the brain are the cause of nerve cell dysfunction and degeneration in scrapie. More recently, we obtained cell physiological evidence that PrPSc accumulates in the plasma membrane of scrapie-..
  66. 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...
  67. 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 ..
  68. 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 ..
  69. MEMBRANE HYPOTHESIS OF NEURONAL DEGENERATION IN PRION DISEASES
    Stephen De Armond; Fiscal Year: 2001
    ..PrPSc and the accumulation of the latter in the brain are the cause of nerve cell dysfunction and degeneration in scrapie. More recently, we obtained cell physiological evidence that PrPSc accumulates in the plasma membrane of scrapie-..
  70. Phosphorylation of Prion Protein as a novel mechanism for conversion
    Andrea LeBlanc; Fiscal Year: 2009
    ..Our preliminary evidence indicates cdk5-independent phosphorylation of PrP in N2A cells and in scrapie- infected mouse brains...
  71. CWD: ROLE OF THE LYMPHOID TISSUE PHASE IN PRION DISEASE
    Christina Sigurdson; Fiscal Year: 2000
    ..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...
  72. CWD: ROLE OF THE LYMPHOID TISSUE PHASE IN PRION DISEASE
    Christina Sigurdson; Fiscal Year: 2001
    ..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...
  73. 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...