prion protein

Summary

Gene Symbol: prion protein
Description: prion protein
Alias: ASCR, AltPrP, CD230, CJD, GSS, KURU, PRIP, PrP, PrP27-30, PrP33-35C, PrPc, p27-30, CD230 antigen, major prion protein, prion-related protein
Species: human

Top Publications

  1. ncbi First experimental transmission of fatal familial insomnia
    J Tateishi
    Department of Neuropathology, Kyushu University, Fukuoka, Japan
    Nature 376:434-5. 1995
  2. ncbi Comparative analysis of normal prion protein expression on human, rodent, and ruminant blood cells by using a panel of prion antibodies
    G Robin Barclay
    Scottish National Blood Transfusion Service Cell Therapy Group, University of Edinburgh, Western General Hospital, Edinburgh, United Kingdom
    Transfusion 42:517-26. 2002
  3. ncbi Polymorphisms of the prion protein gene (PRNP) in a Korean population
    Byung Hoon Jeong
    Ilsong Institute of Life Science, Hallym University, Ilsong Building, 1605 4, Gwanyang dong, Dongan Gu, 431 060, Anyang, Kyounggi Do, South Korea
    J Hum Genet 49:319-24. 2004
  4. pmc 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
  5. pmc Prion protein interacts with BACE1 protein and differentially regulates its activity toward wild type and Swedish mutant amyloid precursor protein
    Heledd H Griffiths
    Institute of Molecular and Cellular Biology, Faculty of Biological Sciences, University of Leeds, Leeds LS2 9JT, United Kingdom
    J Biol Chem 286:33489-500. 2011
  6. pmc Interaction between human prion protein and amyloid-beta (Abeta) oligomers: role OF N-terminal residues
    Shugui Chen
    Department of Physiology and Biophysics, Case Western Reserve University, Cleveland, Ohio 44106, USA
    J Biol Chem 285:26377-83. 2010
  7. pmc Co-existence of scrapie prion protein types 1 and 2 in sporadic Creutzfeldt-Jakob disease: its effect on the phenotype and prion-type characteristics
    Ignazio Cali
    Department of Pathology, Case Western Reserve University, 2085 Adelbert Road, Cleveland, OH 44106, USA
    Brain 132:2643-58. 2009
  8. pmc Oxidation of Helix-3 methionines precedes the formation of PK resistant PrP
    Tamar Canello
    Department of Neurology, The Agnes Ginges Center for Human Neurogenetics, Hadassah University Hospital, and Institute of Chemistry, The Hebrew University of Jerusalem, Jerusalem, Israel
    PLoS Pathog 6:e1000977. 2010
  9. pmc NMR solution structure of the human prion protein
    R Zahn
    Institut fur Molekularbiologie und Biophysik, Eidgenossische Technische Hochschule Honggerberg, CH 8093 Zurich, Switzerland
    Proc Natl Acad Sci U S A 97:145-50. 2000
  10. pmc Expression of mutant or cytosolic PrP in transgenic mice and cells is not associated with endoplasmic reticulum stress or proteasome dysfunction
    Elena Quaglio
    Dulbecco Telethon Institute, Milan, Italy
    PLoS ONE 6:e19339. 2011

Research Grants

Detail Information

Publications309 found, 100 shown here

  1. ncbi First experimental transmission of fatal familial insomnia
    J Tateishi
    Department of Neuropathology, Kyushu University, Fukuoka, Japan
    Nature 376:434-5. 1995
    ..Here we report the successful transmission of the disease to experimental animals, placing FFI within the group of infectious cerebral amyloidoses...
  2. ncbi Comparative analysis of normal prion protein expression on human, rodent, and ruminant blood cells by using a panel of prion antibodies
    G Robin Barclay
    Scottish National Blood Transfusion Service Cell Therapy Group, University of Edinburgh, Western General Hospital, Edinburgh, United Kingdom
    Transfusion 42:517-26. 2002
    ..The expression of normal cellular prion protein (PrPC) by different blood cell types may permit selective uptake and dissemination of infectivity.
  3. ncbi Polymorphisms of the prion protein gene (PRNP) in a Korean population
    Byung Hoon Jeong
    Ilsong Institute of Life Science, Hallym University, Ilsong Building, 1605 4, Gwanyang dong, Dongan Gu, 431 060, Anyang, Kyounggi Do, South Korea
    J Hum Genet 49:319-24. 2004
    Human prion protein gene (PRNP) has been considered to be involved in the susceptibility of humans to prion diseases...
  4. pmc 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
    ..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))...
  5. pmc Prion protein interacts with BACE1 protein and differentially regulates its activity toward wild type and Swedish mutant amyloid precursor protein
    Heledd H Griffiths
    Institute of Molecular and Cellular Biology, Faculty of Biological Sciences, University of Leeds, Leeds LS2 9JT, United Kingdom
    J Biol Chem 286:33489-500. 2011
    ..We have reported previously that the cellular prion protein (PrP(C)) inhibited the action of BACE1 toward human wild type APP (APP(WT)) in cellular models and that the ..
  6. pmc Interaction between human prion protein and amyloid-beta (Abeta) oligomers: role OF N-terminal residues
    Shugui Chen
    Department of Physiology and Biophysics, Case Western Reserve University, Cleveland, Ohio 44106, USA
    J Biol Chem 285:26377-83. 2010
    ..that at least some of the neurotoxic effects of these oligomers may be mediated by specific binding to the prion protein, PrP(C), on the cell surface (Laurén, J., Gimbel, D. A., Nygaard, H. B., Gilbert, J. W., and Strittmatter, S...
  7. pmc Co-existence of scrapie prion protein types 1 and 2 in sporadic Creutzfeldt-Jakob disease: its effect on the phenotype and prion-type characteristics
    Ignazio Cali
    Department of Pathology, Case Western Reserve University, 2085 Adelbert Road, Cleveland, OH 44106, USA
    Brain 132:2643-58. 2009
    ..Creutzfeldt-Jakob disease (sCJD), based on the methionine/valine polymorphic genotype of codon 129 of the prion protein (PrP) gene and the presence of either one of the two protease K-resistant scrapie prion protein (PrP(Sc)) ..
  8. pmc Oxidation of Helix-3 methionines precedes the formation of PK resistant PrP
    Tamar Canello
    Department of Neurology, The Agnes Ginges Center for Human Neurogenetics, Hadassah University Hospital, and Institute of Chemistry, The Hebrew University of Jerusalem, Jerusalem, Israel
    PLoS Pathog 6:e1000977. 2010
    ..at both Met residues 206 and 213 destabilize the alpha-fold, suggesting oxidation may facilitate the conversion of PrPC into PrPSc...
  9. pmc NMR solution structure of the human prion protein
    R Zahn
    Institut fur Molekularbiologie und Biophysik, Eidgenossische Technische Hochschule Honggerberg, CH 8093 Zurich, Switzerland
    Proc Natl Acad Sci U S A 97:145-50. 2000
    The NMR structures of the recombinant human prion protein, hPrP(23-230), and two C-terminal fragments, hPrP(90-230) and hPrP(121-230), include a globular domain extending from residues 125-228, for which a detailed structure was obtained,..
  10. pmc Expression of mutant or cytosolic PrP in transgenic mice and cells is not associated with endoplasmic reticulum stress or proteasome dysfunction
    Elena Quaglio
    Dulbecco Telethon Institute, Milan, Italy
    PLoS ONE 6:e19339. 2011
    The cellular pathways activated by mutant prion protein (PrP) in genetic prion diseases, ultimately leading to neuronal dysfunction and degeneration, are not known...
  11. doi Green tea extracts interfere with the stress-protective activity of PrP and the formation of PrP
    Angelika S Rambold
    Department of Biochemistry, Neurobiochemistry, Ludwig Maximilians Universitat Munchen, Munchen, Germany
    J Neurochem 107:218-29. 2008
    A hallmark in prion diseases is the conformational transition of the cellular prion protein (PrP(C)) into a pathogenic conformation, designated scrapie prion protein (PrP(Sc)), which is the essential constituent of infectious prions...
  12. pmc Defining sporadic Creutzfeldt-Jakob disease strains and their transmission properties
    Matthew T Bishop
    National Creutzfeldt Jakob Disease Surveillance Unit, Western General Hospital, University of Edinburgh, Edinburgh EH4 2XU, United Kingdom
    Proc Natl Acad Sci U S A 107:12005-10. 2010
    ..To categorize sCJD cases, the prion protein (PrP) codon 129 genotype and the biochemical characteristics of the disease-associated form of PrP (PrP(Sc)) ..
  13. 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
    ..disorders including Creutzfeldt-Jakob disease (CJD) in humans, is the conversion of the normal or cellular prion protein (PrPC) into the abnormal or scrapie isoform (PrPSc)...
  14. pmc Cellular prion protein expression is not regulated by the Alzheimer's amyloid precursor protein intracellular domain
    Victoria Lewis
    Institute of Molecular and Cellular Biology, Faculty of Biological Sciences, University of Leeds, Leeds, United Kingdom
    PLoS ONE 7:e31754. 2012
    ..The cellular prion protein, PrP(C), modulates the post-translational processing of the AD amyloid precursor protein (APP), through its ..
  15. pmc Unaltered prion protein expression in Alzheimer disease patients
    Eri Saijo
    Department of Microbiology, Immunology and Molecular Genetic, University of Kentucky Medical Center, Lexington, KY, USA
    Prion 5:109-16. 2011
    The suggested role of cellular prion protein (PrP (C) ) in mediating the toxic effects of oligomeric amyloid β peptide (Aβ) in Alzheimer disease (AD) is controversial...
  16. ncbi Molecular modulation of expression of prion protein by heat shock
    Woei Cherng Shyu
    Department of Neurology, Mackay Memorial Hospital, Taipei, Taiwan, ROC
    Mol Neurobiol 26:1-12. 2002
    ..spongiform encephalopathies) are associated with the conversion of the normal cellular form of the prion protein (PrPC) to an abnormal scrapie-isoform (PrP(Sc)...
  17. doi Prion-like aggregates: infectious agents in human disease
    Gunilla T Westermark
    Department of Medical Cell Biology and Department of Genetics and Pathology, Uppsala University, Uppsala, Sweden
    Trends Mol Med 16:501-7. 2010
    ..The misfolding and aggregation of the prion protein are akin to the genesis of amyloid fibrils formed by several human and animal proteins associated with more ..
  18. doi Human prion protein mutants with deleted and inserted octarepeats undergo different pathways to trigger cell apoptosis
    Kun Xu
    Department of Microbiology, School of Medicine, Xi an Jiao Tong University, Xi an, 710061, People s Republic of China
    J Mol Neurosci 43:225-34. 2011
    Octarepeats region sequence is one of the most important characteristics of PrP topology...
  19. pmc Familial CJD associated PrP mutants within transmembrane region induced Ctm-PrP retention in ER and triggered apoptosis by ER stress in SH-SY5Y cells
    Xin Wang
    State Key Laboratory for Infectious Disease Prevention and Control, National Institute for Viral Disease Control and Prevention, Chinese Center for Disease Control and Prevention, Beijing, People s Republic of China
    PLoS ONE 6:e14602. 2011
    Genetic prion diseases are linked to point and inserted mutations in the prion protein (PrP) gene that are presumed to favor conversion of the cellular isoform of PrP (PrP(C)) to the pathogenic one (PrP(Sc))...
  20. ncbi Clinical features of rapidly progressive Alzheimer's disease
    Christian Schmidt
    Department of Neurology, Hospital of the Georg August University Goettingen, Goettingen, Germany
    Dement Geriatr Cogn Disord 29:371-8. 2010
    ..To characterize clinical features, CSF biomarkers and genetic polymorphisms of patients suffering from a rapidly progressing subtype of Alzheimer's dementia (rpAD)...
  21. doi PRNP allelic series from 19 years of prion protein gene sequencing at the MRC Prion Unit
    Jon A Beck
    MRC Prion Unit, Department of Neurodegenerative Disease, UCL Institute of Neurology, National Hospital for Neurology and Neurosurgery, Queen Square, London, WC1N 3BG, UK
    Hum Mutat 31:E1551-63. 2010
    Mutation of the human prion protein gene (PRNP) open reading frame (ORF) accounts for almost all reported familial concurrence of prion disease...
  22. doi Heat shock protein 104 inhibited the fibrillization of prion peptide 106-126 and disassembled prion peptide 106-126 fibrils in vitro
    Ying hui Liu
    State Key Laboratory for Infectious Disease Prevention and Control, National Institute for Viral Disease Control and Prevention, Chinese Center for Disease Control and Prevention, Ying Xin Rd 100, Beijing 100052, People s Republic of China
    Int J Biochem Cell Biol 43:768-74. 2011
    ..Prion peptide of aa 106-126 (PrP106-126) exhibits many PrP(Sc)-like biochemical features, forming amyloid-like fibrils in vitro...
  23. doi Alzheimer's disease brain-derived amyloid-β-mediated inhibition of LTP in vivo is prevented by immunotargeting cellular prion protein
    Andrew E Barry
    Department of Pharmacology and Therapeutics and Trinity College Institute of Neuroscience, Trinity College Dublin, Dublin 2, Ireland
    J Neurosci 31:7259-63. 2011
    Synthetic amyloid-β protein (Aβ) oligomers bind with high affinity to cellular prion protein (PrP(C)), but the role of this interaction in mediating the disruption of synaptic plasticity by such soluble Aβ in vitro is controversial...
  24. pmc Assessment of Alzheimer's disease case-control associations using family-based methods
    Brit Maren M Schjeide
    MassGeneral Institute for Neurodegenerative Disease MIND, Department of Neurology, Massachusetts General Hospital, Charlestown, MA 02129, USA
    Neurogenetics 10:19-25. 2009
    ..Further fine-mapping and functional analyses are warranted to elucidate the potential biochemical mechanisms and epidemiological relevance of these genes...
  25. pmc Pathogenic mutations in the hydrophobic core of the human prion protein can promote structural instability and misfolding
    Marc W van der Kamp
    Department of Bioengineering, University of Washington, Seattle, WA 98195 5013, USA
    J Mol Biol 404:732-48. 2010
    Transmissible spongiform encephalopathies, or prion diseases, are caused by misfolding and aggregation of the prion protein PrP...
  26. doi Regulation of prion gene expression by transcription factors SP1 and metal transcription factor-1
    Shayne A Bellingham
    Department of Biochemistry and Molecular Biology, The University of Melbourne, Victoria, Australia
    J Biol Chem 284:1291-301. 2009
    Prion diseases are associated with the conformational conversion of the host-encoded cellular prion protein into an abnormal pathogenic isoform...
  27. ncbi The expression and potential function of cellular prion protein in human lymphocytes
    R Li
    Institute of Pathology, Division of Neuropathology, Case Western Reserve University School of Medicine, 10900 Euclid Avenue, Cleveland, Ohio 44106 4943, USA
    Cell Immunol 207:49-58. 2001
    We examined expression of the normal cellular prion protein (PrP(C)) in human peripheral blood mononuclear cells (PBMC) and in transfected neuroblastoma cells with a panel of six monoclonal antibodies (Mabs)...
  28. pmc Methionine sulfoxides on prion protein Helix-3 switch on the alpha-fold destabilization required for conversion
    Giorgio Colombo
    Isto di Chimica del Riconoscimento Molecolare, Consiglio Nazionale delle Ricerche, Milano, Italy
    PLoS ONE 4:e4296. 2009
    The conversion of the cellular prion protein (PrP(C)) into the infectious form (PrP(Sc)) is the key event in prion induced neurodegenerations...
  29. doi Human prion protein (PrP) 219K is converted to PrPSc but shows heterozygous inhibition in variant Creutzfeldt-Jakob disease infection
    Masaki Hizume
    Division of CJD Science and Technology, Tohoku University Graduate School of Medicine, Sendai 980 8575, Japan
    J Biol Chem 284:3603-9. 2009
    b>Prion protein gene (PRNP) E219K is a human polymorphism commonly occurring in Asian populations but is rarely found in patients with sporadic Creutzfeldt-Jakob disease (CJD)...
  30. pmc Prion protein amyloidosis with divergent phenotype associated with two novel nonsense mutations in PRNP
    Casper Jansen
    Dutch Surveillance Centre for Prion Diseases, University Medical Centre Utrecht, 3584 CX Utrecht, The Netherlands
    Acta Neuropathol 119:189-97. 2010
    Stop codon mutations in the gene encoding the prion protein (PRNP) are very rare and have thus far only been described in two patients with prion protein cerebral amyloid angiopathy (PrP-CAA)...
  31. pmc Molecular biology and pathology of prion strains in sporadic human prion diseases
    Pierluigi Gambetti
    Department of Pathology, Case Western Reserve University, 2085 Adelbert Road, Cleveland, OH, 44106, USA
    Acta Neuropathol 121:79-90. 2011
    ..to propagate by the mechanism involving self-perpetuating conformational conversion of the normal form of the prion protein, PrP(C), to the misfolded, pathogenic state, PrP(Sc)...
  32. doi Cytosolic PrP induces apoptosis of cell by disrupting microtubule assembly
    Xiao Li Li
    State Key Laboratory for Infectious Disease Prevention and Control, National Institute for Viral Disease Control and Prevention, Chinese Center for Disease Control and Prevention, Ying Xin Rd 100, Beijing, 100052, People s Republic of China
    J Mol Neurosci 43:316-25. 2011
    b>Prion protein (PrP) is able to bind with tubulin and to interfere with the formation of microtubule. To investigate the influence of accumulation of cytosolic PrP in cytoplasm on microtubule, plasmid pcDNA3...
  33. ncbi Prion proteins with insertion mutations have altered N-terminal conformation and increased ligand binding activity and are more susceptible to oxidative attack
    Shaoman Yin
    Department of Pathology, School of Medicine, Case Western Reserve University, Cleveland, Ohio 44120, USA
    J Biol Chem 281:10698-705. 2006
    We compared the biochemical properties of a wild type recombinant normal human cellular prion protein, rPrP(c), with a recombinant mutant human prion protein that has three additional octapeptide repeats, rPrP(8OR)...
  34. ncbi Regulatory sequences of the PRNP gene influence susceptibility to sporadic Creutzfeldt-Jakob disease
    Jolanta Bratosiewicz-Wasik
    Department of Virology, Medical University of Silesia, 41 200 Sosnowiec, Narcyzow 1, Poland, and Department of Pathology and Laboratory Medicine, The Ottawa Hospital, Canada
    Neurosci Lett 411:163-7. 2007
    The prion diseases are fatal neurodegenerative disorders that afflict both humans and animals. They comprise kuru, Creutzfeldt-Jakob disease (CJD), Gerstmman-Straussler-Scheinker syndrome (GSS), and fatal familial insomnia (FFI)...
  35. pmc Molecular architecture of human prion protein amyloid: a parallel, in-register beta-structure
    Nathan J Cobb
    Department of Physiology and Biophysics, Case Western Reserve University, Cleveland, OH 44106, USA
    Proc Natl Acad Sci U S A 104:18946-51. 2007
    ..diseases that are associated with conformational conversion of the normally monomeric and alpha-helical prion protein, PrP(C), to the beta-sheet-rich PrP(Sc)...
  36. doi Gerstmann-Sträussler-Scheinker disease
    Paweł P Liberski
    Department of Molecular Pathology and Neuropathology, Medical University Lodz, Lodz, Poland
    Adv Exp Med Biol 724:128-37. 2012
    ..and the first human transmissible spongiform encephalopathy (TSE) in which a mutation in a gene encoding for prion protein (PrP) was discovered...
  37. pmc Identification of adjacent binding sites for the YY1 and E4BP4 transcription factors in the ovine PrP (Prion) gene promoter
    Stewart T G Burgess
    Roslin Institute and R D SVS, Neuropathogenesis Division, University of Edinburgh, Roslin, Midlothian EH25 9PS, United Kingdom
    J Biol Chem 284:6716-24. 2009
    The PrP gene encodes the cellular isoform of the prion protein (PrP(c)) which has been shown to be crucial to the development of transmissible spongiform encephalopathies (TSEs)...
  38. pmc A polymorphism in the regulatory region of PRNP is associated with increased risk of sporadic Creutzfeldt-Jakob disease
    Pascual Sanchez-Juan
    Neurology Department, University Hospital Marqués de Valdecilla and CIBERNED, Santander, Spain
    BMC Med Genet 12:73. 2011
    ..An important determinant for CJD risk and phenotype is the M129V polymorphism of the human prion protein gene (PRNP), but there are also other coding and non-coding polymorphisms inside this gene.
  39. ncbi Identification of distinct N-terminal truncated forms of prion protein in different Creutzfeldt-Jakob disease subtypes
    Gianluigi Zanusso
    Department of Neurological and Visual Sciences, Section of Neurology and Agricultural and Industrial Biotechnologies, University of Verona, 37134 Verona, Italy
    J Biol Chem 279:38936-42. 2004
    In prion diseases, the cellular prion protein (PrP(C)) is converted to an insoluble and protease-resistant abnormal isoform termed PrP(Sc)...
  40. ncbi The prion protein has RNA binding and chaperoning properties characteristic of nucleocapsid protein NCP7 of HIV-1
    C Gabus
    LaboRetro, Unité de Virologie Humaine INSERM Ecole Normale Superieure de Lyon ENS 412, ENS de Lyon, 46 allee d Italie, Lyon 69364, France
    J Biol Chem 276:19301-9. 2001
    ..are fatal neurodegenerative diseases associated with the accumulation of a protease-resistant form of the prion protein (PrP). Although PrP is conserved in vertebrates, its function remains to be identified...
  41. doi Prion protein expression alters APP cleavage without interaction with BACE-1
    Patrick C McHugh
    Department of Biology and Biochemistry, University of Bath, Bath BA2 7AY, UK
    Neurochem Int 61:672-80. 2012
    The prion protein (PrP) and the beta-site amyloid precursor protein (APP) cleaving enzyme 1 (BACE-1) are both copper binding proteins, but are associated with two separate neurodegenerative diseases...
  42. doi No evidence for prion protein gene locus multiplication in Creutzfeldt-Jakob disease
    Steven J Collins
    Australian National CJD Registry, Department of Pathology, The University of Melbourne, Parkville 3010, Australia
    Neurosci Lett 472:16-8. 2010
    ..of a similar disorder occurs in around 50-90% of patients harboring the most common, disease-associated, prion protein gene (PRNP) mutations...
  43. pmc Structural polymorphism in amyloids: new insights from studies with Y145Stop prion protein fibrils
    Eric M Jones
    Department of Physiology and Biophysics, Case Western Reserve University, Cleveland, Ohio 44106, USA
    J Biol Chem 286:42777-84. 2011
    The C-terminally-truncated human prion protein variant Y145Stop (or PrP23-144), associated with a familial prion disease, provides a valuable model for studying the fundamental properties of protein amyloids...
  44. pmc Mutant prion protein expression is associated with an alteration of the Rab GDP dissociation inhibitor alpha (GDI)/Rab11 pathway
    Tania Massignan
    Dulbecco Telethon Institute DTI c o Istituto di Ricerche Farmacologiche Mario Negri, Milan, Italy
    Mol Cell Proteomics 9:611-22. 2010
    The prion protein (PrP) is a glycosylphosphatidylinositol-anchored membrane glycoprotein that plays a vital role in prion diseases, a class of fatal neurodegenerative disorders of humans and animals...
  45. doi Hypoxia-inducible factor-1 α regulates prion protein expression to protect against neuron cell damage
    Jae Kyo Jeong
    Center for Healthcare Technology Development, Korea Zoonoses Research Institute, College of Veterinary Medicine, Chonbuk National University, Jeonju, Jeonbuk, South Korea
    Neurobiol Aging 33:1006.e1-10. 2012
    The human prion protein fragment, PrP (106-126), may contain a majority of the pathological features associated with the infectious scrapie isoform of PrP, known as PrP(Sc)...
  46. pmc Spontaneous generation of prion infectivity in fatal familial insomnia knockin mice
    Walker S Jackson
    Whitehead Institute for Biomedical Research, Nine Cambridge Center, Cambridge, MA 02142, USA
    Neuron 63:438-50. 2009
    A crucial tenet of the prion hypothesis is that misfolding of the prion protein (PrP) induced by mutations associated with familial prion disease is, in an otherwise normal mammalian brain, sufficient to generate the infectious agent...
  47. ncbi Truncated forms of the human prion protein in normal brain and in prion diseases
    S G Chen
    Division of Neuropathology, Case Western Reserve University, Cleveland, Ohio 44106, USA
    J Biol Chem 270:19173-80. 1995
    The cellular form of the prion protein (PrPc) is a glycoprotein anchored to the cell membrane by a glycosylphosphatidylinositol moiety...
  48. doi Total prion protein levels in the cerebrospinal fluid are reduced in patients with various neurological disorders
    Felix Meyne
    National TSE Reference Center at Department of Neurology, Georg August University, Gottingen, Germany
    J Alzheimers Dis 17:863-73. 2009
    We performed a study on levels of the total prion protein (PrP) in humans affected by different neurological diseases and assessed the influence of several factors such as age, gender, and disease severity on the cerebrospinal fluid PrP ..
  49. pmc Co-occurrence of types 1 and 2 PrP(res) in sporadic Creutzfeldt-Jakob disease MM1
    Atsushi Kobayashi
    Division of Neurological Science, Center for Prion Research, Tohoku University Graduate School of Medicine, Sendai, Japan
    Am J Pathol 178:1309-15. 2011
    The genotype (M/M, M/V, or V/V) at polymorphic codon 129 of the human prion protein (PrP) gene and the type (1 or 2) of protease-resistant PrP (PrP(res)) in the brain are major determinants of the clinicopathological phenotypes of ..
  50. ncbi A prion-linked psychiatric disorder
    H B Samaia
    Nature 390:241. 1997
  51. doi D178N, 129Val and N171S, 129Val genotype in a family with Creutzfeldt-Jakob disease
    Brian S Appleby
    Division of Geriatric Psychiatry and Neuropsychiatry, Johns Hopkins University School of Medicine, Baltimore, MD, USA
    Dement Geriatr Cogn Disord 30:424-31. 2010
    ..disease (CJD(D178N,)(129V)) are two phenotypes that share a common point mutation at codon 178 of the prion protein gene (PRNP), but differ in their polymorphism at codon 129 of the mutant allele...
  52. ncbi Atypical Creutzfeldt-Jakob disease in an American family with an insert mutation in the PRNP amyloid precursor gene
    P Brown
    Laboratory of CNS Studies, NINDS, NIH, Bethesda, MD 20892
    Neurology 42:422-7. 1992
    ..family of English origin with an unusually early onset and long-duration form of Creutzfeldt-Jakob disease (CJD) had a heterozygous insert mutation in the region of repeating octapeptide coding sequences between codons 51 and ..
  53. pmc PRNP variation in UK sporadic and variant Creutzfeldt Jakob disease highlights genetic risk factors and a novel non-synonymous polymorphism
    Matthew T Bishop
    National CJD Surveillance Unit, University of Edinburgh, Bryan Matthews Building, Western General Hospital, Crewe Road, Edinburgh, EH4 2XU, UK
    BMC Med Genet 10:146. 2009
    Genetic analysis of the human prion protein gene (PRNP) in suspect cases of Creutzfeldt-Jakob disease (CJD) is necessary for accurate diagnosis and case classification...
  54. pmc Design of anti- and pro-aggregation variants to assess the effects of methionine oxidation in human prion protein
    Christina Wolschner
    Max Planck Institut fur Biochemie, Am Klopferspitz 18, D 82152 Martinsried, Germany
    Proc Natl Acad Sci U S A 106:7756-61. 2009
    Prion disease is characterized by the alpha-->beta structural conversion of the cellular prion protein (PrP(C)) into the misfolded and aggregated "scrapie" (PrP(Sc)) isoform...
  55. pmc Beta-sheet core of human prion protein amyloid fibrils as determined by hydrogen/deuterium exchange
    Xiaojun Lu
    Department of Physiology and Biophysics, Case Western Reserve University, Cleveland, OH 44106, USA
    Proc Natl Acad Sci U S A 104:1510-5. 2007
    Propagation of transmissible spongiform encephalopathies is associated with the conversion of normal prion protein, PrP(C), into a misfolded, oligomeric form, PrP(Sc)...
  56. ncbi Presence of a "CAGA box" in the APP gene unique to amyloid plaque-forming species and absent in all APLP-1/2 genes: implications in Alzheimer's disease
    Bryan Maloney
    Departments of Psychiatry, Institute of Psychiatric Research, Indiana University School of Medicine, Indianapolis, Indiana 46202, USA
    FASEB J 18:1288-90. 2004
    ....
  57. ncbi Prion protein gene M129 allele is a risk factor for Alzheimer's disease
    M Gacia
    Department of Neurodegenerative Disorders, Medical Research Center, Polish Academy of Sciences, Warszawa, Poland
    J Neural Transm 113:1747-51. 2006
    b>Prion protein gene polymorphism M129V represents a known risk factor for Creutzfeldt-Jakob disease. Recently, the meta-analysis revealed that homozygosity at codon 129 is connected with increased risk of Alzheimer's disease (AD)...
  58. pmc A New Transgenic Mouse Model of Gerstmann-Straussler-Scheinker Syndrome Caused by the A117V Mutation of PRNP
    Wenbin Yang
    Department of Neurology, University of Chicago, Illinois 60637, USA
    J Neurosci 29:10072-80. 2009
    ..clinically by the development of progressive ataxia and dementia, and histopathologically by the presence of prion protein (PrP) amyloid plaques in the CNS, especially within the cerebellum...
  59. doi A novel protective prion protein variant that colocalizes with kuru exposure
    Simon Mead
    Medical Research Council Prion Unit, Department of Neurodegenerative Disease, University College London Institute of Neurology, United Kingdom
    N Engl J Med 361:2056-65. 2009
    b>Kuru is a devastating epidemic prion disease that affected a highly restricted geographic area of the Papua New Guinea highlands; at its peak, it predominantly affected adult women and children of both sexes...
  60. ncbi Signal transduction through prion protein
    S Mouillet-Richard
    Différenciation Cellulaire, CNRS Institut Pasteur, 75724 Paris Cedex 15, France
    Science 289:1925-8. 2000
    The cellular prion protein PrPc is a glycosylphosphatidylinositol-anchored cell-surface protein whose biological function is unclear...
  61. doi A novel insertional mutation in the prion protein gene: clinical and bio-molecular findings
    C Mauro
    Department of Neurological Sciences, First Division of Neurology, Second University of Naples, Via Pansini 5, 80131 Naples, Italy
    J Neurol Neurosurg Psychiatry 79:1395-8. 2008
    ..marked bi-parietal cerebral atrophy, was found to carry a novel seven extra-repeat insertional mutation in the prion protein gene (PRNP)...
  62. doi Codon 129 polymorphism of prion protein gene in sporadic Alzheimer's disease
    A Poleggi
    Department of Cell Biology and Neurosciences, Istituto Superiore di Sanita, Rome, Italy
    Eur J Neurol 15:173-8. 2008
    Codon 129 polymorphism of the prion protein gene represents a major genetic risk factor for Creutzfeldt-Jakob disease (CJD)...
  63. doi Survival in Alzheimer's disease is shorter in women carrying heterozygosity at codon 129 of the PRNP gene and no APOE epsilon 4 allele
    Claudia Giannattasio
    Department of Cell Biology and Neurosciences, Istituto Superiore di Sanita, Rome, Italy
    Dement Geriatr Cogn Disord 25:354-8. 2008
    We assessed the role of the APOE genotype and prion protein polymorphism at codon 129 in predicting the clinical duration of 92 neuropathologically confirmed sporadic Alzheimer's disease patients...
  64. ncbi Cytosolic prion protein induces apoptosis in human neuronal cell SH-SY5Y via mitochondrial disruption pathway
    Xin Wang
    State Key Laboratory for Infectious Disease Prevention and Control, National Institute for Viral Disease Control and Prevention, Chinese Center for Disease Control and Prevention, Ying Xin Rd 100, Beijing 100052, China
    BMB Rep 42:444-9. 2009
    ..Reverse-transfected cytosolic prion protein (PrP) and PrP expressed in the cytosol have been shown to be neurotoxic...
  65. ncbi The effect of disease-associated mutations on the folding pathway of human prion protein
    Adrian C Apetri
    Department of Physiology and Biophysics and Department of Chemistry, Case Western Reserve University, Cleveland, Ohio 44106, USA
    J Biol Chem 279:18008-14. 2004
    Propagation of transmissible spongiform encephalopathies is believed to involve the conversion of cellular prion protein, PrP(C), into a misfolded oligomeric form, PrP(Sc)...
  66. pmc Stress-inducible protein 1 is a cell surface ligand for cellular prion that triggers neuroprotection
    Silvio M Zanata
    Ludwig Institute for Cancer Research, Sao Paulo Branch, Rua Prof Antônio Prudente 109 4A, São Paulo 01509010, Brasil
    EMBO J 21:3307-16. 2002
    Prions are composed of an isoform of a normal sialoglycoprotein called PrP(c), whose physiological role has been under investigation, with focus on the screening for ligands...
  67. ncbi The C-terminal globular domain of the prion protein is necessary and sufficient for import into the endoplasmic reticulum
    Johanna Heske
    Department of Cellular Biochemistry, Max Planck Institut fur Biochemie, Am Klopferspitz 18, D 82152 Martinsried, Germany
    J Biol Chem 279:5435-43. 2004
    The mammalian prion protein (PrP) is composed of an unstructured flexible N-terminal region and a C-terminal globular domain...
  68. pmc Sporadic--but not variant--Creutzfeldt-Jakob disease is associated with polymorphisms upstream of PRNP exon 1
    S Mead
    MRC Prion Unit and Department of Neurogenetics, Imperial College, St Mary s Hospital, London, United Kingdom
    Am J Hum Genet 69:1225-35. 2001
    ..The etiology of classical (sporadic) CJD, which has a worldwide incidence, remains obscure...
  69. ncbi Cellular phenotyping of secretory and nuclear prion proteins associated with inherited prion diseases
    Holger Lorenz
    Institut für Neuropathologie, Ludwig Maximilians Universitat Munchen, D 81377 Munchen, Germany
    J Biol Chem 277:8508-16. 2002
    The pathogenic mechanisms leading from mutations in the prion protein (PrP) gene to infectious disease are not understood...
  70. ncbi All-trans retinoic acid down-regulates prion protein expression independently of granulocyte maturation
    C Rybner
    INSERM U 496, Institut Universitaire d Hématologie IUH, Hopital Saint Louis, Paris, France
    Leukemia 16:940-8. 2002
    The cellular prion protein (PrPc) is a sialoglycoprotein involved in the pathogenesis of prion diseases. It has been identified at the plasma membrane of several cell types...
  71. ncbi PRNP contains both intronic and upstream regulatory regions that may influence susceptibility to Creutzfeldt-Jakob Disease
    James E McCormack
    Neuropathogenesis Unit, Institute for Animal Health, West Mains Road, Edinburgh EH9 3JF, UK
    Gene 288:139-46. 2002
    The Prion protein (PrP) plays a central role in Creutzfeldt-Jakob Disease (CJD) and other transmissible spongiform encephalopathies (TSEs)...
  72. pmc Abbreviated incubation times for human prions in mice expressing a chimeric mouse-human prion protein transgene
    Carsten Korth
    Institute for Neurodegenerative Diseases and Department of Neurology, University of California, San Francisco, CA 94143, USA
    Proc Natl Acad Sci U S A 100:4784-9. 2003
    Transgenic (Tg) mouse lines that express chimeric mouse-human prion protein (PrP), designated MHu2M, are susceptible to prions from patients with sporadic Creutzfeldt-Jakob disease (sCJD)...
  73. ncbi The population distribution of the Met allele at the PRNP129 polymorphism (a high risk factor for Creutzfeldt-Jakob disease) in various regions of France and in West Europe
    Gérard Lucotte
    Centre de Neurogénétique Moléculaire, 44 rue Monge, Paris 75005, France
    Infect Genet Evol 5:141-4. 2005
    The present study has been conducted to ascertain the level of allelic variation at codon 129 of the prion protein gene in France...
  74. ncbi PRNP Val129 homozygosity increases risk for early-onset Alzheimer's disease
    Bart Dermaut
    Department of Molecular Genetics, Flanders Interuniversity Institute of Biotechnology VIB8, University of Antwerp, Antwerpen, Belgium
    Ann Neurol 53:409-12. 2003
    ..2; 95% CI, 1.4-7.1; p < 0.01). In patients with a positive family history, these risks increased to 2.6 (95% CI, 1.3-5.3; p < 0.01) and 3.5 (95% CI, 1.3-9.3; p = 0.01), respectively...
  75. ncbi Aggresome formation by mutant prion proteins: the unfolding role of proteasomes in familial prion disorders
    Ravi Shankar Mishra
    The Institute of Pathology, Case Western Reserve University, 2085, Adelbert Road, Cleveland, Ohio 44106, USA
    J Alzheimers Dis 5:15-23. 2003
    Although familial prion disorders are a direct consequence of mutations in the prion protein gene, the underlying mechanisms leading to neurodegeneration remain unclear...
  76. ncbi Alpha- and beta- cleavages of the amino-terminus of the cellular prion protein
    Alain Mangé
    Institut de Genetique Humaine, CNRS U P R 1142, 141, rue de la Cardonille, 34396 Montpellier Cedex 5, France
    Biol Cell 96:125-32. 2004
    It is commonly assumed that the physiological isoform of prion protein, PrP(C), is cleaved during its normal processing between residues 111/112, whereas the pathogenic isoform, PrP(Sc), is cleaved at an alternate site in the octapeptide ..
  77. doi Earlier onset of Alzheimer's disease: risk polymorphisms within PRNP, PRND, CYP46, and APOE genes
    Ewa Golanska
    Department of Molecular Pathology and Neuropathology, Medical University of Lodz, Lodz, Poland
    J Alzheimers Dis 17:359-68. 2009
    ..Moreover, there may be multiple sufficient risk sets for AD. Looking at multiple genes together rather than analyzing them individually, may improve identification of risk alleles...
  78. pmc Genetic risk factors for variant Creutzfeldt-Jakob disease: a genome-wide association study
    Simon Mead
    Medical Research Council Prion Unit and Department of Neurodegenerative Disease, Institute of Neurology, Queen Square, London, UK
    Lancet Neurol 8:57-66. 2009
    Human and animal prion diseases are under genetic control, but apart from PRNP (the gene that encodes the prion protein), we understand little about human susceptibility to bovine spongiform encephalopathy (BSE) prions, the causal agent ..
  79. ncbi PRNP 1368 polymorphism is not associated with sporadic Creutzfeldt-Jakob disease in the Korean population
    B H Jeong
    Ilsong Institute of Life Science, Hallym University, Dongan Gu, Anyang, Kyounggi Do, South Korea
    Eur J Neurol 15:846-50. 2008
    Human prion protein gene (PRNP) is considered a critical and fundamental gene in determining the incidence of human prion diseases...
  80. ncbi Association between deposition of beta-amyloid and pathological prion protein in sporadic Creutzfeldt-Jakob disease
    Laura Debatin
    Institute of Neuropathology, University Hospital Zurich, Zurich, Switzerland
    Neurodegener Dis 5:347-54. 2008
    ..an association between the deposition of the proteolytically processed form of the amyloid precursor protein, beta-amyloid (Abeta), which deposits in AD, and the abnormal form of the prion protein, PrP(Sc), which deposits in sCJD.
  81. ncbi Two different clinical phenotypes of Creutzfeldt-Jakob disease with a M232R substitution
    Yusei Shiga
    Department of Neurology, Tohoku University Graduate School of Medicine, 1 1 Seiryo machi, Aoba ku, Sendai 980 8574, Japan
    J Neurol 254:1509-17. 2007
    To describe the clinical features of Creutzfeldt-Jakob disease with a substitution of arginine for methionine (M232R substitution) at codon 232 (CJD232) of the prion protein gene (PRNP).
  82. ncbi Conditions of endoplasmic reticulum stress favor the accumulation of cytosolic prion protein
    Andrea Orsi
    Universita Vita Salute San Raffaele, DiBiT Istituto Scientifico San Raffaele, Via Olgettina 58, 20132 Milano, Italy
    J Biol Chem 281:30431-8. 2006
    After signal sequence-dependent targeting to the endoplasmic reticulum (ER), prion protein (PrP) undergoes several post-translational modifications, including glycosylation, disulfide bond formation, and the addition of a ..
  83. ncbi Codon 129 polymorphism of the human prion protein influences the kinetics of amyloid formation
    Patrick A Lewis
    MRC Prion Unit, Department of Neurodegenerative Disease, Institute of Neurology, University College London, Queen Square, London WC1N 3BG, UK
    J Gen Virol 87:2443-9. 2006
    The human prion protein (PrP) has a common polymorphism at residue 129, which can be valine or methionine. This polymorphism has a strong influence on susceptibility to prion diseases and on prion-strain properties...
  84. pmc Recombinant prion protein does not possess SOD-1 activity
    Samantha Jones
    MRC Prion Unit, Department of Neurodegenerative Diseases, Institute of Neurology, University College London, Queen Square, London WC1N 3BG, UK
    Biochem J 392:309-12. 2005
    A considerable body of evidence now shows that PrP (prion protein) binds metal ions with high affinity and it has been claimed that the binding of copper (II) ions to PrP confers SOD (superoxide dismutase) activity...
  85. ncbi PrP glycoforms are associated in a strain-specific ratio in native PrPSc
    Azadeh Khalili-Shirazi
    MRC Prion Unit, Department of Neurodegenerative Disease, Institute of Neurology, University College London, Queen Square, London WC1N 3BG, UK
    J Gen Virol 86:2635-44. 2005
    Prion diseases involve conversion of host-encoded cellular prion protein (PrPC) to a disease-related isoform (PrPSc)...
  86. ncbi Is M129V of PRNP gene associated with Alzheimer's disease? A case-control study and a meta-analysis
    Roberto Del Bo
    Dino Ferrari Centre, Department of Neurological Sciences, University of Milan and Fondazione IRCCS Ospedale Maggiore Policlinico, Mangiagalli e Regina Elena, Padiglione Ponti, Milano, Italy
    Neurobiol Aging 27:770.e1-770.e5. 2006
    The methionine/valine (M/V) polymorphism at codon 129 within the prion protein gene (PRNP) represents a known risk factor for Creutzfeldt-Jakob disease (CJD)...
  87. ncbi The chaperone protein BiP binds to a mutant prion protein and mediates its degradation by the proteasome
    T Jin
    Division of Neuropathology, Institute of Pathology, Case Western Reserve University, Cleveland, Ohio 44106, USA
    J Biol Chem 275:38699-704. 2000
    Familial prion diseases are thought to result from a change in structure of the mutant prion protein (PrP), which takes a pathogenic conformation...
  88. ncbi The residue 129 polymorphism in human prion protein does not confer susceptibility to Creutzfeldt-Jakob disease by altering the structure or global stability of PrPC
    Laszlo L P Hosszu
    Medical Research Council Prion Unit, Department of Neurodegenerative Disease, Institute of Neurology, University College London, Queen Square, London WC1N 3BG, United Kingdom
    J Biol Chem 279:28515-21. 2004
    There are two common forms of prion protein (PrP) in humans, with either methionine or valine at position 129...
  89. pmc Folding kinetics of the human prion protein probed by temperature jump
    Tanya Hart
    Medical Research Council Prion Unit, Institute of Neurology, Queen Square, London WC1N 3BG, United Kingdom
    Proc Natl Acad Sci U S A 106:5651-6. 2009
    Temperature-jump perturbation was used to examine the relaxation kinetics of folding of the human prion protein. Measured rates were very fast (approximately 3,000 s(-1)), with the extrapolated folding rate constant at approximately 20 ..
  90. pmc NMR structures of three single-residue variants of the human prion protein
    L Calzolai
    Institut fur Molekularbiologie und Biophysik, Eidgenossische Technische Hochschule, Honggerberg, CH 8093 Zurich, Switzerland
    Proc Natl Acad Sci U S A 97:8340-5. 2000
    The NMR structures of three single-amino acid variants of the C-terminal domain of the human prion protein, hPrP(121-230), are presented...
  91. ncbi A new point mutation of the prion protein gene in Creutzfeldt-Jakob disease
    M Pocchiari
    Laboratory of Virology, Istituto Superiore di Sanita, Rome, Italy
    Ann Neurol 34:802-7. 1993
    Complete sequencing of the prion protein open reading frame of a 68-year-old woman affected by a familial form of Creutzfeldt-Jakob disease (CJD) revealed a new mutation at codon 210 resulting in the substitution of isoleucine for valine...
  92. ncbi Increased susceptibility to Kuru of carriers of the PRNP 129 methionine/methionine genotype
    H S Lee
    Clinical Neurogenetics Unit, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD, USA
    J Infect Dis 183:192-196. 2001
    b>Kuru reached epidemic proportions by the mid-twentieth century among the Fore people of New Guinea and disappeared after the abolition of cannibalistic rituals...
  93. ncbi Genetic and infectious prion diseases
    S B Prusiner
    Department of Neurology, University of California, San Francisco
    Arch Neurol 50:1129-53. 1993
    Enriching fractions from Syrian hamster (SHa) brain for scrapie prion infectivity led to the discovery of the prion protein (PrP)...
  94. ncbi Fatal familial insomnia, a prion disease with a mutation at codon 178 of the prion protein gene
    R Medori
    Division of Neuropathology, Case Western Reserve University, Cleveland, OH 44106
    N Engl J Med 326:444-9. 1992
    ..Five other family members who died of this disease, which we termed "fatal familial insomnia," had broader neuropathologic changes suggesting that fatal familial insomnia could be a prion disease...
  95. ncbi Polymorphism at codon 129 or codon 219 of PRNP and clinical heterogeneity in a previously unreported family with Gerstmann-Sträussler-Scheinker disease (PrP-P102L mutation)
    P Barbanti
    Department of Neurological Sciences, Universita di Roma La Sapienza, Italy
    Neurology 47:734-41. 1996
    ..syndrome (GSS) associated with the Pro to Leu point mutation at codon 102 of the prion protein gene (PRNP)...
  96. ncbi Diagnosis of Gerstmann-Sträussler syndrome in familial dementia with prion protein gene analysis
    J Collinge
    Division of Psychiatry, Clinical Research Centre, Harrow, Middlesex, UK
    Lancet 2:15-7. 1989
    ..samples from 12 unrelated individuals with various familial dementias and ataxias for mutation in part of the prion protein (PrP) gene, an abnormality that occurs in individuals with the spongiform encephalopathies, Gerstmann-Strä..
  97. ncbi Mutation in codon 200 and polymorphism in codon 129 of the prion protein gene in Libyan Jews with Creutzfeldt-Jakob disease
    R Gabizon
    Department of Neurology, Hadassah University Hospital, Jerusalem, Israel
    Philos Trans R Soc Lond B Biol Sci 343:385-90. 1994
    Various mutations in the prion protein (PrP) gene are associated with Creutzfeldt-Jakob disease (CJD), a transmissible fatal neurodegenerative disorder...
  98. ncbi Phenotypic variability of Gerstmann-Sträussler-Scheinker disease is associated with prion protein heterogeneity
    P Piccardo
    Indiana University School of Medicine, Indianapolis 46202 5120, USA
    J Neuropathol Exp Neurol 57:979-88. 1998
    ..disease (GSS), a cerebello-pyramidal syndrome associated with dementia and caused by mutations in the prion protein gene (PRNP), is phenotypically heterogeneous...
  99. ncbi Complete genomic sequence and analysis of the prion protein gene region from three mammalian species
    I Y Lee
    Department of Molecular Biotechnology, University of Washington, Seattle, Washington 98195 7730 USA
    Genome Res 8:1022-37. 1998
    The prion protein (PrP), first identified in scrapie-infected rodents, is encoded by a single exon of a single-copy chromosomal gene. In addition to the protein-coding exon, PrP genes in mammals contain one or two 5'-noncoding exons...
  100. ncbi Fatal familial insomnia and familial Creutzfeldt-Jakob disease: disease phenotype determined by a DNA polymorphism
    L G Goldfarb
    Laboratory of Central Nervous System Studies, National Institute of Neurological Diseases and Stroke, National Institutes of Health, Bethesda, Maryland 20892
    Science 258:806-8. 1992
    ..clinically and pathologically distinct diseases, are linked to the same mutation at codon 178 (Asn178) of the prion protein gene...

Research Grants91

  1. Characterization of de novo generation of infectious prions
    Joaquin Castilla; Fiscal Year: 2009
    ..appears to be composed uniquely of a protein which is a conformationally-modified version (PrPSc) of a natural prion protein (PrPC)...
  2. Development of a Biochemical Diagnosis for Creutzfeldt-Jakob disease
    Claudio Soto; Fiscal Year: 2013
    ..neurodegenerative diseases, including sporadic Creutzfeldt-Jakob disease (sCJD), the most common form, and variant CJD (vCJD) which is associated to consumption of cattle meat infected by bovine spongiform encephalopathy...
  3. CNS DEGENERATION CAUSED BY NOVEL PATHOGENS
    STANLEY PRUSINER; Fiscal Year: 1992
    ..Infectious prion particles are composed largely, if not entirely, of an abnormal isoform of the prion protein (PrP) which is encoded by a chromosomal gene...
  4. TRANSMISSIBLE AND GENETIC NEURODENGENERATIVE DISEASES
    STANLEY PRUSINER; Fiscal Year: 1999
    ..Convergence of many lines of experimental evidence asserts that the scrapie isoform of the prion protein (PrP/Sc) is a necessary component of the infectious scrapie prion yet both PrP/Sc and the normal isoform, PrP/..
  5. Functional Genetics of Susceptibility to Prions
    George Carlson; Fiscal Year: 2001
    ..by applicant) Prion diseases are neurodegenerative disorders of humans and animals that involve misfolding of prion protein (PrP)...
  6. Functional Genetics of Susceptibility to Prions
    George Carlson; Fiscal Year: 2005
    ..by applicant) Prion diseases are neurodegenerative disorders of humans and animals that involve misfolding of prion protein (PrP)...
  7. Functional Genetics of Susceptibility to Prions
    George Carlson; Fiscal Year: 2002
    ..by applicant) Prion diseases are neurodegenerative disorders of humans and animals that involve misfolding of prion protein (PrP)...
  8. Functional Genetics of Susceptibility to Prions
    George Carlson; Fiscal Year: 2004
    ..by applicant) Prion diseases are neurodegenerative disorders of humans and animals that involve misfolding of prion protein (PrP)...
  9. Functional Genetics of Susceptibility to Prions
    George Carlson; Fiscal Year: 2003
    ..by applicant) Prion diseases are neurodegenerative disorders of humans and animals that involve misfolding of prion protein (PrP)...
  10. LEADERSHIP AND EXCELLENCE IN ALZHEIMER'S DISEASE
    STANLEY PRUSINER; Fiscal Year: 1990
    ..syndrome (GSS) in humans - is genetically linked to mutations in the open reading frame (ORF) of the prion protein (PrP) gene...
  11. NOVEL THERAPEUTICS FOR PRION DISEASES
    STANLEY PRUSINER; Fiscal Year: 2007
    ..Treatment of patients with sporadic (s) Creutzfeldt-Jakob disease (CJD), the most common human prion disease, employing quinacrine will be studied...
  12. NOVEL THERAPEUTICS FOR PRION DISEASES
    STANLEY PRUSINER; Fiscal Year: 2006
    ..Treatment of patients with sporadic (s) Creutzfeldt-Jakob disease (CJD), the most common human prion disease, employing quinacrine will be studied...
  13. NOVEL THERAPEUTICS FOR PRION DISEASES
    STANLEY PRUSINER; Fiscal Year: 2005
    ..Treatment of patients with sporadic (s) Creutzfeldt-Jakob disease (CJD), the most common human prion disease, employing quinacrine will be studied...
  14. NOVEL THERAPEUTICS FOR PRION DISEASES
    STANLEY PRUSINER; Fiscal Year: 2003
    ..Treatment of patients with sporadic (s) Creutzfeldt-Jakob disease (CJD), the most common human prion disease, employing quinacrine will be studied...
  15. NOVEL THERAPEUTICS FOR PRION DISEASES
    STANLEY PRUSINER; Fiscal Year: 2009
    ..The accumulation of an alternatively folded isoform of the prion protein (PrPSc) in the brains of humans and animals is the hallmark of the prion disorders that include Creutzfeldt-..
  16. MURINE TRANSGENIC MODELS OF PRION DISEASES
    David A Harris; Fiscal Year: 2011
    ..we investigated how three, distinct genetic mechanisms (loss, gain, and subversion of the normal function of PrPC) contribute to neurodegeneration in two different transgenic models: Tg(PG14) mice, which express an aggregation-..
  17. Investigation of the Establishment of the Prion State
    Irina Derkatch; Fiscal Year: 2009
    Prions cause tremendous concern as the causative agents of lethal neurodegenerative disorders such as CJD and BSE...
  18. Investigation of the Establishment of the Prion State
    Irina Derkatch; Fiscal Year: 2007
    Prions cause tremendous concern as the causative agents of lethal neurodegenerative disorders such as CJD and BSE...
  19. Investigation of the Establishment of the Prion State
    Irina Derkatch; Fiscal Year: 2006
    Prions cause tremendous concern as the causative agents of lethal neurodegenerative disorders such as CJD and BSE...
  20. Investigation of the Establishment of the Prion State
    Irina L Derkatch; Fiscal Year: 2010
    Prions cause tremendous concern as the causative agents of lethal neurodegenerative disorders such as CJD and BSE...
  21. NOVEL THERAPEUTICS FOR PRION DISEASES
    Stanley B Prusiner; Fiscal Year: 2010
    ..The accumulation of an alternatively folded isoform of the prion protein (PrPSc) in the brains of humans and animals is the hallmark of the prion disorders that include Creutzfeldt-..
  22. NOVEL THERAPEUTICS FOR PRION DISEASES
    Stanley B Prusiner; Fiscal Year: 2013
    ..The accumulation of an alternatively folded isoform of the prion protein (PrPSc) in the brains of humans and animals is the hallmark of the prion disorders that include Creutzfeldt-..
  23. INVESTIGATIONS OF HUMAN PRION DISEASE
    STANLEY PRUSINER; Fiscal Year: 2009
    ..These Tg mice are susceptible to prions from patients who died of sporadic (s) Creutzfeldt- Jakob disease (CJD), in just 80 days...
  24. DEGENERATIVE AND DEMENTING DISEASES OF AGING
    STANLEY PRUSINER; Fiscal Year: 2009
    ..The accumulation of an alternatively folded isoform of the prion protein (PrPSc) in the brains of humans and animals is the hallmark of the prion disorders that include Creutzfeldt-..
  25. MURINE TRANSGENIC MODELS OF PRION DISEASES
    David A Harris; Fiscal Year: 2013
    ..we investigated how three, distinct genetic mechanisms (loss, gain, and subversion of the normal function of PrPC) contribute to neurodegeneration in two different transgenic models: Tg(PG14) mice, which express an aggregation-..
  26. Structure and Mechanism of a Prion-remodeling Factor
    Francis T F Tsai; Fiscal Year: 2013
    ....
  27. Emergence of drug resistance in prion populations
    Charles Weissmann; Fiscal Year: 2010
    ..they consist mainly, if not entirely of multimers of PrPSc, a conformational isomer of the host- encoded protein PrPC. Prions occur in the form of diverse strains, which differ in various phenotypic properties but whose PrPSc has the ..
  28. Absorption, Metabolism and Biodistribution of Prions after Oral Ingestion
    Claudio Soto; Fiscal Year: 2013
    ..The central pathogenic event is the conversion of the host-encoded prion protein (PrPC) into a misfolded isoform (PrPSc)...
  29. MOLECULAR PATHOGENESIS OF AGE-DEPENDENT CNS DEGENERATION
    STANLEY PRUSINER; Fiscal Year: 1999
    ..Investigations directed toward elucidating the role of molecular chaperones in the conversion of the cellular prion protein (PrPC) into the scrapie isoform (PrPSc) are proposed (Project 1)...
  30. Pathogenesis, Transmission and Detection of Zoonotic Prion Diseases (P01)
    Claudio Soto; Fiscal Year: 2009
    ..In specific aim 4 we plan to evaluate the differential susceptibility of PrPc from various species (human, sheep, cattle and deer) to be converted in vitro by PrPsc derived from various forms ..
  31. CNS DEGENERATION CAUSED BY NOVEL PATHOGENS
    STANLEY PRUSINER; Fiscal Year: 1999
    ..Investigation directed toward elucidating the molecular mechanism of conversion of the cellular prion protein) (PrP/c) into the scrapie isoform (PrP/sc) are proposed...
  32. CNS DEGENERATION CAUSED BY NOVEL PATHOGENS
    STANLEY PRUSINER; Fiscal Year: 2002
    ..Investigation directed toward elucidating the molecular mechanism of conversion of the cellular prion protein) (PrP/c) into the scrapie isoform (PrP/sc) are proposed...
  33. CNS DEGENERATION CAUSED BY NOVEL PATHOGENS
    STANLEY PRUSINER; Fiscal Year: 2000
    ..Investigation directed toward elucidating the molecular mechanism of conversion of the cellular prion protein) (PrP/c) into the scrapie isoform (PrP/sc) are proposed...
  34. CNS DEGENERATION CAUSED BY NOVEL PATHOGENS
    STANLEY PRUSINER; Fiscal Year: 2000
    ..Investigation directed toward elucidating the molecular mechanism of conversion of the cellular prion protein) (PrP/c) into the scrapie isoform (PrP/sc) are proposed...
  35. CNS DEGENERATION CAUSED BY NOVEL PATHOGENS
    STANLEY PRUSINER; Fiscal Year: 2000
    ..Investigation directed toward elucidating the molecular mechanism of conversion of the cellular prion protein) (PrP/c) into the scrapie isoform (PrP/sc) are proposed...
  36. CNS DEGENERATION CAUSED BY NOVEL PATHOGENS
    STANLEY PRUSINER; Fiscal Year: 2001
    ..Investigation directed toward elucidating the molecular mechanism of conversion of the cellular prion protein) (PrP/c) into the scrapie isoform (PrP/sc) are proposed...
  37. JACOB JAVISTS CENTER OF EXCELLENCE IN NEUROSCIENCE
    STANLEY PRUSINER; Fiscal Year: 1990
    ..Convergence of many lines of experimental evidence asserts that the scrapie isoform of the prion protein (PrPSc) is a necessary component of the infectious scrapie prion...
  38. ION CHANNEL MODULATION BY THE PRION PROTEIN: A NOVEL TOXIC MECHANISM
    David A Harris; Fiscal Year: 2013
    ..In contrast, how abnormal forms of the prion protein (PrP) kill nerve cells is still a mystery...
  39. Mechanisms of prion spread
    Christina Sigurdson; Fiscal Year: 2013
    ..by applicant): Prion diseases are fatal neurodegenerative disorders caused by an aggregated form of the prion protein, PrPSc...
  40. The Role of Competitive Forces in Prion Propagation and Appearance
    Tricia R Serio; Fiscal Year: 2013
    ..Through this unique perspective, we will begin to reveal the cellular pathways underlying transitions between prion-associated phenotypes, a crucial yet poorly understood aspect of prion biology. ..
  41. Cyclic Amplification of Prion Protein Misfolding
    Claudio Soto; Fiscal Year: 2010
    This application is a competing renewal of a very successful project entitled "Cyclic Amplification of Prion Protein Misfolding"...
  42. Cyclic Amplification of Prion Protein Misfolding
    Claudio Soto; Fiscal Year: 2013
    ..This application is a competing renewal of a very successful project entitled "Cyclic Amplification of Prion Protein Misfolding"...
  43. An improved expression vector to create transgenic mice for prion research.
    GULTEKIN TAMGUNEY; Fiscal Year: 2007
    ..These disorders are characterized by the conversion of the host-encoded cellular prion protein (PrPc) into an abnormally folded and infectious isoform (PrPSc) that accumulates in the brain and causes ..
  44. Structural basis of species-specific infectivities of two prion strains
    Peter M Tessier; Fiscal Year: 2010
    ..Unfortunately, it is currently not possible to form highly infectious prions from recombinant mammalian prion protein (PrP) for structural studies, but this is readily possible for yeast prions...
  45. Absorption, Metabolism and Biodistribution of Prions after Oral Ingestion
    Claudio Soto; Fiscal Year: 2012
    ..The central pathogenic event is the conversion of the host-encoded prion protein (PrPC) into a misfolded isoform (PrPSc)...
  46. Structural basis of species-specific infectivities of two prion strains
    PETER TESSIER; Fiscal Year: 2009
    ..Unfortunately, it is currently not possible to form highly infectious prions from recombinant mammalian prion protein (PrP) for structural studies, but this is readily possible for yeast prions...
  47. Genetics of Prion Susceptibility in vitro
    George A Carlson; Fiscal Year: 2010
    Prion diseases are neurodegenerative disorders of humans and animals caused by misfolding of prion protein (PrP)...
  48. Mechanisms of prion strain selection
    Jason C Bartz; Fiscal Year: 2010
    ..abstract_text> ..
  49. Mechanisms of prion strain selection
    Jason Bartz; Fiscal Year: 2009
    ..abstract_text> ..
  50. Mechanisms of prion strain selection
    Jason Bartz; Fiscal Year: 2009
    ..abstract_text> ..
  51. Mechanisms of prion strain selection
    Jason Bartz; Fiscal Year: 2009
    ..abstract_text> ..
  52. Mechanisms of prion strain selection
    Jason Bartz; Fiscal Year: 2006
    ....
  53. Mechanisms of prion strain selection
    Jason Bartz; Fiscal Year: 2007
    ....
  54. DEGENERATIVE AND DEMENTING DISEASES OF AGING
    STANLEY PRUSINER; Fiscal Year: 2004
    ..We propose a series of investigations on the structure of the pathogenic isoform of the prion protein (PrPSc) using cryo-electron crystallography, computational modeling, and small-angle X-ray fiber (Project 1)...
  55. DEGENERATIVE AND DEMENTING DISEASES OF AGING
    STANLEY PRUSINER; Fiscal Year: 2005
    ..We propose a series of investigations on the structure of the pathogenic isoform of the prion protein (PrPSc) using cryo-electron crystallography, computational modeling, and small-angle X-ray fiber (Project 1)...
  56. MOLECULAR DETERMINANTS OF HUMAN PRION DISEASES
    James Mastrianni; Fiscal Year: 2000
    ..The C-terminus of PrPc is thought to bind to protein X, a putative molecular chaperone involved in the conversion of PrPc into PrPSc...
  57. MOLECULAR DETERMINANTS OF HUMAN PRION DISEASES
    James Mastrianni; Fiscal Year: 1999
    ..The C-terminus of PrPc is thought to bind to protein X, a putative molecular chaperone involved in the conversion of PrPc into PrPSc...
  58. Animals
    STANLEY PRUSINER; Fiscal Year: 2009
    ..Prions seem to be composed solely of PrPSc molecules, which are derived from a precursor PrPc by a poorly understood process...
  59. DEGENERATIVE AND DEMENTING DISEASES OF AGING
    STANLEY PRUSINER; Fiscal Year: 2009
    ..Prions seem to be composed solely of PrPSc molecules, which are derived from a precursor PrPc by a poorly understood process...
  60. DEGENERATIVE AND DEMENTING DISEASES OF AGING
    Stanley B Prusiner; Fiscal Year: 2010
    ..Prions seem to be composed solely of PrPSc molecules, which are derived from a precursor PrPc by a poorly understood process...
  61. MURINE TRANSGENIC MODELS OF PRION DISEASES
    David Harris; Fiscal Year: 2007
    ..A major focus of our work has been on Tg mice that express a PrP molecule with a nine-octapeptide insertional mutation (PG14) associated with a familial form of Creutzfeldt- Jakob ..
  62. MURINE TRANSGENIC MODELS OF PRION DISEASES
    David A Harris; Fiscal Year: 2009
    ..A major focus of our work has been on Tg mice that express a PrP molecule with a nine-octapeptide insertional mutation (PG14) associated with a familial form of Creutzfeldt- Jakob ..
  63. MURINE TRANSGENIC MODELS OF PRION DISEASES
    David Harris; Fiscal Year: 2009
    ..A major focus of our work has been on Tg mice that express a PrP molecule with a nine-octapeptide insertional mutation (PG14) associated with a familial form of Creutzfeldt- Jakob ..
  64. MURINE TRANSGENIC MODELS OF PRION DISEASES
    David A Harris; Fiscal Year: 2010
    ..A major focus of our work has been on Tg mice that express a PrP molecule with a nine-octapeptide insertional mutation (PG14) associated with a familial form of Creutzfeldt- Jakob ..
  65. MURINE TRANSGENIC MODELS OF PRION DISEASES
    David Harris; Fiscal Year: 2006
    ..A major focus of our work has been on Tg mice that express a PrP molecule with a nine-octapeptide insertional mutation (PG14) associated with a familial form of Creutzfeldt- Jakob ..
  66. National Biomedical EPR Center
    James Hyde; Fiscal Year: 2003
    ..Low frequency L-band EPR of copper (II) in prion protein and in prion octapeptide repeats...
  67. Preventing Human Prion Diseases - Inactivation of Prions
    STANLEY PRUSINER; Fiscal Year: 2006
    ..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. ..
  68. 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. ..
  69. 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. ..
  70. 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. ..
  71. Phosphorylation of Prion Protein as a novel mechanism for conversion
    Andrea C LeBlanc; Fiscal Year: 2010
    ..long-term objective of this proposal is to determine if phosphorylation is involved in normal or pathological prion protein (PrP) biology...
  72. Phosphorylation of Prion Protein as a novel mechanism for conversion
    Andrea LeBlanc; Fiscal Year: 2009
    ..long-term objective of this proposal is to determine if phosphorylation is involved in normal or pathological prion protein (PrP) biology...
  73. MECHANISM OF CELL DEATH BY PRIONS
    Neena Singh; Fiscal Year: 2000
    ..is believed to result from a conformationally transformed, scrapie isoform (PrPSc) of the normal host prion protein (PrPC)...
  74. MECHANISM OF CELL DEATH BY PRIONS
    Neena Singh; Fiscal Year: 2004
    ..is believed to result from a conformationally transformed, scrapie isoform (PrPSc) of the normal host prion protein (PrPC)...
  75. MECHANISM OF CELL DEATH BY PRIONS
    Neena Singh; Fiscal Year: 2001
    ..is believed to result from a conformationally transformed, scrapie isoform (PrPSc) of the normal host prion protein (PrPC)...
  76. MECHANISM OF CELL DEATH BY PRIONS
    Neena Singh; Fiscal Year: 2002
    ..is believed to result from a conformationally transformed, scrapie isoform (PrPSc) of the normal host prion protein (PrPC)...
  77. MECHANISM OF CELL DEATH BY PRIONS
    Neena Singh; Fiscal Year: 2003
    ..is believed to result from a conformationally transformed, scrapie isoform (PrPSc) of the normal host prion protein (PrPC)...
  78. TRANSGENETIC STUDIES OF PRION DISEASE IN CERVIDS
    Glenn Telling; Fiscal Year: 2000
    ..The mechanism of prion replication is unique, the central event being the coercion of host encoded prion protein (PrPc) by the disease-associated isoform (PrPSc) to adopt to the infectivity-associated conformation...
  79. TRANSGENETIC STUDIES OF PRION DISEASE IN CERVIDS
    Glenn Telling; Fiscal Year: 2002
    ..The mechanism of prion replication is unique, the central event being the coercion of host encoded prion protein (PrPc) by the disease-associated isoform (PrPSc) to adopt to the infectivity-associated conformation...
  80. TRANSGENETIC STUDIES OF PRION DISEASE IN CERVIDS
    Glenn Telling; Fiscal Year: 2001
    ..The mechanism of prion replication is unique, the central event being the coercion of host encoded prion protein (PrPc) by the disease-associated isoform (PrPSc) to adopt to the infectivity-associated conformation...
  81. TRANSGENETIC STUDIES OF PRION DISEASE IN CERVIDS
    Glenn Telling; Fiscal Year: 2001
    ..The mechanism of prion replication is unique, the central event being the coercion of host encoded prion protein (PrPc) by the disease-associated isoform (PrPSc) to adopt to the infectivity-associated conformation...
  82. Investigation of the yeast prion factor [PSI+]
    Susan W Liebman; Fiscal Year: 2010
    ....
  83. NIH Director's Pioneer Award
    Junying Yuan; Fiscal Year: 2009
    ..No Abstract provided ..
  84. De NOVO GENERATION OF MAMMALIAL PRION STRAINS
    STANLEY PRUSINER; Fiscal Year: 2005
    ..Encouraged by investigations demonstrating the production of synthetic prions was possible by mutating the prion protein (PrP) gene, we chose to study a subset of recombinant (rec) PrPs folded into beta-rich structures...