Published in Cell on October 06, 1995
Prions. Proc Natl Acad Sci U S A (1998) 27.80
Formation of native prions from minimal components in vitro. Proc Natl Acad Sci U S A (2007) 5.94
NMR solution structure of the human prion protein. Proc Natl Acad Sci U S A (2000) 4.80
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
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
Compelling transgenetic evidence for transmission of bovine spongiform encephalopathy prions to humans. Proc Natl Acad Sci U S A (1999) 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
Prions. Cold Spring Harb Perspect Biol (2011) 3.17
Subcellular colocalization of the cellular and scrapie prion proteins in caveolae-like membranous domains. Proc Natl Acad Sci U S A (1996) 2.79
Creutzfeldt-Jakob disease and bovine spongiform encephalopathy: any connection? BMJ (1995) 2.74
Polyglutamine aggregates alter protein folding homeostasis in Caenorhabditis elegans. Proc Natl Acad Sci U S A (2000) 2.63
Strain-specified relative conformational stability of the scrapie prion protein. Protein Sci (2001) 2.60
NMR structure of the bovine prion protein. Proc Natl Acad Sci U S A (2000) 2.56
Design and construction of diverse mammalian prion strains. Proc Natl Acad Sci U S A (2009) 2.41
Transmission of prions from mule deer and elk with chronic wasting disease to transgenic mice expressing cervid PrP. J Virol (2004) 2.34
Successful transmission of three mouse-adapted scrapie strains to murine neuroblastoma cell lines overexpressing wild-type mouse prion protein. J Virol (2000) 2.18
The 37-kDa/67-kDa laminin receptor acts as the cell-surface receptor for the cellular prion protein. EMBO J (2001) 2.16
Diagnosis of human prion disease. Proc Natl Acad Sci U S A (2005) 2.13
Recombinant scrapie-like prion protein of 106 amino acids is soluble. Proc Natl Acad Sci U S A (1996) 2.12
Evidence for α-synuclein prions causing multiple system atrophy in humans with parkinsonism. Proc Natl Acad Sci U S A (2015) 2.08
Mutant PrPSc conformers induced by a synthetic peptide and several prion strains. J Virol (2004) 2.07
Transmission of elk and deer prions to transgenic mice. J Virol (2006) 2.03
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
Sulfated glycans and elevated temperature stimulate PrP(Sc)-dependent cell-free formation of protease-resistant prion protein. EMBO J (2001) 1.95
COOH-terminal sequence of the cellular prion protein directs subcellular trafficking and controls conversion into the scrapie isoform. Proc Natl Acad Sci U S A (1997) 1.89
Accelerated high fidelity prion amplification within and across prion species barriers. PLoS Pathog (2008) 1.89
Identification of a prion protein epitope modulating transmission of bovine spongiform encephalopathy prions to transgenic mice. Proc Natl Acad Sci U S A (1997) 1.87
Markedly increased susceptibility to natural sheep scrapie of transgenic mice expressing ovine prp. J Virol (2001) 1.86
Prion protein NMR structure and species barrier for prion diseases. Proc Natl Acad Sci U S A (1997) 1.86
Immunodetection of disease-associated mutant PrP, which accelerates disease in GSS transgenic mice. EMBO J (2005) 1.83
Chemical chaperones interfere with the formation of scrapie prion protein. EMBO J (1996) 1.81
Ectopic expression of prion protein (PrP) in T lymphocytes or hepatocytes of PrP knockout mice is insufficient to sustain prion replication. Proc Natl Acad Sci U S A (1999) 1.81
Chaperone-supervised conversion of prion protein to its protease-resistant form. Proc Natl Acad Sci U S A (1997) 1.79
Chronic wasting disease prions in elk antler velvet. Emerg Infect Dis (2009) 1.77
Selective incorporation of polyanionic molecules into hamster prions. J Biol Chem (2007) 1.71
Efficient transmission and characterization of Creutzfeldt-Jakob disease strains in bank voles. PLoS Pathog (2006) 1.70
Species-dependent differences in cofactor utilization for formation of the protease-resistant prion protein in vitro. Biochemistry (2010) 1.68
Dominant-negative inhibition of prion replication in transgenic mice. Proc Natl Acad Sci U S A (2002) 1.65
Synthetic prions generated in vitro are similar to a newly identified subpopulation of PrPSc from sporadic Creutzfeldt-Jakob Disease. Protein Sci (2005) 1.62
Evaluation of the human transmission risk of an atypical bovine spongiform encephalopathy prion strain. J Virol (2008) 1.59
Stress-inducible protein 1 is a cell surface ligand for cellular prion that triggers neuroprotection. EMBO J (2002) 1.58
Transgenic fatal familial insomnia mice indicate prion infectivity-independent mechanisms of pathogenesis and phenotypic expression of disease. PLoS Pathog (2015) 1.54
Scrapie susceptibility-linked polymorphisms modulate the in vitro conversion of sheep prion protein to protease-resistant forms. Proc Natl Acad Sci U S A (1997) 1.54
Conversion of alpha-lactalbumin to a protein inducing apoptosis. Proc Natl Acad Sci U S A (2000) 1.54
Dominant-negative inhibition of prion formation diminished by deletion mutagenesis of the prion protein. J Virol (2000) 1.52
Trafficking of prion proteins through a caveolae-mediated endosomal pathway. J Cell Biol (2003) 1.51
Spontaneous generation of prion infectivity in fatal familial insomnia knockin mice. Neuron (2009) 1.49
Endogenous proteolytic cleavage of normal and disease-associated isoforms of the human prion protein in neural and non-neural tissues. Am J Pathol (1998) 1.48
Prions in skeletal muscle. Proc Natl Acad Sci U S A (2002) 1.48
Cellular biology of prion diseases. Clin Microbiol Rev (1999) 1.48
Prions on the move. EMBO Rep (2011) 1.47
The expanding universe of prion diseases. PLoS Pathog (2006) 1.45
PrP(ST), a soluble, protease resistant and truncated PrP form features in the pathogenesis of a genetic prion disease. PLoS One (2013) 1.45
Intramuscular injection of α-synuclein induces CNS α-synuclein pathology and a rapid-onset motor phenotype in transgenic mice. Proc Natl Acad Sci U S A (2014) 1.44
Transmission barriers for bovine, ovine, and human prions in transgenic mice. J Virol (2005) 1.43
A molecular switch controls interspecies prion disease transmission in mice. J Clin Invest (2010) 1.41
Mimicking dominant negative inhibition of prion replication through structure-based drug design. Proc Natl Acad Sci U S A (2000) 1.41
Prion hypothesis: the end of the controversy? Trends Biochem Sci (2010) 1.40
Propagation of prion strains through specific conformers of the prion protein. J Virol (1997) 1.35
Changing a single amino acid in the N-terminus of murine PrP alters TSE incubation time across three species barriers. EMBO J (2001) 1.30
Disruption of prion rods generates 10-nm spherical particles having high alpha-helical content and lacking scrapie infectivity. J Virol (1996) 1.30
NMR structures of three single-residue variants of the human prion protein. Proc Natl Acad Sci U S A (2000) 1.28
Prion protein PrPc interacts with molecular chaperones of the Hsp60 family. J Virol (1996) 1.28
Cell division modulates prion accumulation in cultured cells. Proc Natl Acad Sci U S A (2007) 1.25
Amphotericin B inhibits the generation of the scrapie isoform of the prion protein in infected cultures. J Virol (2000) 1.22
Dissociation of pathological and molecular phenotype of variant Creutzfeldt-Jakob disease in transgenic human prion protein 129 heterozygous mice. Proc Natl Acad Sci U S A (2006) 1.22
Trans-dominant inhibition of prion propagation in vitro is not mediated by an accessory cofactor. PLoS Pathog (2009) 1.21
Evidence for the role of PrP(C) helix 1 in the hydrophilic seeding of prion aggregates. Proc Natl Acad Sci U S A (1999) 1.19
De novo generation of prion strains. Nat Rev Microbiol (2011) 1.17
Humanized knock-in mice expressing chimeric prion protein showed varied susceptibility to different human prions. Am J Pathol (2003) 1.16
Prion strain-dependent differences in conversion of mutant prion proteins in cell culture. J Virol (2006) 1.14
Antihypertensive drug guanabenz is active in vivo against both yeast and mammalian prions. PLoS One (2008) 1.13
Cell-based quantification of chronic wasting disease prions. J Virol (2010) 1.13
The immunobiology of prion diseases. Nat Rev Immunol (2013) 1.13
Generation of a new form of human PrP(Sc) in vitro by interspecies transmission from cervid prions. J Biol Chem (2011) 1.12
Review: contribution of transgenic models to understanding human prion disease. Neuropathol Appl Neurobiol (2010) 1.11
Prion protein complexed to N2a cellular RNAs through its N-terminal domain forms aggregates and is toxic to murine neuroblastoma cells. J Biol Chem (2008) 1.11
Progress and problems in the biology, diagnostics, and therapeutics of prion diseases. J Clin Invest (2004) 1.09
The intriguing prion disorders. Cell Mol Life Sci (2006) 1.09
Amyloidogenesis of natively unfolded proteins. Curr Alzheimer Res (2008) 1.08
Small protease sensitive oligomers of PrPSc in distinct human prions determine conversion rate of PrP(C). PLoS Pathog (2012) 1.07
Cellular factors implicated in prion replication. FEBS Lett (2010) 1.07
Conformational diversity in prion protein variants influences intermolecular beta-sheet formation. EMBO J (2009) 1.07
PrP(C) association with lipid rafts in the early secretory pathway stabilizes its cellular conformation. Mol Biol Cell (2004) 1.07
High CJD infectivity remains after prion protein is destroyed. J Cell Biochem (2011) 1.06
Human prion strain selection in transgenic mice. Ann Neurol (2010) 1.06
Fatal prion disease in a mouse model of genetic E200K Creutzfeldt-Jakob disease. PLoS Pathog (2011) 1.06
Evidence that bank vole PrP is a universal acceptor for prions. PLoS Pathog (2014) 1.03
Unique structural characteristics of the rabbit prion protein. J Biol Chem (2010) 1.02
The role of dimerization in prion replication. Biophys J (2002) 1.02
Molecular pathology of human prion disease. Acta Neuropathol (2010) 1.01
Spontaneous generation of mammalian prions. Proc Natl Acad Sci U S A (2010) 1.00
PrP polymorphisms tightly control sheep prion replication in cultured cells. J Virol (2003) 0.99
Propagation of RML prions in mice expressing PrP devoid of GPI anchor leads to formation of a novel, stable prion strain. PLoS Pathog (2012) 0.98
Mouse models for studying the formation and propagation of prions. J Biol Chem (2014) 0.98
Cellular prion protein conformation and function. Proc Natl Acad Sci U S A (2011) 0.97
Exploring the propensities of helices in PrP(C) to form beta sheet using NMR structures and sequence alignments. Biophys J (2002) 0.96
Variety of antiprion compounds discovered through an in silico screen based on cellular-form prion protein structure: Correlation between antiprion activity and binding affinity. Antimicrob Agents Chemother (2008) 0.95
Iatrogenic CJD due to pituitary-derived growth hormone with genetically determined incubation times of up to 40 years. Brain (2015) 0.95
Novel proteinaceous infectious particles cause scrapie. Science (1982) 24.09
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
An evolutionary trace method defines binding surfaces common to protein families. J Mol Biol (1996) 9.31
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
Bayesian statistical analysis of protein side-chain rotamer preferences. Protein Sci (1997) 7.92
Scrapie prions aggregate to form amyloid-like birefringent rods. Cell (1983) 7.92
Identification of a protein that purifies with the scrapie prion. Science (1982) 7.00
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
Weight-supported treadmill vs over-ground training for walking after acute incomplete SCI. Neurology (2006) 6.66
Transgenetic studies implicate interactions between homologous PrP isoforms in scrapie prion replication. Cell (1990) 6.17
Purification and structural studies of a major scrapie prion protein. Cell (1984) 5.98
Scrapie prion protein contains a phosphatidylinositol glycolipid. Cell (1987) 5.12
Formic acid pretreatment enhances immunostaining of cerebral and systemic amyloids. Lab Invest (1987) 4.98
Scrapie and cellular PrP isoforms are encoded by the same chromosomal gene. Cell (1986) 4.82
Prediction of protein side-chain rotamers from a backbone-dependent rotamer library: a new homology modeling tool. J Mol Biol (1997) 4.79
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
Separation and properties of cellular and scrapie prion proteins. Proc Natl Acad Sci U S A (1986) 4.38
Prion protein biology. Cell (1998) 4.20
Spontaneous neurodegeneration in transgenic mice with mutant prion protein. Science (1990) 4.19
Improvements in protein secondary structure prediction by an enhanced neural network. J Mol Biol (1990) 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
Further purification and characterization of scrapie prions. Biochemistry (1982) 4.01
Information access. Building a "GenBank" of the published literature. Science (2001) 3.95
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
A transmembrane form of the prion protein in neurodegenerative disease. Science (1998) 3.76
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
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
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
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
Compelling transgenetic evidence for transmission of bovine spongiform encephalopathy prions to humans. Proc Natl Acad Sci U S A (1999) 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
Acridine and phenothiazine derivatives as pharmacotherapeutics for prion disease. Proc Natl Acad Sci U S A (2001) 3.39
Scrapie-infected murine neuroblastoma cells produce protease-resistant prion proteins. J Virol (1988) 3.37
Co-evolution of proteins with their interaction partners. J Mol Biol (2000) 3.23
Distinct prion proteins in short and long scrapie incubation period mice. Cell (1987) 3.21
Antibodies to a scrapie prion protein. Nature (1984) 3.09
Identification of prion amyloid filaments in scrapie-infected brain. Cell (1985) 3.04
A conformational transition at the N terminus of the prion protein features in formation of the scrapie isoform. J Mol Biol (1997) 2.95
Propagation of prions with artificial properties in transgenic mice expressing chimeric PrP genes. Cell (1993) 2.93
Ataxia in prion protein (PrP)-deficient mice is associated with upregulation of the novel PrP-like protein doppel. J Mol Biol (1999) 2.92
Rapid detection of Creutzfeldt-Jakob disease and scrapie prion proteins. Neurology (1990) 2.90
Replication of distinct scrapie prion isolates is region specific in brains of transgenic mice and hamsters. Genes Dev (1992) 2.87
Transmissible and genetic prion diseases share a common pathway of neurodegeneration. Nature (1999) 2.83
Subcellular colocalization of the cellular and scrapie prion proteins in caveolae-like membranous domains. Proc Natl Acad Sci U S A (1996) 2.79
Antibodies inhibit prion propagation and clear cell cultures of prion infectivity. Nature (2001) 2.78
Transmission of Creutzfeldt-Jakob disease from humans to transgenic mice expressing chimeric human-mouse prion protein. Proc Natl Acad Sci U S A (1994) 2.70
Degeneration of skeletal muscle, peripheral nerves, and the central nervous system in transgenic mice overexpressing wild-type prion proteins. Cell (1994) 2.68
Evidence for synthesis of scrapie prion proteins in the endocytic pathway. J Biol Chem (1992) 2.67
Chimeric prion protein expression in cultured cells and transgenic mice. Protein Sci (1992) 2.67
Predicted alpha-helical regions of the prion protein when synthesized as peptides form amyloid. Proc Natl Acad Sci U S A (1992) 2.64