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. ncbi 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
  2. ncbi Cells release prions in association with exosomes
    Benoit Fevrier
    Centre National de la Recherche Scientifique, , Institut Curie, 75005 Paris, France
    Proc Natl Acad Sci U S A 101:9683-8. 2004
  3. ncbi 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
  4. ncbi 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
  5. ncbi 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
  6. 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
  7. ncbi 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
  8. ncbi 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
  9. ncbi 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
  10. ncbi 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

Research Grants

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

Detail Information

Publications305 found, 100 shown here

  1. ncbi 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...
  2. ncbi Cells release prions in association with exosomes
    Benoit Fevrier
    Centre National de la Recherche Scientifique, , 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...
  3. ncbi 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.
  4. ncbi 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...
  5. ncbi 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...
  6. 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...
  7. ncbi 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)...
  8. ncbi 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
    ....
  9. ncbi 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)...
  10. ncbi 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
    ....
  11. ncbi 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
    ....
  12. ncbi 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...
  13. ncbi 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 ..
  14. 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...
  15. ncbi Prion diseases are efficiently transmitted by blood transfusion in sheep
    Fiona Houston
    Neuropathogenesis Division, Roslin Institute, Compton, United Kingdom
    Blood 112:4739-45. 2008
    ..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...
  16. 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...
  17. ncbi 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...
  18. 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
    ....
  19. ncbi 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))...
  20. ncbi 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...
  21. ncbi 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...
  22. 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...
  23. ncbi 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
    ....
  24. ncbi 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)...
  25. ncbi 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...
  26. 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...
  27. ncbi 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...
  28. 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...
  29. 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...
  30. ncbi 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...
  31. ncbi 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...
  32. ncbi 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...
  33. ncbi 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...
  34. 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...
  35. ncbi Isolation of proteinase K-sensitive prions using pronase E and phosphotungstic acid
    Laura D'Castro
    MRC Prion Unit and Department of Neurodegenerative Disease, UCL Institute of Neurology, National Hospital for Neurology and Neurosurgery, London, United Kingdom
    PLoS ONE 5:e15679. 2010
    ..This procedure now allows characterization of proteinase K-sensitive prions and investigation of their clinical relevance in human and animal prion disease without being confounded by contaminating PrP(C)...
  36. 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 ..
  37. ncbi 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...
  38. ncbi Single treatment with RNAi against prion protein rescues early neuronal dysfunction and prolongs survival in mice with prion disease
    Melanie D White
    Department of Neurodegenerative Disease, Medical Research Council, Prion Unit Institute of Neurology, University College London, London, United Kingdom
    Proc Natl Acad Sci U S A 105:10238-43. 2008
    b>Prion diseases are fatal neurodegenerative conditions for which there is no effective treatment...
  39. 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...
  40. ncbi 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...
  41. 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))...
  42. ncbi 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
    ....
  43. 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 ..
  44. 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) ..
  45. ncbi 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 ..
  46. 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
    ....
  47. ncbi 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...
  48. 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 ..
  49. 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...
  50. ncbi 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...
  51. 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
    ....
  52. ncbi 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...
  53. ncbi 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)...
  54. ncbi 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...
  55. ncbi 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...
  56. ncbi 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...
  57. ncbi 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...
  58. ncbi 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...
  59. ncbi 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)...
  60. ncbi 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...
  61. ncbi 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...
  62. ncbi The structural intolerance of the PrP alpha-fold for polar substitution of the helix-3 methionines
    Silvia Lisa
    Insto Química Física Rocasolano, CSIC, 28006 Madrid, Spain
    Cell Mol Life Sci 67:2825-38. 2010
    ..Our work supports that M206 and M213 function as alpha-fold gatekeepers and suggests that their redox state regulate misfolding routes...
  63. ncbi Meat and bone meal and mineral feed additives may increase the risk of oral prion disease transmission
    Christopher J Johnson
    Prion Research Laboratory, USGS National Wildlife Health Center, Madison, Wisconsin, USA
    J Toxicol Environ Health A 74:161-6. 2011
    ..Our data suggest that in feed or other prion-contaminated substances consumed by animals or, potentially, humans, the addition of MBM or the presence of microparticles could heighten risks of prion disease acquisition...
  64. ncbi Pharmacological chaperone for the structured domain of human prion protein
    Andrew J Nicoll
    Department of Neurodegenerative Disease and Medical Research Council Prion Unit, University College of London Institute of Neurology, Queen Square, London WCN1 3BG, United Kingdom
    Proc Natl Acad Sci U S A 107:17610-5. 2010
    In prion diseases, the misfolded protein aggregates are derived from cellular prion protein (PrP(C))...
  65. ncbi Differential expression of erythroid genes in prion disease
    A R Brown
    Division of Gene Function and Development, The Roslin Institute, Roslin, Midlothian EH25 9PS, UK
    Biochem Biophys Res Commun 364:366-71. 2007
    ....
  66. ncbi Role of copper in prion diseases: deleterious or beneficial?
    Lorena Varela-Nallar
    Centro de Regulación Celular y Patología Joaquín V Luco, Facultad de Ciencias Biologicas, P Universidad Catolica de Chile
    Curr Pharm Des 12:2587-95. 2006
    b>Prion diseases are fatal neurodegenerative disorders associated with conformational conversion of the cellular prion protein (PrP(C)) into an isoform designated PrP(Sc)...
  67. 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
    ....
  68. ncbi Surgery and risk of sporadic Creutzfeldt-Jakob disease in Denmark and Sweden: registry-based case-control studies
    Ignacio Mahillo-Fernandez
    Department of Applied Epidemiology, National Center for Epidemiology, and Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas CIBERNED, Carlos III Institute of Health, Madrid, Spain
    Neuroepidemiology 31:229-40. 2008
    ..Epidemiologic evidence of surgical transmission of sporadic Creutzfeldt-Jakob disease (sCJD) remains controversial...
  69. ncbi Comparison of inflammatory and acute-phase responses in the brain and peripheral organs of the ME7 model of prion disease
    Colm Cunningham
    CNS Inflammation Group, School of Biological Sciences, Bassett Crescent East, Southampton, Hampshire SO16 7PX, United Kingdom
    J Virol 79:5174-84. 2005
    ..We also propose that serum cytokine and APP measurements are not useful during preclinical disease. Possible consequences of the clear chronic elevation of APPs in the CNS are discussed...
  70. 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
    ....
  71. ncbi Prions in the environment: occurrence, fate and mitigation
    Samuel E Saunders
    Department of Civil Engineering, University of Nebraska Lincoln, Peter Kiewit Institute, Omaha, Nebraska 68182 0178, USA
    Prion 2:162-9. 2008
    ..Mitigation of contaminated carcasses or soil might be accomplished with enzyme treatments or composting in lieu of incineration...
  72. ncbi Induced neuroprotection independently from PrPSc accumulation in a mouse model for prion disease treated with simvastatin
    Yaron Haviv
    Department of Neurology, The Agnes Ginges Center for Human Neurogenetics, Hadassah University Hospital, Jerusalem, Israel
    Arch Neurol 65:762-75. 2008
    ..In prion diseases, progressive disease and neuronal loss are associated with the accumulation of PrP(Sc), the misfolded isoform ..
  73. ncbi Immunolocalisation of PrPSc in scrapie-infected N2a mouse neuroblastoma cells by light and electron microscopy
    Nathalie M Veith
    Molecular Toxicology, Department of Biology, University of Konstanz, Box X911, D 78457 Konstanz, Germany
    Eur J Cell Biol 88:45-63. 2009
    ..Finally, we observed the release of PrPC/PrPSc via exocytotic pathways, i.e. via exosomes and as an opaque electron-dense mass which may represent a mechanism of intercellular spreading of infectious prions...
  74. ncbi Prion shedding from olfactory neurons into nasal secretions
    Richard A Bessen
    Veterinary Molecular Biology, Montana State University, Bozeman, Montana, United States of America
    PLoS Pathog 6:e1000837. 2010
    ..turnover and replacement of mature ORNs throughout the adult lifespan may also contribute to prion shedding from the nasal passage and could play a role in transmission of natural prion diseases in domestic and free-ranging ruminants.
  75. ncbi Neurodegeneration and oxidative stress: prion disease results from loss of antioxidant defence
    David R Brown
    Department of Biology and Biochemistry, University of Bath, Bath, BA2 7AY, UK
    Folia Neuropathol 43:229-43. 2005
    b>Prion diseases or transmissible spongiform encephalopathies (TSEs) are rare neurodegenerative disorders that can be acquired either by direct transmission, inherited through dominant mutations in the prion protein gene or via an unknown ..
  76. ncbi Silent prions lying in wait: a two-hit model of prion/amyloid formation and infection
    Damien Hall
    Laboratory of Biochemistry and Genetics, National Institute of Diabetes Digestive and Kidney Diseases, National Institutes of Health Bethesda, MD 20892 0830, USA
    J Mol Biol 336:775-86. 2004
    ..We additionally raise the possibility that infectious amyloid strains may be characterized by a size distribution of length or radius...
  77. ncbi Multiple biochemical similarities between infectious and non-infectious aggregates of a prion protein carrying an octapeptide insertion
    Emiliano Biasini
    Department of Cell Biology and Physiology, Washington University School of Medicine, St Louis, Missouri 63110, USA
    J Neurochem 104:1293-308. 2008
    ..Collectively, these results suggest that infectious and non-infectious aggregates of PrP share common structural features accounting for their toxicity, and that self-propagation of PrP involves more subtle molecular differences...
  78. ncbi Absence of spontaneous disease and comparative prion susceptibility of transgenic mice expressing mutant human prion proteins
    Emmanuel A Asante
    MRC Prion Unit and Department of Neurodegenerative Disease, UCL Institute of Neurology, National Hospital for Neurology and Neurosurgery, Queen Square, London WC1N 3BG, UK
    J Gen Virol 90:546-58. 2009
    ..These data indicate that P102L or E200K mutations of human PrP have differing effects on prion propagation that depend upon prion strain type and can be significantly influenced by mismatch at the polymorphic residue 129...
  79. ncbi Plasma noradrenalin as marker of neuroinvasion in prion diseases
    C Pollera
    Department of Veterinary Pathology, Hygiene and Public Health, Faculty of Veterinary Medicine, University of Milan, Milan, Italy
    Vet Res Commun 31:249-52. 2007
  80. ncbi Cell-based immunotherapy of prion diseases by adoptive transfer of antigen-loaded dendritic cells or antigen-primed CD(4+) T lymphocytes
    Claude Carnaud
    Inserm UMR_S 938, UPMC University Paris 06, Hopital St Antoine, Paris, France
    Prion 4:66-71. 2010
    b>Prion diseases are neurodegenerative conditions caused by the transconformation of a normal host glycoprotein, the cellular prion protein (PrPc) into a neurotoxic, self-aggregating conformer (PrPSc)...
  81. ncbi Understanding the natural variability of prion diseases
    Markus Geissen
    Institute of Neuropathology, Universitatsklinikum Hamburg Eppendorf, 20246 Hamburg, Germany
    Vaccine 25:5631-6. 2007
    b>Prion diseases are a heterogeneous group of disorders with an invariably fatal disease course. Although various etiologies have been proposed it is apparent that at least a subset of these diseases are of infectious nature...
  82. ncbi Diverse effects on the native β-sheet of the human prion protein due to disease-associated mutations
    Wei Chen
    Department of Bioengineering, University of Washington, Seattle, Washington 98195 5013, United States
    Biochemistry 49:9874-81. 2010
    b>Prion diseases are fatal neurodegenerative disorders that involve the conversion of the normal cellular form of the prion protein (PrP(C)) to a misfolded pathogenic form (PrP(Sc))...
  83. ncbi Psychotropic medications and the treatment of human prion diseases
    Brian S Appleby
    Johns Hopkins University School of Medicine, Department of Psychiatry and Behavioral Sciences, Division of Geriatric Psychiatry and Neuropsychiatry, Baltimore, MD, USA
    CNS Neurol Disord Drug Targets 8:353-62. 2009
    b>Prion diseases are rare, rapidly progressive, fatal neurodegenerative illnesses caused by an abnormal isoform of the native prion protein...
  84. ncbi Alzheimer's and prion diseases: distinct pathologies, common proteolytic denominators
    Frederic Checler
    IPMC du CNRS, UMR6097, 660 route des Lucioles, 06560 Valbonne, France
    Trends Neurosci 25:616-20. 2002
    ..It is our opinion that targeting these disintegrins with specific 'activators' could be a suitable strategy to slow down, or even arrest, betaAPP and PrP(c)-related aggregation and toxicity...
  85. ncbi Immunopurification of pathological prion protein aggregates
    Emiliano Biasini
    Dulbecco Telethon Institute, Milan, Italy
    PLoS ONE 4:e7816. 2009
    b>Prion diseases are fatal neurodegenerative disorders that can arise sporadically, be genetically inherited or acquired through infection...
  86. ncbi Prion infection of mouse neurospheres
    Ranjit K Giri
    McLaughlin Research Institute, Great Falls, MT 59405, USA
    Proc Natl Acad Sci U S A 103:3875-80. 2006
    ..Neurosphere lines from Tg4053 mice provide a sensitive in vitro bioassay for mouse prions; neurosphere lines from other Tg mice overexpressing PrP might be used to assay prions from other species, including humans...
  87. ncbi Prominent pancreatic endocrinopathy and altered control of food intake disrupt energy homeostasis in prion diseases
    J D Bailey
    Department of Veterinary Molecular Biology, Montana State University, Bozeman, Montana 59717, USA
    J Endocrinol 197:251-63. 2008
    b>Prion diseases are fatal neurodegenerative diseases that can induce endocrinopathies...
  88. ncbi p38 MAP kinase mediates the cell death induced by PrP106-126 in the SH-SY5Y neuroblastoma cells
    Stefano Thellung
    Department of Oncology, Biology and Genetics, National Institute for Cancer Research IST c o, Genova, Italy
    Neurobiol Dis 9:69-81. 2002
    b>Prion diseases are neurodegenerative pathologies characterized by the accumulation in the brain of a protease-resistant form of the prion protein (PrP(c)), named PrP(Sc)...
  89. ncbi Immunotherapy for prion diseases: opportunities and obstacles
    Li Li
    University of British Columbia and Vancouver Coastal Health Research Institute, Vancouver, British Columbia, Canada
    Immunotherapy 2:269-82. 2010
    ..These potentials, in addition to the ongoing baseline of familial and sporadic human prion diseases, necessitate development of effective treatment options...
  90. ncbi Creutzfeldt-Jakob disease: reflections on the risk from blood product therapy
    P Brown
    Commissariat a l Energie Atomique, Fontenay aux Roses, France and Fondation Alliance BioSécure, Les Ulis, France
    Haemophilia 13:33-40. 2007
    ..Strategies to prevent iatrogenic transmissions include low-risk sourcing, leucodepletion, and a variety of infectivity-reducing plasma processing steps; screening tests to detect infection in preclinical donors are under development...
  91. ncbi Nonenzymatic glycation at the N terminus of pathogenic prion protein in transmissible spongiform encephalopathies
    Yeong Gon Choi
    Ilsong Institute of Life Science, College of Medicine, Hallym University, Anyang, Kyeonggi Do, Republic of Korea
    J Biol Chem 279:30402-9. 2004
    ..Furthermore, immunostaining studies indicate that, at least in clinically affected hamsters, astrocytes are the first site of this glycation process...
  92. ncbi Prion protein with an insertional mutation accumulates on axonal and dendritic plasmalemma and is associated with distinctive ultrastructural changes
    Martin Jeffrey
    Veterinary Laboratories Agency, Lasswade Laboratory, Pentlands Science Park, Bush Loan, Penicuik, Midlothian, Scotland
    Am J Pathol 175:1208-17. 2009
    b>Prion diseases are fatal neurological diseases characterized by central nervous system deposition of abnormal forms of a membrane glycoprotein designated PrP (prion protein)...
  93. ncbi Prions: health scare and biological challenge
    A Aguzzi
    Institute of Neuropathology, University of Zurich, Schmelzbergstrasse 12, CH 8091 Zurich, Switzerland
    Nat Rev Mol Cell Biol 2:118-26. 2001
    Although human prion diseases are rare, the incidence of 'new variant' Creutzfeldt-Jakob disease in the United Kingdom is increasing exponentially...
  94. ncbi Progress and problems in the biology, diagnostics, and therapeutics of prion diseases
    Adriano Aguzzi
    Institute of Neuropathology, University Hospital Zurich, Zurich, Switzerland
    J Clin Invest 114:153-60. 2004
    ..In this article, we refer to the prion as the transmissible principle underlying prion diseases, without necessarily implying any specific biochemical or structural identity...
  95. ncbi Current and future molecular diagnostics for prion diseases
    Marty T Lehto
    Amorfix Life Sciences, 3080 Yonge Street, Suite 6020, Toronto, M4N 3N1, Canada
    Expert Rev Mol Diagn 6:597-611. 2006
    ..This review highlights some of the conventional and disruptive technologies developed to respond to this challenge...
  96. ncbi A mathematical analysis of the dynamics of prion proliferation
    Meredith L Greer
    Department of Mathematics, Bates College, 213 Hathorn Hall Bates College, Lewiston, Maine 04240, USA
    J Theor Biol 242:598-606. 2006
    ..We also prove local stability results for the disease steady state, and a global stability result for the disease free steady state. Finally, we give numerical simulations, which are confirmed by experimental data...
  97. ncbi [Neuropathological diagnosis of prion disease]
    Shigeo Murayama
    Department of Neuropathology, Tokyo Metropolitan Institute of Gerontology and Tokyo Metropolitan Geriatric Hospital
    Nihon Rinsho 65:1401-6. 2007
    ..Variant CJD, apparently derived from bovine spongiformic encephalopathy, requires shift in disease control strategy, in that PrP(Sc) is present in peripheral lymphatic organs...
  98. ncbi A novel, resistance-linked ovine PrP variant and its equivalent mouse variant modulate the in vitro cell-free conversion of rPrP to PrP(res)
    Louise Kirby
    Institute for Animal Health, Compton Laboratories, Newbury, Berkshire RG20 7NN, UK
    J Gen Virol 87:3747-51. 2006
    b>Prion diseases are associated with the conversion of the normal cellular prion protein, PrP(c), to the abnormal, disease-associated form, PrP(Sc). This conversion can be mimicked in vitro by using a cell-free conversion assay...
  99. ncbi Prions: protein only or something more? Overview of potential prion cofactors
    Carlo Fasano
    Unité de Trafic Membranaire et Pathogénèse, Institut Pasteur, 75724 Paris Cedex 15, France
    J Mol Neurosci 29:195-214. 2006
    ..In this review we discuss recent findings such as the cellular factors that might be involved in the conformational conversion of prion proteins and the potential mechanisms by which they could operate...
  100. ncbi The expanding universe of prion diseases
    Joel C Watts
    Centre for Research in Neurodegenerative Diseases and Department of Laboratory Medicine and Pathobiology, University of Toronto, Toronto, Ontario, Canada
    PLoS Pathog 2:e26. 2006
    ..Unfortunately, the known universe of prion diseases is expanding...
  101. ncbi 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))...

Research Grants95

  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
    Judd Aiken; Fiscal Year: 2009
    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
    Joel A Pedersen; Fiscal Year: 2010
    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 D Zabel; Fiscal Year: 2010
    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 Zabel; Fiscal Year: 2009
    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 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. ..
  13. 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. ..
  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. 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. ..
  22. 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 ..
  23. 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 ..
  24. 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...
  25. 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...
  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 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. ..
  28. 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. ..
  29. 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...
  30. 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...
  31. 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...
  32. 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...
  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. ..