prion diseases

Summary

Summary: A group of genetic, infectious, or sporadic degenerative human and animal nervous system disorders associated with abnormal PRIONS. These diseases are characterized by conversion of the normal prion protein to an abnormal configuration via a post-translational process. In humans, these conditions generally feature DEMENTIA; ATAXIA; and a fatal outcome. Pathologic features include a spongiform encephalopathy without evidence of inflammation. The older literature occasionally refers to these as unconventional SLOW VIRUS DISEASES. (From Proc Natl Acad Sci USA 1998 Nov 10;95(23):13363-83)

Top Publications

  1. pmc Cells release prions in association with exosomes
    Benoit Fevrier
    Centre National de la Recherche Scientifique, Unite Mixte de Recherche 144, Institut Curie, 75005 Paris, France
    Proc Natl Acad Sci U S A 101:9683-8. 2004
  2. 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
  3. ncbi Packaging of prions into exosomes is associated with a novel pathway of PrP processing
    L J Vella
    Department of Biochemistry and Molecular Biology, The University of Melbourne, Parkville, Victoria 3010, Australia
    J Pathol 211:582-90. 2007
  4. pmc Prions
    David W Colby
    Institute for Neurodegenerative Diseases, University of California, San Francisco, San Francisco, California 94143, USA
    Cold Spring Harb Perspect Biol 3:a006833. 2011
  5. pmc Microglia in the degenerating brain are capable of phagocytosis of beads and of apoptotic cells, but do not efficiently remove PrPSc, even upon LPS stimulation
    Martina M Hughes
    Trinity College Institute of Neuroscience and School of Biochemistry and Immunology, Trinity College Dublin, Dublin 2, Republic of Ireland
    Glia 58:2017-30. 2010
  6. pmc Follicular dendritic cell-specific prion protein (PrP) expression alone is sufficient to sustain prion infection in the spleen
    Laura McCulloch
    The Roslin Institute and Royal Dick School of Veterinary Sciences, University of Edinburgh, Midlothian, United Kingdom
    PLoS Pathog 7:e1002402. 2011
  7. pmc Rapid cell-surface prion protein conversion revealed using a novel cell system
    R Goold
    Department of Neurodegenerative Disease, Institute of Neurology, University College London, Queen Square, London WC1N 3BG, UK
    Nat Commun 2:281. 2011
  8. doi Physiology of the prion protein
    Rafael Linden
    Instituto de Biofísica da Universidade Federal do Rio de Janeiro, Rio de Janeiro, RJ, Brazil
    Physiol Rev 88:673-728. 2008
  9. doi Prions: protein aggregation and infectious diseases
    Adriano Aguzzi
    Institute of Neuropathology, University Hospital of Zurich, Zurich, Switzerland
    Physiol Rev 89:1105-52. 2009
  10. 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

Detail Information

Publications342 found, 100 shown here

  1. pmc Cells release prions in association with exosomes
    Benoit Fevrier
    Centre National de la Recherche Scientifique, Unite Mixte de Recherche 144, Institut Curie, 75005 Paris, France
    Proc Natl Acad Sci U S A 101:9683-8. 2004
    b>Prion diseases are infectious neurodegenerative disorders linked to the accumulation in the central nervous system of the abnormally folded prion protein (PrP) scrapie (PrPsc), which is thought to be the infectious agent...
  2. 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
    ....
  3. ncbi Packaging of prions into exosomes is associated with a novel pathway of PrP processing
    L J Vella
    Department of Biochemistry and Molecular Biology, The University of Melbourne, Parkville, Victoria 3010, Australia
    J Pathol 211:582-90. 2007
    b>Prion diseases are fatal, transmissible neurodegenerative disorders associated with conversion of the host-encoded prion protein (PrP(C)) into an abnormal pathogenic isoform (PrP(Sc))...
  4. pmc Prions
    David W Colby
    Institute for Neurodegenerative Diseases, University of California, San Francisco, San Francisco, California 94143, USA
    Cold Spring Harb Perspect Biol 3:a006833. 2011
    ..The prion diseases are characterized by neurodegeneration and lethality...
  5. pmc Microglia in the degenerating brain are capable of phagocytosis of beads and of apoptotic cells, but do not efficiently remove PrPSc, even upon LPS stimulation
    Martina M Hughes
    Trinity College Institute of Neuroscience and School of Biochemistry and Immunology, Trinity College Dublin, Dublin 2, Republic of Ireland
    Glia 58:2017-30. 2010
    ..Furthermore, microglia/macrophages remain IL-1β-negative during phagocytosis of apoptotic cells. The data demonstrate that phagocytic activity and pro-inflammatory microglial phenotype do not necessarily correlate...
  6. pmc Follicular dendritic cell-specific prion protein (PrP) expression alone is sufficient to sustain prion infection in the spleen
    Laura McCulloch
    The Roslin Institute and Royal Dick School of Veterinary Sciences, University of Edinburgh, Midlothian, United Kingdom
    PLoS Pathog 7:e1002402. 2011
    b>Prion diseases are characterised by the accumulation of PrP(Sc), an abnormally folded isoform of the cellular prion protein (PrP(C)), in affected tissues...
  7. pmc Rapid cell-surface prion protein conversion revealed using a novel cell system
    R Goold
    Department of Neurodegenerative Disease, Institute of Neurology, University College London, Queen Square, London WC1N 3BG, UK
    Nat Commun 2:281. 2011
    b>Prion diseases are fatal neurodegenerative disorders with unique transmissible properties. The infectious and pathological agent is thought to be a misfolded conformer of the prion protein...
  8. doi Physiology of the prion protein
    Rafael Linden
    Instituto de Biofísica da Universidade Federal do Rio de Janeiro, Rio de Janeiro, RJ, Brazil
    Physiol Rev 88:673-728. 2008
    b>Prion diseases are transmissible spongiform encephalopathies (TSEs), attributed to conformational conversion of the cellular prion protein (PrP(C)) into an abnormal conformer that accumulates in the brain...
  9. doi Prions: protein aggregation and infectious diseases
    Adriano Aguzzi
    Institute of Neuropathology, University Hospital of Zurich, Zurich, Switzerland
    Physiol Rev 89:1105-52. 2009
    ..PrPC, and therefore, understanding the physiological role of PrPC may help to clarify the mechanism underlying prion diseases. Here we discuss the evolution of the prion concept and how prion-like mechanisms may apply to other protein ..
  10. 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
    Much evidence supports the hypothesis that the infectious agents of prion diseases are devoid of nucleic acid, and instead are composed of a specific infectious protein...
  11. pmc A molecular switch controls interspecies prion disease transmission in mice
    Christina J Sigurdson
    Department of Pathology and Department of Medicine, University of California, San Diego, La Jolla, California 92093, USA
    J Clin Invest 120:2590-9. 2010
    ..These findings point to a triggering role of the local beta2-alpha2 loop structure for prion transmissibility between different species...
  12. pmc MicroRNA 146a (miR-146a) is over-expressed during prion disease and modulates the innate immune response and the microglial activation state
    Reuben Saba
    Molecular Pathobiology, National Microbiology Laboratory, Public Health Agency of Canada, Winnipeg, Manitoba, Canada
    PLoS ONE 7:e30832. 2012
    ..Based on our results, we propose a role for miR-146a as a potent modulator of microglial function by regulating the activation state during prion induced neurodegeneration...
  13. pmc Detection and characterization of proteinase K-sensitive disease-related prion protein with thermolysin
    Sabrina Cronier
    MRC Prion Unit and Department of Neurodegenerative Disease, University College London Institute of Neurology, National Hospital for Neurology and Neurosurgery, Queen Square, London WC1N3BG, UK
    Biochem J 416:297-305. 2008
    ..Detection of PK-sensitive isoforms of disease-related PrP using thermolysin should be useful for improving diagnostic sensitivity in human prion diseases.
  14. pmc Generating a prion with bacterially expressed recombinant prion protein
    Fei Wang
    Department of Molecular and Cellular Biochemistry, Ohio State University, Columbus, OH 43210, USA
    Science 327:1132-5. 2010
    ..Thus, as postulated by the prion hypothesis, the infectivity in mammalian prion disease results from an altered conformation of PrP...
  15. doi Mouse neuroblastoma cells release prion infectivity associated with exosomal vesicles
    Sandrine Alais
    LaboRétro INSERM U758, Unité de Virologie Humaine, Ecole Normale Superieure de Lyon, 69364 Lyon Cedex 07, France
    Biol Cell 100:603-15. 2008
    ..With this aim, we initiated characterization of the prion agent and the pathways by which it can be propagated using the cellular model system neuroblastoma (N2a)...
  16. doi Prion propagation and toxicity in vivo occur in two distinct mechanistic phases
    Malin K Sandberg
    MRC Prion Unit and Department of Neurodegenerative Disease, UCL Institute of Neurology, Queen Square, London WC1N 3BG, UK
    Nature 470:540-2. 2011
    ..Production of neurotoxic species is triggered when prion propagation saturates, leading to a switch from autocatalytic production of infectivity (phase 1) to a toxic (phase 2) pathway...
  17. pmc Recombinant prion protein induces a new transmissible prion disease in wild-type animals
    Natallia Makarava
    Medical Biotechnology Center, University of Maryland Biotechnology Institute, Baltimore, MD 21201, USA
    Acta Neuropathol 119:177-87. 2010
    ..The length of the clinical duration is more typical of human and large animal prion diseases, than those of rodents...
  18. pmc Cellular prion protein regulates beta-secretase cleavage of the Alzheimer's amyloid precursor protein
    Edward T Parkin
    Proteolysis Research Group, Institute of Molecular and Cellular Biology, Faculty of Biological Sciences, and Leeds Institute of Genetics, Health and Therapeutics, University of Leeds, Leeds LS2 9JT, United Kingdom
    Proc Natl Acad Sci U S A 104:11062-7. 2007
    ..Two mutants of PrP, PG14 and A116V, that are associated with familial human prion diseases failed to inhibit the beta-secretase cleavage of APP...
  19. pmc Molecular cross talk between misfolded proteins in animal models of Alzheimer's and prion diseases
    Rodrigo Morales
    Protein Misfolding Disorders Laboratory, Department of Neurology, Mitchell Center for Alzheimer s Disease and Related Brain Disorders, University of Texas Medical School at Houston, Houston, Texas 77030, USA
    J Neurosci 30:4528-35. 2010
    ..of this study was to analyze the interaction of the protein misfolding processes implicated in Alzheimer's and prion diseases. For this purpose, we inoculated prions in an Alzheimer's transgenic mouse model that develop typical amyloid ..
  20. pmc Getting a grip on prions: oligomers, amyloids, and pathological membrane interactions
    Byron Caughey
    Laboratory of Persistent Viral Diseases, Rocky Mountain Laboratories, National Institute for Allergy and Infectious Diseases, National Institutes of Health, Hamilton, MT 59840, USA
    Annu Rev Biochem 78:177-204. 2009
    ....
  21. doi The transcellular spread of cytosolic amyloids, prions, and prionoids
    Adriano Aguzzi
    Institute of Neuropathology, University Hospital of Zurich, Schmelzbergstrasse 12, CH 8091 Zurich, Switzerland
    Neuron 64:783-90. 2009
    ....
  22. doi Increased proportions of C1 truncated prion protein protect against cellular M1000 prion infection
    Victoria Lewis
    Department of Pathology, University of Melbourne, Parkville, Victoria, Australia
    J Neuropathol Exp Neurol 68:1125-35. 2009
    ....
  23. pmc Redox control of prion and disease pathogenesis
    Neena Singh
    Department of Pathology, Case Western Reserve University, Cleveland, Ohio 44106, USA
    Antioxid Redox Signal 12:1271-94. 2010
    ..Finally, we discuss possible therapeutic avenues directed at restoring brain metal homeostasis and alleviating metal-induced oxidative stress in prion disorders...
  24. pmc Sustained translational repression by eIF2α-P mediates prion neurodegeneration
    Julie A Moreno
    MRC Toxicology Unit, Hodgkin Building, University of Leicester, Lancaster Road, Leicester LE1 9HN, UK
    Nature 485:507-11. 2012
    ..Many of these disorders, including Alzheimer's, Parkinson's and prion diseases, are associated with the accumulation of misfolded disease-specific proteins...
  25. pmc Prion uptake in the gut: identification of the first uptake and replication sites
    Pekka Kujala
    Section of Cell Biology II, Netherlands Cancer Institute, Amsterdam, The Netherlands
    PLoS Pathog 7:e1002449. 2011
    ..Together, these data identify a novel potential M cell-independent mechanism for prion transport, mediated by FAE enterocytes, which acts to initiate conversion and replication upon FDCs and subsequent infection of enteric nerves...
  26. pmc Variably protease-sensitive prionopathy: a new sporadic disease of the prion protein
    Wen Quan Zou
    Department of Pathology, National Prion Disease Pathology Surveillance Center, Case Western Reserve University, Cleveland, OH 44106, USA
    Ann Neurol 68:162-72. 2010
    ..The 2 new PSPr forms affect individuals who are either homozygous for methionine (129MM) or heterozygous for methionine/valine (129MV)...
  27. pmc A systems approach to prion disease
    Daehee Hwang
    Institute for Systems Biology, Seattle, WA 98103, USA
    Mol Syst Biol 5:252. 2009
    ..Our systems analysis provides a comprehensive basis for developing models for prion replication and disease, and suggests some possible therapeutic approaches...
  28. ncbi Synaptic changes characterize early behavioural signs in the ME7 model of murine prion disease
    C Cunningham
    CNS Inflammation Group, Southampton Neuroscience Group, School of Biological Sciences, University of Southampton, Southampton SO16 7PX, UK
    Eur J Neurosci 17:2147-55. 2003
    b>Prion diseases are fatal, chronic neurodegenerative diseases of mammals, characterized by amyloid deposition, astrogliosis, microglial activation, tissue vacuolation and neuronal loss...
  29. pmc Mammalian prions generated from bacterially expressed prion protein in the absence of any mammalian cofactors
    Jae Il Kim
    Department of Physiology and Biophysics, Case Western Reserve University, Cleveland, Ohio 44106, USA
    J Biol Chem 285:14083-7. 2010
    ....
  30. pmc MicroRNAs as a molecular basis for mental retardation, Alzheimer's and prion diseases
    Patrick Provost
    CHUL Research Center CHUQ and Faculty of Medicine, Universite Laval, Quebec, QC, Canada
    Brain Res 1338:58-66. 2010
    ....
  31. ncbi Oxidative stress and the prion protein in transmissible spongiform encephalopathies
    Ollivier Milhavet
    Institut de Genetique Humaine, CNRS U P R 1142, 141, rue de la Cardonille, 34396 Cedex 5, Montpellier, France
    Brain Res Brain Res Rev 38:328-39. 2002
    ..Subsequently, we will discuss the relationship between the prion protein and the cellular response to oxidative stress, attempting ultimately to link PrP function and neurodegeneration in these disorders...
  32. pmc M cell-depletion blocks oral prion disease pathogenesis
    D S Donaldson
    The Roslin Institute and Royal Dick School of Veterinary Sciences, University of Edinburgh, Edinburgh, UK
    Mucosal Immunol 5:216-25. 2012
    Many prion diseases are orally acquired...
  33. 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
    ..This experiment has established the value of using sheep as a model for studying transmission of variant Creutzfeld-Jakob disease by blood products in humans...
  34. pmc Prion diseases and their biochemical mechanisms
    Nathan J Cobb
    Department of Physiology, Case Western Reserve University, Cleveland, Ohio 44106, USA
    Biochemistry 48:2574-85. 2009
    b>Prion diseases, also known as the transmissible spongiform encephalopathies (TSEs), are a group of fatal neurodegenerative disorders that affect humans and animals...
  35. pmc Cellular factors implicated in prion replication
    Karim Abid
    Department of Neurology, University of Texas Medical Branch, Galveston, TX, USA
    FEBS Lett 584:2409-14. 2010
    Prions are the unconventional infectious agents responsible for prion diseases, which are composed mainly by the misfolded prion protein (PrP(Sc)) that replicates by converting the host associated cellular prion protein (PrP(C))...
  36. ncbi A general model of prion strains and their pathogenicity
    John Collinge
    MRC Prion Unit, Department of Neurodegenerative Disease, UCL Institute of Neurology, London WC1N 3BG, UK
    Science 318:930-6. 2007
    ..Recent advances suggest that prions themselves are not directly neurotoxic, but rather their propagation involves production of toxic species, which may be uncoupled from infectivity...
  37. pmc Detection of protease-resistant cervid prion protein in water from a CWD-endemic area
    T A Nichols
    Department of Microbiology, Immunology and Pathology, Colorado State University, Fort Collins, USA
    Prion 3:171-83. 2009
    ..These data demonstrate detection of very low levels of PrP(CWD) in the environment by sPMCA and suggest persistence and accumulation of prions in the environment that may promote CWD transmission...
  38. pmc De novo generation of a transmissible spongiform encephalopathy by mouse transgenesis
    Christina J Sigurdson
    Universitatsspital Zurich, Institute of Neuropathology, CH 8091 Zurich, Switzerland
    Proc Natl Acad Sci U S A 106:304-9. 2009
    ..This shows that expression of a subtly altered prion protein, with known 3D structure, efficiently generates a prion disease...
  39. pmc Mutant PrPSc conformers induced by a synthetic peptide and several prion strains
    Patrick Tremblay
    Institute for Neurodegenerative Diseases and Department of Neurology, University of California, San Francisco, California 94143, USA
    J Virol 78:2088-99. 2004
    ....
  40. pmc All clinically-relevant blood components transmit prion disease following a single blood transfusion: a sheep model of vCJD
    Sandra McCutcheon
    The Roslin Institute and Royal Dick School of Veterinary Studies, University of Edinburgh, Roslin, Edinburgh, United Kingdom
    PLoS ONE 6:e23169. 2011
    ..These data suggest that all blood components are vectors for prion disease transmission, and highlight the importance of multiple control measures to minimise the risk of human to human transmission of vCJD by blood transfusion...
  41. ncbi Identification of novel proteinase K-resistant C-terminal fragments of PrP in Creutzfeldt-Jakob disease
    Wen Quan Zou
    Institute of Pathology, Case Western Reserve University, Cleveland, Ohio 44106, USA
    J Biol Chem 278:40429-36. 2003
    The central event in the pathogenesis of prion diseases, a group of fatal, transmissible neurodegenerative disorders including Creutzfeldt-Jakob disease (CJD) in humans, is the conversion of the normal or cellular prion protein (PrPC) ..
  42. ncbi Enzymatic degradation of prion protein in brain stem from infected cattle and sheep
    Jan P M Langeveld
    Division of Infectious Diseases and Food Chain Quality, Institute for Animal Science and Health, Lelystad, The Netherlands
    J Infect Dis 188:1782-9. 2003
    ..This enzymatic process could lead to the development of a method for the decontamination of medical and laboratory equipment. The ultimate effectiveness of this method of prion inactivation has to be tested in mouse bioassays...
  43. pmc A new method for the characterization of strain-specific conformational stability of protease-sensitive and protease-resistant PrP
    Laura Pirisinu
    Department of Veterinary Public Health and Food Safety, Istituto Superiore di Sanita, Rome, Italy
    PLoS ONE 5:e12723. 2010
    ....
  44. ncbi Targeting cellular prion protein reverses early cognitive deficits and neurophysiological dysfunction in prion-infected mice
    Giovanna R Mallucci
    MRC Prion Unit, Department of Neurodegenerative Disease, Institute of Neurology, Queen Square, London WC1N 3BG, United Kingdom
    Neuron 53:325-35. 2007
    ..These data suggest that early intervention in human prion disease may lead to recovery of cognitive and behavioral symptoms...
  45. ncbi Stromal complement receptor CD21/35 facilitates lymphoid prion colonization and pathogenesis
    Mark D Zabel
    Institute for Neuropathology, University Hospital of Zurich, Zurich, Switzerland
    J Immunol 179:6144-52. 2007
    ..Because both PrP(C) and CD21/35 are highly expressed on follicular dendritic cells, CD21/35 appears to be involved in targeting prions to follicular dendritic cells and expediting neuroinvasion following peripheral exposure to prions...
  46. ncbi Endoplasmic reticulum stress features are prominent in Alzheimer disease but not in prion diseases in vivo
    Ursula Unterberger
    Institute of Neurology, Medical University of Vienna, Austria
    J Neuropathol Exp Neurol 65:348-57. 2006
    b>Prion diseases and Alzheimer disease (AD) share a variety of clinical and neuropathologic features (e.g...
  47. pmc De novo generation of infectious prions in vitro produces a new disease phenotype
    Marcelo A Barria
    George and Cynthia Mitchell Center for Neurodegenerative Diseases, University of Texas Medical Branch, Galveston, TX, USA
    PLoS Pathog 5:e1000421. 2009
    ..The findings also suggest that prion diversity is not restricted to those currently known, and that likely new forms of infectious protein foldings may be produced, resulting in novel disease phenotypes...
  48. pmc Protease-sensitive synthetic prions
    David W Colby
    Institute for Neurodegenerative Diseases, University of California, San Francisco, California, United States of America
    PLoS Pathog 6:e1000736. 2010
    ..These novel synthetic prions demonstrate that conformational changes in wild-type PrP can produce mouse prions composed exclusively of sPrP(Sc)...
  49. doi Common structural traits across pathogenic mutants of the human prion protein and their implications for familial prion diseases
    Giulia Rossetti
    Statistical and Biological Physics Sector, Scuola Internazionale Superiore di Studi Avanzati SISSA, via Bonomea 265, I 34136 Trieste, Italy
    J Mol Biol 411:700-12. 2011
    Human (Hu) familial prion diseases are associated with about 40 point mutations of the gene coding for the prion protein (PrP). Most of the variants associated with these mutations are located in the globular domain of the protein...
  50. pmc Discovery of 2-aminothiazoles as potent antiprion compounds
    Sina Ghaemmaghami
    Institute for Neurodegenerative Diseases, San Francisco, CA 94143 0518, USA
    J Virol 84:3408-12. 2010
    b>Prion diseases are fatal, untreatable neurodegenerative diseases caused by the accumulation of the misfolded, infectious isoform of the prion protein (PrP), termed PrP(Sc)...
  51. doi Facilitated cross-species transmission of prions in extraneural tissue
    Vincent Beringue
    Institut National de la Recherche Agronomique UR892, Virologie Immunologie Moléculaires, Jouy en Josas, France
    Science 335:472-5. 2012
    ..Thus, prion cross-species transmission efficacy can exhibit a marked tissue dependence...
  52. pmc Dissociation of infectivity from seeding ability in prions with alternate docking mechanism
    Michael B Miller
    Department of Biochemistry, Dartmouth Medical School, Hanover, New Hampshire, United States of America
    PLoS Pathog 7:e1002128. 2011
    ..These results suggest that the propagation of PrP(Sc) molecules may not depend on a single stereotypic mechanism, but that normal PrP(C)/PrP(Sc) interaction through polybasic domains may be required to generate prion infectivity...
  53. pmc Orally administered prion protein is incorporated by m cells and spreads into lymphoid tissues with macrophages in prion protein knockout mice
    Ikuro Takakura
    Cellular Biology Laboratory, Graduate School of Agricultural Science, Tohoku University, Sendai, Japan
    Am J Pathol 179:1301-9. 2011
    ....
  54. ncbi The role of lipid rafts in prion protein biology
    Victoria Lewis
    Department of Pathology, University of Melbourne, Victoria 3010, Australia
    Front Biosci (Landmark Ed) 16:151-68. 2011
    ..the misfolded isoform PrP(Sc )is the central pathogenic event in the uniquely transmissible neurodegenerative prion diseases. As both PrP(C) and PrP(Sc) are associated with membranes, the nature of the membrane microenvironment may ..
  55. pmc Prion protein facilitates uptake of zinc into neuronal cells
    Nicole T Watt
    Faculty of Biological Sciences, School of Molecular and Cellular Biology, University of Leeds, Leeds LS2 9JT, UK
    Nat Commun 3:1134. 2012
    ..These data suggest that alterations in the cellular prion protein-mediated zinc uptake may contribute to neurodegeneration in prion and other neurodegenerative diseases...
  56. pmc Biochemical properties of highly neuroinvasive prion strains
    Cyrus Bett
    Department of Pathology, University of California, San Diego, La Jolla, California, USA
    PLoS Pathog 8:e1002522. 2012
    ....
  57. pmc Darwinian evolution of prions in cell culture
    Jiali Li
    Department of Infectology, Scripps Florida, 130 Scripps Way, Jupiter, FL 33458, USA
    Science 327:869-72. 2010
    ..Prions, albeit devoid of a nucleic acid genome, are thus subject to mutation and selective amplification...
  58. pmc Genome-wide association study in multiple human prion diseases suggests genetic risk factors additional to PRNP
    Simon Mead
    MRC Prion Unit and Department of Neurodegenerative Disease, UCL Institute of Neurology, Queen Square, London WC1N 3BG, UK
    Hum Mol Genet 21:1897-906. 2012
    b>Prion diseases are fatal neurodegenerative diseases of humans and animals caused by the misfolding and aggregation of prion protein (PrP)...
  59. ncbi Microglia and the pathogenesis of spongiform encephalopathies
    P Rezaie
    Department of Neuropathology, Institute of Psychiatry, King's College London, DeCrespigny Park, SE5 8AF, London, UK
    Brain Res Brain Res Rev 35:55-72. 2001
    ..The prion diseases, also known as spongiform encephalopathies, are fatal neurodegenerative disorders with sporadic, genetic or ..
  60. ncbi Synthetic mammalian prions
    Giuseppe Legname
    Institute for Neurodegenerative Diseases, University of California, San Francisco, CA 94143, USA
    Science 305:673-6. 2004
    ..Neuropathological findings suggest that a novel prion strain was created. Our results provide compelling evidence that prions are infectious proteins...
  61. doi A highly sensitive immunoassay for the detection of prion-infected material in whole human blood without the use of proteinase K
    M Howard Tattum
    MRC Prion Unit and the Department of Neurodegenerative Disease, Institute of Neurology, University College London, London, UK
    Transfusion 50:2619-27. 2010
    ....
  62. pmc Genesis of mammalian prions: from non-infectious amyloid fibrils to a transmissible prion disease
    Natallia Makarava
    Center for Biomedical Engineering and Technology, University of Maryland, Baltimore, Maryland, United States of America
    PLoS Pathog 7:e1002419. 2011
    ..The current work illustrates that transmissible prion diseases can be induced by PrP structures different from that of authentic PrP(Sc) and suggests that a new mechanism ..
  63. pmc Prion protein misfolding and disease
    Roger A Moore
    Rocky Mountain Laboratories, Laboratory of Persistent Viral Diseases, NIAID, NIH, 903 S 4th Street, Hamilton, MT 59840, United States
    Curr Opin Struct Biol 19:14-22. 2009
    Transmissible spongiform encephalopathies (TSEs or prion diseases) are a rare group of invariably fatal neurodegenerative disorders that affect humans and other mammals...
  64. pmc Prion disease susceptibility is affected by beta-structure folding propensity and local side-chain interactions in PrP
    M Qasim Khan
    Campbell Family Institute for Cancer Research, Department of Biochemistry, University of Toronto, Toronto Medical Discovery Tower 4 307, 101 College Street, Toronto, ON, Canada M5G 1L7
    Proc Natl Acad Sci U S A 107:19808-13. 2010
    b>Prion diseases occur when the normally α-helical prion protein (PrP) converts to a pathological β-structured state with prion infectivity (PrP(Sc)). Exposure to PrP(Sc) from other mammals can catalyze this conversion...
  65. ncbi Prion protein is necessary for normal synaptic function
    J Collinge
    Department of Biochemistry and Molecular Genetics, St Mary s Hospital Medical School, Imperial College, London, UK
    Nature 370:295-7. 1994
    The prion diseases are neurodegenerative conditions, transmissible by inoculation, and in some cases inherited as an autosomal dominant disorder...
  66. 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)...
  67. doi Variant Creutzfeldt-Jakob disease
    J W Ironside
    National Creutzfeldt Jakob Disease Surveillance Unit, Division of Pathology, School of Molecular and Clinical Medicine, University of Edinburgh, Western General Hospital, Edinburgh, UK
    Haemophilia 16:175-80. 2010
    ..Continued surveillance is required to investigate the prevalence of secondary variant CJD infection in other patients with bleeding disorders who have been treated with UK-sourced pooled plasma products...
  68. 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
    ..Studies of prion diseases may advance investigations of other neurodegenerative disorders and of how neurons differentiate, function for ..
  69. pmc Safety and efficacy of quinacrine in human prion disease (PRION-1 study): a patient-preference trial
    John Collinge
    National Prion Clinic, National Hospital for Neurology and Neurosurgery, University College London Hospital National Health Service Foundation Trust, Queen Square, London WC1N 3BG, UK
    Lancet Neurol 8:334-44. 2009
    The propagation of prions, the causative agents of Creutzfeldt-Jakob disease and other human prion diseases, requires post-translational conversion of normal cellular prion protein to disease-associated forms...
  70. doi Bovine macrophage degradation of scrapie and BSE PrPSc
    Yukiko Sassa
    Laboratory of Food and Environmental Hygiene, Department of Veterinary Medicine, Faculty of Applied Biological Sciences, Gifu University, 1 1 Yanagido, Gifu City, Gifu, 501 1193, Japan
    Vet Immunol Immunopathol 133:33-9. 2010
    ..These results suggest that bovine macrophages have the ability to take up and degrade PrP(Sc), resulting in decreased TSE infectivity in mice...
  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
    b>Prion diseases are fatal neurodegenerative diseases characterized by the accumulation of PrP(Sc), the infectious and protease-resistant form of the cellular prion protein (PrP(C))...
  72. pmc Strain-specific viral properties of variant Creutzfeldt-Jakob disease (vCJD) are encoded by the agent and not by host prion protein
    Laura Manuelidis
    Yale Medical School, 333 Cedar Street, New Haven, Connecticut 06510, USA
    J Cell Biochem 106:220-31. 2009
    ..Monotypic cultures can be used to identify the intrinsic, strain-determining molecules of TSE infectious particles...
  73. doi Prospective 10-year surveillance of human prion diseases in Japan
    Ichiro Nozaki
    Department of Neurology and Neurobiology of Ageing, Kanazawa University Graduate School of Medical Science, 13 1 Takara machi, Kanazawa, Japan
    Brain 133:3043-57. 2010
    We analysed the epidemiological data and clinical features of patients with prion diseases that had been registered by the Creutzfeldt-Jakob Disease Surveillance Committee, Japan, over the past 10 years, since 1999...
  74. pmc Genes contributing to prion pathogenesis
    GULTEKIN TAMGUNEY
    Institute for Neurodegenerative Diseases, University of California, San Francisco, CA, USA
    J Gen Virol 89:1777-88. 2008
    b>Prion diseases are caused by conversion of a normally folded, non-pathogenic isoform of the prion protein (PrP(C)) to a misfolded, pathogenic isoform (PrP(Sc))...
  75. ncbi Prion protein: orchestrating neurotrophic activities
    Vilma R Martins
    Ludwig Institute for Cancer Research, Hospital Alemão, Oswaldo Cruz, Sao Paulo, SP, Brazil
    Curr Issues Mol Biol 12:63-86. 2010
    ....
  76. ncbi Autophagy, prion infection and their mutual interactions
    Andreas Heiseke
    Institute of Virology, Prion Research Group, Technische Universitat Munchen, Trogerstr 30, 81675 Munich, Germany
    Curr Issues Mol Biol 12:87-97. 2010
    b>Prion diseases are infectious and fatal neurodegenerative disorders of man and animals which are characterized by spongiform degeneration in the central nervous system...
  77. ncbi Eight prion strains have PrP(Sc) molecules with different conformations
    J Safar
    Department of Neurology, University of California, San Francisco 94143 0518, USA
    Nat Med 4:1157-65. 1998
    ....
  78. pmc Endogenous proteolytic cleavage of disease-associated prion protein to produce C2 fragments is strongly cell- and tissue-dependent
    Michel Dron
    INRA, U892 Virologie Immunologie Moléculaires, F 78350 Jouy en Josas, France
    J Biol Chem 285:10252-64. 2010
    ..These findings have implications on the molecular analysis of PrP(Sc) and cell pathogenesis of prion infection...
  79. pmc Lymph nodal prion replication and neuroinvasion in mice devoid of follicular dendritic cells
    Marco Prinz
    Institute of Neuropathology, University Hospital of Zurich, Schmelzbergstrasse 12, CH 8091 Zurich, Switzerland
    Proc Natl Acad Sci U S A 99:919-24. 2002
    ..Macrophage subsets are plausible candidates for lymphoreticular prion pathogenesis and neuroinvasion in the absence of FDCs, and may represent a novel target for postexposure prophylaxis...
  80. pmc Single treatment with RNAi against prion protein rescues early neuronal dysfunction and prolongs survival in mice with prion disease
    Melanie D White
    Department of Neurodegenerative Disease, Medical Research Council, Prion Unit Institute of Neurology, University College London, London, United Kingdom
    Proc Natl Acad Sci U S A 105:10238-43. 2008
    b>Prion diseases are fatal neurodegenerative conditions for which there is no effective treatment...
  81. ncbi Complement facilitates early prion pathogenesis
    M A Klein
    Institute of Neuropathology, University of Zurich, Schmelzbergstrasse, Zurich, Switzerland
    Nat Med 7:488-92. 2001
    ..Splenic accumulation of prion infectivity and PrPSc was delayed, indicating that activation of specific complement components is involved in the initial trapping of prions in lymphoreticular organs early after infection...
  82. pmc Infectious prions in pre-clinical deer and transmission of chronic wasting disease solely by environmental exposure
    Candace K Mathiason
    Department of Microbiology, Immunology and Pathology, Colorado State University, Fort Collins, Colorado, United States of America
    PLoS ONE 4:e5916. 2009
    Key to understanding the epidemiology and pathogenesis of prion diseases, including chronic wasting disease (CWD) of cervids, is determining the mode of transmission from one individual to another...
  83. pmc Trans-dominant inhibition of prion propagation in vitro is not mediated by an accessory cofactor
    James C Geoghegan
    Department of Biochemistry, Dartmouth Medical School, Hanover, NH, USA
    PLoS Pathog 5:e1000535. 2009
    ....
  84. pmc Crossing the species barrier by PrP(Sc) replication in vitro generates unique infectious prions
    Joaquin Castilla
    Department of Neurology, University of Texas Medical Branch, Galveston, TX 77555, USA
    Cell 134:757-68. 2008
    ..Our results indicate that PMCA is a valuable tool for the investigation of cross-species transmission and suggest that species barrier and strain generation are determined by the propagation of PrP misfolding...
  85. pmc The most infectious prion protein particles
    Jay R Silveira
    Laboratory of Persistent Viral Diseases, Rocky Mountain Laboratories, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Hamilton, Montana 59840, USA
    Nature 437:257-61. 2005
    ..These results suggest that non-fibrillar particles, with masses equivalent to 14-28 PrP molecules, are the most efficient initiators of TSE disease...
  86. ncbi Altered circadian activity rhythms and sleep in mice devoid of prion protein
    I Tobler
    Institute of Pharmacology, University of Zurich, Switzerland
    Nature 380:639-42. 1996
    There is a wealth of data supporting a central role for the prion protein (PrP) in the neurodegenerative prion diseases of both humans and other species, yet the normal function of PrP, which is expressed at the cell surface of neurons ..
  87. ncbi Protein misfolding cyclic amplification for diagnosis and prion propagation studies
    Joaquin Castilla
    Univeristy of Texas, Medical Branch, Galveston, 77555, USA
    Methods Enzymol 412:3-21. 2006
    ..Among them, prion diseases are some of the most intriguing disorders that can be transmitted by an unprecedented infectious agent, termed ..
  88. doi The prion diseases
    Khalilah Brown
    Center for Comprehensive Care and Research on Memory Disorders, Department of Neurology, University of Chicago, Chicago, IL 60637, USA
    J Geriatr Psychiatry Neurol 23:277-98. 2010
    The prion diseases are a family of rare neurodegenerative disorders that result from the accumulation of a misfolded isoform of the prion protein (PrP), a normal constituent of the neuronal membrane...
  89. pmc Transmissible encephalopathy agents: virulence, geography and clockwork
    Laura Manuelidis
    Yale University, Section of Neuropathology, Surgery, New Haven, CT, USA
    Virulence 1:101-4. 2010
    ..The virus-like biology of unique TSE agents, including epidemic spread, mutation, and superinfection, can be used to systematically define features of virulence that distinguish common endemic from newly emerging strains...
  90. pmc Possible case of maternal transmission of feline spongiform encephalopathy in a captive cheetah
    Anna Bencsik
    Unité ATNC, Agence Française de Sécurité Sanitaire des Aliments AFSSA, Lyon, France
    PLoS ONE 4:e6929. 2009
    ..This new observation may have some impact on our knowledge of vertical transmission of BSE agent-linked TSEs such as in housecat FSE, or vCJD...
  91. pmc Agent-specific Shadoo responses in transmissible encephalopathies
    Kohtaro Miyazawa
    Yale University Medical School, 333 Cedar Street, New Haven, CT 06510, USA
    J Neuroimmune Pharmacol 5:155-63. 2010
    ..Furthermore, because all infected GT1 cells appeared as healthy as uninfected controls, Sho was not needed to protect infected cells from their "toxic" burden of abundant abnormal PrP and intracellular amyloid...
  92. ncbi ERK1/2 and p38 MAP kinases control prion protein fragment 90-231-induced astrocyte proliferation and microglia activation
    Stefano Thellung
    Department of Oncology, Biology and Genetics, University of Genova, Genova, Italy
    Glia 55:1469-85. 2007
    Astrogliosis and microglial activation are a common feature during prion diseases, causing the release of chemoattractant and proinflammatory factors as well as reactive free radicals, involved in neuronal degeneration...
  93. doi Prion diseases
    Sriram Venneti
    Department of Pathology and Laboratory Medicine, Hospital of the University of Pennsylvania, Philadelphia, 19104, USA
    Clin Lab Med 30:293-309. 2010
    b>Prion diseases are disorders affecting the central nervous system caused by alterations in the conformation of the cellular prion protein...
  94. pmc Down-regulation of Shadoo in prion infections traces a pre-clinical event inversely related to PrP(Sc) accumulation
    David Westaway
    Centre for Prions and Protein Folding Diseases, University of Alberta, Edmonton, Alberta, Canada
    PLoS Pathog 7:e1002391. 2011
    ..This cellular response may offer new insights into the homeostatic mechanisms involved in detection and clearance of the misfolded proteins that drive prion disease pathogenesis...
  95. pmc Glypican-1 mediates both prion protein lipid raft association and disease isoform formation
    David R Taylor
    Proteolysis Research Group, Institute of Molecular and Cellular Biology, Faculty of Biological Sciences, and Leeds Institute of Genetics, Health and Therapeutics, University of Leeds, Leeds, UK
    PLoS Pathog 5:e1000666. 2009
    In prion diseases, the cellular form of the prion protein, PrP(C), undergoes a conformational conversion to the infectious isoform, PrP(Sc)...
  96. pmc Comparative prion disease gene expression profiling using the prion disease mimetic, cuprizone
    Laura R Moody
    Program in Cellular and Molecular Biology and Comparative Biosciences, School of Veterinary Medicine, University of Wisconsin, Madison, WI, USA
    Prion 3:99-109. 2009
    ..Here we describe the novel use of the prion disease mimetic, cuprizone, to control for cell population changes in the brain during prion infection...
  97. doi Kuru: its ramifications after fifty years
    P P Liberski
    Laboratory of Electron Microscopy and Neuropathology, Department of Molecular Pathology and Neuropathology, Medical University Lodz, 92 216 Lodz, Poland
    Exp Gerontol 44:63-9. 2009
    Kuru was the first human neurodegenerative disease in the group of transmissible spongiform encephalopathies, prion diseases or, in the past, slow unconventional virus diseases...
  98. pmc Shadoo (Sprn) and prion disease incubation time in mice
    Sarah E Lloyd
    MRC Prion Unit and Department of Neurodegenerative Diseases, UCL Institute of Neurology, London, UK
    Mamm Genome 20:367-74. 2009
    b>Prion diseases are transmissible neurodegenerative disorders of mammalian species and include scrapie, bovine spongiform encephalopathy (BSE), and variant Creutzfeldt-Jakob disease (vCJD)...
  99. ncbi Structural differences between TSEs strains investigated by FT-IR spectroscopy
    Sashko Spassov
    P25, Robert Koch Institut, Nordufer 20, D 13353 Berlin, Germany
    Biochim Biophys Acta 1760:1138-49. 2006
    ....
  100. ncbi Surface decontamination of surgical instruments: an ongoing dilemma
    H Murdoch
    Centre for Emergency Preparedness and Response Laboratory A, Health Protection Agency, Porton Down, Salisbury, UK
    J Hosp Infect 63:432-8. 2006
    ....
  101. ncbi Cyclic amplification of protein misfolding and aggregation
    Paula Saa
    Department of Neurology, University of Texas Medical Branch, Galveston, TX, USA
    Methods Mol Biol 299:53-65. 2005
    ....

Research Grants97

  1. 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 ..
  2. Impact of Microparticles on Oral TSE Infections
    Joel A Pedersen; Fiscal Year: 2010
    b>Prion diseases are inevitably fatal, transmissible disorders inducing a progressive neurologic dysfunction after a prolonged incubation period...
  3. Impact of Microparticles on Oral TSE Infections
    Judd Aiken; Fiscal Year: 2009
    b>Prion diseases are inevitably fatal, transmissible disorders inducing a progressive neurologic dysfunction after a prolonged incubation period...
  4. Characteristics of Prion Entry and Neuroinvasion Following Nasal Cavity Infection
    Anthony E Kincaid; Fiscal Year: 2010
    ..This route of inoculation is likely to be relevant to the natural spread of prion diseases because a study published this year demonstrated that sheep can be infected with scrapie via intranasal ..
  5. Characterization of de novo generation of infectious prions
    Joaquin Castilla; Fiscal Year: 2009
    ..The occurrence of spontaneous cases of prion diseases in humans (sporadic CJD) and in other species, i.e...
  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 ..
  7. Role of Prion Protein in Manganese Neurotoxicity
    ANUMANTHA GOUNDER KANTHASAMY; Fiscal Year: 2010
    ..Therefore, dysregulation of metal homeostasis has been suggested to play a role in the pathogenesis of prion diseases. Recent observations of elevated manganese (Mn) levels in the brain and blood of humans and animals afflicted ..
  8. Key molecular mechanisms of extraneural pathogenesis and transmission of TSEs
    Mark Zabel; Fiscal Year: 2009
    b>Prion diseases are invariably fatal infectious diseases affecting a wide range of animals...
  9. Key molecular mechanisms of extraneural pathogenesis and transmission of TSEs
    Mark D Zabel; Fiscal Year: 2010
    b>Prion diseases are invariably fatal infectious diseases affecting a wide range of animals...
  10. Emergence of drug resistance in prion populations
    Charles Weissmann; Fiscal Year: 2010
    ..This project is to investigate how drug-resistant prions arise, a question that has important consequences for the design of treatment strategies. ..
  11. Intercellular transfer of prion in prion disease
    Man Sun Sy; Fiscal Year: 2006
    Transmissible spongiform encephalopathies (TSE) or prion diseases are a group of fatal neuro-degenerative disorders that affect both humans and animals...
  12. SYNTHESES AND CELLULAR STUDIES OF NEW PHOTOSENSITIZERS FOR MEDICAL APPLICATIONS
    Kevin Smith; Fiscal Year: 2009
    ..The full potential can be realized only when new drugs with higher cellular selectivity and specificity are discovered, their mechanisms of biological action investigated, and their efficacy evaluated relative to existing modalities. ..
  13. SYNTHESES AND CELLULAR STUDIES OF NEW PHOTOSENSITIZERS FOR MEDICAL APPLICATIONS
    Kevin M Smith; Fiscal Year: 2010
    ..The full potential can be realized only when new drugs with higher cellular selectivity and specificity are discovered, their mechanisms of biological action investigated, and their efficacy evaluated relative to existing modalities. ..
  14. SYNTHESES AND CELLULAR STUDIES OF NEW PHOTOSENSITIZERS FOR MEDICAL APPLICATIONS
    Kevin M Smith; Fiscal Year: 2011
    ..The full potential can be realized only when new drugs with higher cellular selectivity and specificity are discovered, their mechanisms of biological action investigated, and their efficacy evaluated relative to existing modalities. ..
  15. Cyclic Amplification of Prion Protein Misfolding
    Claudio Soto; Fiscal Year: 2010
    ..b>Prion diseases are a group of infectious neurodegenerative disorders associated with the cerebral accumulation of misfolded ..
  16. A Proteomic Approach to Biomarker Discovery in Prion Disease
    Lingjun Li; Fiscal Year: 2007
    ..biomarkers will provide a basis for the development of an ante-mortem antibody-based screening test for prion diseases in suspected animals...
  17. Therapeutic Approaches for Prion Disease
    Thomas Wisniewski; Fiscal Year: 2009
    b>Prion diseases are infectious, conformational neurodegenerative disorders characterized by the structural modification of the prion protein, PrPC, into a pathological conformer, PrPSc...
  18. Therapeutic Approaches for Prion Disease
    Thomas M Wisniewski; Fiscal Year: 2010
    b>Prion diseases are infectious, conformational neurodegenerative disorders characterized by the structural modification of the prion protein, PrPC, into a pathological conformer, PrPSc...
  19. Biochemical Detection of Prions in Blood
    Joaquin Castilla; Fiscal Year: 2005
    ..Transmissible spongiform encephalopathies, also called prion diseases, are a group of fatal infectious neurodegenerative disorders affecting humans and animals...
  20. Investigation of the Establishment of the Prion State
    Irina Derkatch; Fiscal Year: 2007
    ..is expected to facilitate the development of new strategies for preventing the onset and transmission of prion diseases. The long-term goal of PI's research is to understand the biogenesis and biological role of prions, whereas ..
  21. 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 ..
  22. Germinal Center Function in Prion Disease
    Alan Young; Fiscal Year: 2007
    ..Blood is known to be infectious in prion diseases. To address this concern, individuals at risk for harboring human prion disease are now excluded from donating ..
  23. Therapeutic Approaches for Prion Disease
    Thomas Wisniewski; Fiscal Year: 2009
    b>Prion diseases are infectious, conformational neurodegenerative disorders characterized by the structural modification of the prion protein, PrPC, into a pathological conformer, PrPSc...
  24. Cyclic Amplification of Prion Protein Misfolding
    Claudio Soto; Fiscal Year: 2009
    b>Prion diseases are a group of infectious neurodegenerative disorders affecting humans and animals...
  25. Preventing Human Prion Diseases - Inactivation of Prions
    Stanley B Prusiner; Fiscal Year: 2010
    ..Effective protocols for inactivating prions will protect the general public as well as laboratory scientists who are investigating prions. Rarely does a research study have such immediate and important implications. ..
  26. Preventing Human Prion Diseases - Inactivation of Prions
    STANLEY PRUSINER; Fiscal Year: 2009
    ..Effective protocols for inactivating prions will protect the general public as well as laboratory scientists who are investigating prions. Rarely does a research study have such immediate and important implications. ..
  27. Preventing Human Prion Diseases - Inactivation of Prions
    STANLEY PRUSINER; Fiscal Year: 2007
    ..Effective protocols for inactivating prions will protect the general public as well as laboratory scientists who are investigating prions. Rarely does a research study have such immediate and important implications. ..
  28. Structural Characterization of Prion Isoforms in Multiple TSE Diseases
    MICHELE MCGUIRL; Fiscal Year: 2009
    ..in its aggregated isoforms, and to elucidate the relationship between isoform structure and different types of prion diseases. Prion protein is the causative agent of many fatal neurodegenerative diseases of mammals...
  29. Structural Characterization of Prion Isoforms in Multiple TSE Diseases
    MICHELE ANN MCGUIRL; Fiscal Year: 2010
    ..in its aggregated isoforms, and to elucidate the relationship between isoform structure and different types of prion diseases. Prion protein is the causative agent of many fatal neurodegenerative diseases of mammals...
  30. Structure/Mechanism of a Prion-remodeling Factor
    Francis T F Tsai; Fiscal Year: 2010
    ..The broad and long-term objective of our research is to further our molecular understanding of prions and prion diseases. [PSI+] is a yeast prion that increases translational read-through of nonsense codons...
  31. Interaction of Prions with Dendritic Cells
    Michael Green; Fiscal Year: 2004
    ..outbreak of chronic wasting disease among deer and elk in the United States have brought the issue of prion diseases into the spotlight...
  32. Transmission of Prions Within and Between Species
    Robert Harrington; Fiscal Year: 2006
    ..Participation in educational and training opportunities from these multiple sources will provide Dr. Harrington with a well rounded scientific background and allow specific research training pertinent to his area of research interest. ..
  33. CWD: ROLE OF THE LYMPHOID TISSUE PHASE IN PRION DISEASE
    Christina Sigurdson; Fiscal Year: 2004
    ..The results of these studies will contribute to understanding PrP trafficking in the lymphoid system and could provide a basis for development of blood-based diagnostic assays and intervention strategies. ..