Published in Proc Natl Acad Sci U S A on November 10, 1998
CJD (Creutzfeldt-Jakob Disease) Quinacrine Study | NCT00183092
Transmission and spreading of tauopathy in transgenic mouse brain. Nat Cell Biol (2009) 7.50
Cellular prion protein mediates impairment of synaptic plasticity by amyloid-beta oligomers. Nature (2009) 6.99
MAVS forms functional prion-like aggregates to activate and propagate antiviral innate immune response. Cell (2011) 5.91
The most infectious prion protein particles. Nature (2005) 5.37
Cells release prions in association with exosomes. Proc Natl Acad Sci U S A (2004) 5.17
NMR solution structure of the human prion protein. Proc Natl Acad Sci U S A (2000) 4.80
The protofilament structure of insulin amyloid fibrils. Proc Natl Acad Sci U S A (2002) 4.08
Ultrasensitive human prion detection in cerebrospinal fluid by real-time quaking-induced conversion. Nat Med (2011) 3.95
Structural insights into a yeast prion illuminate nucleation and strain diversity. Nature (2005) 3.86
Reiterated WG/GW motifs form functionally and evolutionarily conserved ARGONAUTE-binding platforms in RNAi-related components. Genes Dev (2007) 3.75
Prion-like mechanisms in neurodegenerative diseases. Nat Rev Neurosci (2009) 3.68
A test for Creutzfeldt-Jakob disease using nasal brushings. N Engl J Med (2014) 3.58
Self-propagation of pathogenic protein aggregates in neurodegenerative diseases. Nature (2013) 3.55
Environmental sources of prion transmission in mule deer. Emerg Infect Dis (2004) 3.44
Prion-like transmission of protein aggregates in neurodegenerative diseases. Nat Rev Mol Cell Biol (2010) 3.34
Identification of a second bovine amyloidotic spongiform encephalopathy: molecular similarities with sporadic Creutzfeldt-Jakob disease. Proc Natl Acad Sci U S A (2004) 3.29
An amyloid-forming peptide from the yeast prion Sup35 reveals a dehydrated beta-sheet structure for amyloid. Proc Natl Acad Sci U S A (2001) 3.26
Prion-like propagation of mutant superoxide dismutase-1 misfolding in neuronal cells. Proc Natl Acad Sci U S A (2011) 3.23
Quantifying prion disease penetrance using large population control cohorts. Sci Transl Med (2016) 3.04
The amyloid state and its association with protein misfolding diseases. Nat Rev Mol Cell Biol (2014) 2.99
Structural studies of the scrapie prion protein by electron crystallography. Proc Natl Acad Sci U S A (2002) 2.91
[URE3] prion propagation in Saccharomyces cerevisiae: requirement for chaperone Hsp104 and curing by overexpressed chaperone Ydj1p. Mol Cell Biol (2000) 2.83
Distinct tau prion strains propagate in cells and mice and define different tauopathies. Neuron (2014) 2.83
Prion-like polymerization underlies signal transduction in antiviral immune defense and inflammasome activation. Cell (2014) 2.71
Anti-aβ therapeutics in Alzheimer's disease: the need for a paradigm shift. Neuron (2011) 2.67
A natively unfolded yeast prion monomer adopts an ensemble of collapsed and rapidly fluctuating structures. Proc Natl Acad Sci U S A (2007) 2.62
The behaviour of polyamino acids reveals an inverse side chain effect in amyloid structure formation. EMBO J (2002) 2.59
Prion recognition elements govern nucleation, strain specificity and species barriers. Nature (2007) 2.57
Mammalian prions generated from bacterially expressed prion protein in the absence of any mammalian cofactors. J Biol Chem (2010) 2.57
NMR structure of the bovine prion protein. Proc Natl Acad Sci U S A (2000) 2.56
Molecular architecture of human prion protein amyloid: a parallel, in-register beta-structure. Proc Natl Acad Sci U S A (2007) 2.55
Newly identified prion linked to the chromatin-remodeling factor Swi1 in Saccharomyces cerevisiae. Nat Genet (2008) 2.43
Design and construction of diverse mammalian prion strains. Proc Natl Acad Sci U S A (2009) 2.41
Solid-state NMR studies of amyloid fibril structure. Annu Rev Phys Chem (2011) 2.38
Amyloid aggregates of the HET-s prion protein are infectious. Proc Natl Acad Sci U S A (2002) 2.37
Mechanisms of cellular uptake of cell-penetrating peptides. J Biophys (2011) 2.34
Prion detection by an amyloid seeding assay. Proc Natl Acad Sci U S A (2007) 2.34
Caspase-12 and endoplasmic reticulum stress mediate neurotoxicity of pathological prion protein. EMBO J (2003) 2.34
Prions adhere to soil minerals and remain infectious. PLoS Pathog (2006) 2.32
Molecular genetics of heterokaryon incompatibility in filamentous ascomycetes. Microbiol Mol Biol Rev (2000) 2.30
Prion-like disorders: blurring the divide between transmissibility and infectivity. J Cell Sci (2010) 2.29
The prion hypothesis: from biological anomaly to basic regulatory mechanism. Nat Rev Mol Cell Biol (2010) 2.22
Differently anchored influenza hemagglutinin mutants display distinct interaction dynamics with mutual rafts. J Cell Biol (2003) 2.22
Cell-free propagation of prion strains. EMBO J (2008) 2.20
Insufficiently dehydrated hydrogen bonds as determinants of protein interactions. Proc Natl Acad Sci U S A (2002) 2.20
Dissection and design of yeast prions. PLoS Biol (2004) 2.19
Location and properties of metal-binding sites on the human prion protein. Proc Natl Acad Sci U S A (2001) 2.16
Conversion of raft associated prion protein to the protease-resistant state requires insertion of PrP-res (PrP(Sc)) into contiguous membranes. EMBO J (2002) 2.16
Molecular features of the copper binding sites in the octarepeat domain of the prion protein. Biochemistry (2002) 2.16
Protease-sensitive synthetic prions. PLoS Pathog (2010) 2.14
Beta-sheet core of human prion protein amyloid fibrils as determined by hydrogen/deuterium exchange. Proc Natl Acad Sci U S A (2007) 2.14
From conversion to aggregation: protofibril formation of the prion protein. Proc Natl Acad Sci U S A (2004) 2.14
Diagnosis of human prion disease. Proc Natl Acad Sci U S A (2005) 2.13
Neonatal lethality in transgenic mice expressing prion protein with a deletion of residues 105-125. EMBO J (2007) 2.12
Crossing the species barrier by PrP(Sc) replication in vitro generates unique infectious prions. Cell (2008) 2.09
New inhibitors of scrapie-associated prion protein formation in a library of 2000 drugs and natural products. J Virol (2003) 2.09
The genetic epidemiology of neurodegenerative disease. J Clin Invest (2005) 2.08
Mutant PrPSc conformers induced by a synthetic peptide and several prion strains. J Virol (2004) 2.07
The structural basis for biphasic kinetics in the folding of the WW domain from a formin-binding protein: lessons for protein design? Proc Natl Acad Sci U S A (2003) 2.05
Transmission of elk and deer prions to transgenic mice. J Virol (2006) 2.03
Chaperones in control of protein disaggregation. EMBO J (2008) 2.01
Prion protein NMR structures of elk and of mouse/elk hybrids. Proc Natl Acad Sci U S A (2005) 2.01
Monomer adds to preformed structured oligomers of Abeta-peptides by a two-stage dock-lock mechanism. Proc Natl Acad Sci U S A (2006) 2.01
Abbreviated incubation times for human prions in mice expressing a chimeric mouse-human prion protein transgene. Proc Natl Acad Sci U S A (2003) 2.01
Prion protein recruits its neuronal receptor NCAM to lipid rafts to activate p59fyn and to enhance neurite outgrowth. J Cell Biol (2005) 2.00
The power of automated high-resolution behavior analysis revealed by its application to mouse models of Huntington's and prion diseases. Proc Natl Acad Sci U S A (2007) 1.99
Conversion of the BASE prion strain into the BSE strain: the origin of BSE? PLoS Pathog (2007) 1.97
Treatment of transmissible spongiform encephalopathy by intraventricular drug infusion in animal models. J Virol (2004) 1.97
A miRNA signature of prion induced neurodegeneration. PLoS One (2008) 1.96
The delicate balance between secreted protein folding and endoplasmic reticulum-associated degradation in human physiology. Physiol Rev (2012) 1.95
Molecular mechanisms for protein-encoded inheritance. Nat Struct Mol Biol (2009) 1.94
Calpain-mediated signaling mechanisms in neuronal injury and neurodegeneration. Mol Neurobiol (2008) 1.93
Prion protein is expressed on long-term repopulating hematopoietic stem cells and is important for their self-renewal. Proc Natl Acad Sci U S A (2006) 1.92
Structural organization of brain-derived mammalian prions examined by hydrogen-deuterium exchange. Nat Struct Mol Biol (2011) 1.90
Interaction between human prion protein and amyloid-beta (Abeta) oligomers: role OF N-terminal residues. J Biol Chem (2010) 1.88
Markedly increased susceptibility to natural sheep scrapie of transgenic mice expressing ovine prp. J Virol (2001) 1.86
Molecular conformation and dynamics of the Y145Stop variant of human prion protein in amyloid fibrils. Proc Natl Acad Sci U S A (2008) 1.86
Detection of infectious prions in urine. FEBS Lett (2008) 1.85
De novo design of helical bundles as models for understanding protein folding and function. Acc Chem Res (2000) 1.84
De novo generation of infectious prions in vitro produces a new disease phenotype. PLoS Pathog (2009) 1.83
Protein metamorphosis: the two-state behavior of Mad2. Structure (2008) 1.83
Domain organization and structure-function relationship of the HET-s prion protein of Podospora anserina. EMBO J (2003) 1.80
Prediction of aggregation rate and aggregation-prone segments in polypeptide sequences. Protein Sci (2005) 1.80
The octarepeat domain of the prion protein binds Cu(II) with three distinct coordination modes at pH 7.4. J Am Chem Soc (2005) 1.80
Detection and characterization of proteinase K-sensitive disease-related prion protein with thermolysin. Biochem J (2008) 1.77
Exploring protein aggregation and self-propagation using lattice models: phase diagram and kinetics. Protein Sci (2002) 1.77
Protein homeostasis and aging in neurodegeneration. J Cell Biol (2010) 1.76
Prion protein (PrPc) positively regulates neural precursor proliferation during developmental and adult mammalian neurogenesis. Proc Natl Acad Sci U S A (2006) 1.76
Wild-type PrP and a mutant associated with prion disease are subject to retrograde transport and proteasome degradation. Proc Natl Acad Sci U S A (2001) 1.74
Prions in the urine of patients with variant Creutzfeldt-Jakob disease. N Engl J Med (2014) 1.74
The aggregation kinetics of Alzheimer's beta-amyloid peptide is controlled by stochastic nucleation. Protein Sci (2005) 1.74
"Prion-proof" for [PIN+]: infection with in vitro-made amyloid aggregates of Rnq1p-(132-405) induces [PIN+]. J Mol Biol (2006) 1.74
Elimination of prions by branched polyamines and implications for therapeutics. Proc Natl Acad Sci U S A (1999) 1.73
Interactions between heterologous forms of prion protein: binding, inhibition of conversion, and species barriers. Proc Natl Acad Sci U S A (2000) 1.73
Scrapie Agent (Strain 263K) can transmit disease via the oral route after persistence in soil over years. PLoS One (2007) 1.72
Near infrared dyes as lifetime solvatochromic probes for micropolarity measurements of biological systems. Biophys J (2007) 1.71
Aerosols transmit prions to immunocompetent and immunodeficient mice. PLoS Pathog (2011) 1.71
Strains of [PSI(+)] are distinguished by their efficiencies of prion-mediated conformational conversion. EMBO J (2001) 1.70
Proteasomes and ubiquitin are involved in the turnover of the wild-type prion protein. EMBO J (2001) 1.68
Novel proteinaceous infectious particles cause scrapie. Science (1982) 24.09
STUDIES ON THE CHEMICAL NATURE OF THE SUBSTANCE INDUCING TRANSFORMATION OF PNEUMOCOCCAL TYPES : INDUCTION OF TRANSFORMATION BY A DESOXYRIBONUCLEIC ACID FRACTION ISOLATED FROM PNEUMOCOCCUS TYPE III. J Exp Med (1944) 24.08
Principles that govern the folding of protein chains. Science (1973) 23.40
Independent functions of viral protein and nucleic acid in growth of bacteriophage. J Gen Physiol (1952) 22.87
A new variant of Creutzfeldt-Jakob disease in the UK. Lancet (1996) 20.15
Genetical implications of the structure of deoxyribonucleic acid. Nature (1953) 16.01
Transmissions to mice indicate that 'new variant' CJD is caused by the BSE agent. Nature (1997) 15.73
Infectivity of ribonucleic acid from tobacco mosaic virus. Nature (1956) 14.78
Molecular analysis of prion strain variation and the aetiology of 'new variant' CJD. Nature (1996) 14.40
The same prion strain causes vCJD and BSE. Nature (1997) 12.49
Mice devoid of PrP are resistant to scrapie. Cell (1993) 11.46
[URE3] as an altered URE2 protein: evidence for a prion analog in Saccharomyces cerevisiae. Science (1994) 10.94
Molecular biology of prion diseases. Science (1991) 10.83
Conversion of alpha-helices into beta-sheets features in the formation of the scrapie prion proteins. Proc Natl Acad Sci U S A (1993) 10.36
Normal development and behaviour of mice lacking the neuronal cell-surface PrP protein. Nature (1992) 9.01
Eight prion strains have PrP(Sc) molecules with different conformations. Nat Med (1998) 9.01
A cellular gene encodes scrapie PrP 27-30 protein. Cell (1985) 8.86
Role of the chaperone protein Hsp104 in propagation of the yeast prion-like factor [psi+]. Science (1995) 8.66
Transcriptional selectivity of viral genes in mammalian cells. Cell (1986) 7.95
Scrapie prions aggregate to form amyloid-like birefringent rods. Cell (1983) 7.92
Support for the prion hypothesis for inheritance of a phenotypic trait in yeast. Science (1996) 7.24
Mouse polyclonal and monoclonal antibody to scrapie-associated fibril proteins. J Virol (1987) 7.05
Identification of a protein that purifies with the scrapie prion. Science (1982) 7.00
The Determination of Hereditary Antigenic Differences in Genically Identical Paramecium Cells. Proc Natl Acad Sci U S A (1948) 6.80
A protease-resistant protein is a structural component of the scrapie prion. Cell (1983) 6.72
Evidence for the conformation of the pathologic isoform of the prion protein enciphering and propagating prion diversity. Science (1996) 6.71
Prion protein (PrP) with amino-proximal deletions restoring susceptibility of PrP knockout mice to scrapie. EMBO J (1996) 6.66
Unconventional viruses and the origin and disappearance of kuru. Science (1977) 6.38
NMR structure of the mouse prion protein domain PrP(121-231). Nature (1996) 6.37
Transgenetic studies implicate interactions between homologous PrP isoforms in scrapie prion replication. Cell (1990) 6.17
Transmission dynamics and epidemiology of BSE in British cattle. Nature (1996) 6.15
Self-replication and scrapie. Nature (1967) 6.01
Purification and structural studies of a major scrapie prion protein. Cell (1984) 5.98
The Wilson disease gene is a putative copper transporting P-type ATPase similar to the Menkes gene. Nat Genet (1993) 5.97
Creutzfeldt-Jakob disease (spongiform encephalopathy): transmission to the chimpanzee. Science (1968) 5.76
Creutzfeldt-Jakob disease: patterns of worldwide occurrence and the significance of familial and sporadic clustering. Ann Neurol (1979) 5.53
Secondary structure analysis of the scrapie-associated protein PrP 27-30 in water by infrared spectroscopy. Biochemistry (1991) 5.53
Abnormal fibrils from scrapie-infected brain. Acta Neuropathol (1981) 5.35
RECONSTITUTION OF ACTIVE TOBACCO MOSAIC VIRUS FROM ITS INACTIVE PROTEIN AND NUCLEIC ACID COMPONENTS. Proc Natl Acad Sci U S A (1955) 5.25
Self-seeded fibers formed by Sup35, the protein determinant of [PSI+], a heritable prion-like factor of S. cerevisiae. Cell (1997) 5.24
Genesis and variability of [PSI] prion factors in Saccharomyces cerevisiae. Genetics (1996) 5.19
Prion propagation in mice expressing human and chimeric PrP transgenes implicates the interaction of cellular PrP with another protein. Cell (1995) 5.16
Distinct PrP properties suggest the molecular basis of strain variation in transmissible mink encephalopathy. J Virol (1994) 5.12
Scrapie prion protein contains a phosphatidylinositol glycolipid. Cell (1987) 5.12
Isolation of a candidate gene for Menkes disease and evidence that it encodes a copper-transporting ATPase. Nat Genet (1993) 4.93
Scrapie and cellular PrP isoforms are encoded by the same chromosomal gene. Cell (1986) 4.82
Transgenic mice expressing hamster prion protein produce species-specific scrapie infectivity and amyloid plaques. Cell (1989) 4.73
Linkage of a prion protein missense variant to Gerstmann-Sträussler syndrome. Nature (1989) 4.69
A novel progressive spongiform encephalopathy in cattle. Vet Rec (1987) 4.39
Separation and properties of cellular and scrapie prion proteins. Proc Natl Acad Sci U S A (1986) 4.38
Experimental transmission of a Kuru-like syndrome to chimpanzees. Nature (1966) 4.29
The scrapie-associated form of PrP is made from a cell surface precursor that is both protease- and phospholipase-sensitive. J Biol Chem (1991) 4.23
Non-genetic propagation of strain-specific properties of scrapie prion protein. Nature (1995) 4.22
Spontaneous neurodegeneration in transgenic mice with mutant prion protein. Science (1990) 4.19
Does the agent of scrapie replicate without nucleic acid? Nature (1967) 4.14
Molecular basis of phenotypic variability in sporadic Creutzfeldt-Jakob disease. Ann Neurol (1996) 4.10
Structure of the recombinant full-length hamster prion protein PrP(29-231): the N terminus is highly flexible. Proc Natl Acad Sci U S A (1997) 4.10
Isolation of cDNAs of scrapie-modulated RNAs by subtractive hybridization of a cDNA library. Proc Natl Acad Sci U S A (1988) 4.04
Prion (PrPSc)-specific epitope defined by a monoclonal antibody. Nature (1997) 4.03
Further purification and characterization of scrapie prions. Biochemistry (1982) 4.01
Bovine spongiform encephalopathy: epidemiological studies on the origin. Vet Rec (1991) 3.99
The cellular prion protein binds copper in vivo. Nature (1998) 3.92
NMR characterization of the full-length recombinant murine prion protein, mPrP(23-231). FEBS Lett (1997) 3.90
The protein product of the het-s heterokaryon incompatibility gene of the fungus Podospora anserina behaves as a prion analog. Proc Natl Acad Sci U S A (1997) 3.85
Evidence for protein X binding to a discontinuous epitope on the cellular prion protein during scrapie prion propagation. Proc Natl Acad Sci U S A (1997) 3.78
Prion diseases and the BSE crisis. Science (1997) 3.77
A transmembrane form of the prion protein in neurodegenerative disease. Science (1998) 3.76
"Friendly fire" in medicine: hormones, homografts, and Creutzfeldt-Jakob disease. Lancet (1992) 3.74
Linkage of prion protein and scrapie incubation time genes. Cell (1986) 3.69
Measurement of the scrapie agent using an incubation time interval assay. Ann Neurol (1982) 3.68
Transmission of the BSE agent to mice in the absence of detectable abnormal prion protein. Science (1997) 3.68
Regional mapping of prion proteins in brain. Proc Natl Acad Sci U S A (1992) 3.68
Cholesterol depletion and modification of COOH-terminal targeting sequence of the prion protein inhibit formation of the scrapie isoform. J Cell Biol (1995) 3.66
Isolation of a candidate gene for Menkes disease that encodes a potential heavy metal binding protein. Nat Genet (1993) 3.63
129/Ola mice carrying a null mutation in PrP that abolishes mRNA production are developmentally normal. Mol Neurobiol (1995) 3.60
The sequential development of the brain lesion of scrapie in three strains of mice. J Comp Pathol (1968) 3.60
Expression of amino-terminally truncated PrP in the mouse leading to ataxia and specific cerebellar lesions. Cell (1998) 3.59
CYTOPLASMIC INHERITANCE OF THE ORGANIZATION OF THE CELL CORTEX IN PARAMECIUM AURELIA. Proc Natl Acad Sci U S A (1965) 3.58
Ablation of the prion protein (PrP) gene in mice prevents scrapie and facilitates production of anti-PrP antibodies. Proc Natl Acad Sci U S A (1993) 3.56
Structural studies of the scrapie prion protein using mass spectrometry and amino acid sequencing. Biochemistry (1993) 3.54
The disease characteristics of different strains of scrapie in Sinc congenic mouse lines: implications for the nature of the agent and host control of pathogenesis. J Gen Virol (1991) 3.47
Scrapie and cellular prion proteins differ in their kinetics of synthesis and topology in cultured cells. J Cell Biol (1990) 3.46
Complete genomic sequence and analysis of the prion protein gene region from three mammalian species. Genome Res (1998) 3.44
Structural clues to prion replication. Science (1994) 3.43
Solution structure of a 142-residue recombinant prion protein corresponding to the infectious fragment of the scrapie isoform. Proc Natl Acad Sci U S A (1997) 3.42
Fatal familial insomnia, a prion disease with a mutation at codon 178 of the prion protein gene. N Engl J Med (1992) 3.41
Identification of scrapie prion protein-specific mRNA in scrapie-infected and uninfected brain. Nature (1985) 3.38
Pathogenesis of scrapie virus infection in the mouse. J Infect Dis (1967) 3.34
Identification of a gene which controls the incubation period of some strains of scrapie agent in mice. J Comp Pathol (1968) 3.31
Distinct prion proteins in short and long scrapie incubation period mice. Cell (1987) 3.21
Prion-inducing domain 2-114 of yeast Sup35 protein transforms in vitro into amyloid-like filaments. Proc Natl Acad Sci U S A (1997) 3.21
Scrapie infectivity, fibrils and low molecular weight protein. Nature (1984) 3.18
Antibodies to a scrapie prion protein. Nature (1984) 3.09
Unaltered susceptibility to BSE in transgenic mice expressing human prion protein. Nature (1996) 3.09
In vitro propagation of the prion-like state of yeast Sup35 protein. Science (1997) 3.05
Predicting the CJD epidemic in humans. Nature (1997) 3.04
Identification of prion amyloid filaments in scrapie-infected brain. Cell (1985) 3.04
Mutations in familial Creutzfeldt-Jakob disease and Gerstmann-Sträussler-Scheinker's syndrome. Exp Neurol (1989) 3.00
A conformational transition at the N terminus of the prion protein features in formation of the scrapie isoform. J Mol Biol (1997) 2.95