Published in N Engl J Med on March 08, 1984
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Novel proteinaceous infectious particles cause scrapie. Science (1982) 24.09
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
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
Purification and structural studies of a major scrapie prion protein. Cell (1984) 5.98
Prion propagation in mice expressing human and chimeric PrP transgenes implicates the interaction of cellular PrP with another protein. Cell (1995) 5.16
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
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
Spontaneous neurodegeneration in transgenic mice with mutant prion protein. Science (1990) 4.19
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
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
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
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
Mapping the prion protein using recombinant antibodies. J Virol (1998) 2.64
Purified prion proteins and scrapie infectivity copartition into liposomes. Proc Natl Acad Sci U S A (1987) 2.61
Strain-specified relative conformational stability of the scrapie prion protein. Protein Sci (2001) 2.60
Scrapie prion proteins are synthesized in neurons. Am J Pathol (1986) 2.54
Pathologic conformations of prion proteins. Annu Rev Biochem (1998) 2.50
Copper binding to the prion protein: structural implications of four identical cooperative binding sites. Proc Natl Acad Sci U S A (1999) 2.44
Prion protein gene variation among primates. J Mol Biol (1995) 2.43
Molecular cloning of a human prion protein cDNA. DNA (1986) 2.43
Scrapie prion proteins accumulate in the cytoplasm of persistently infected cultured cells. J Cell Biol (1990) 2.42
Scrapie PrP 27-30 is a sialoglycoprotein. J Virol (1985) 2.40
Interactions between wild-type and mutant prion proteins modulate neurodegeneration in transgenic mice. Genes Dev (1996) 2.39
Cultured cell sublines highly susceptible to prion infection. J Virol (2000) 2.38
Prion isolate specified allotypic interactions between the cellular and scrapie prion proteins in congenic and transgenic mice. Proc Natl Acad Sci U S A (1994) 2.38
Serial transmission in rodents of neurodegeneration from transgenic mice expressing mutant prion protein. Proc Natl Acad Sci U S A (1994) 2.37
Purification and properties of the cellular and scrapie hamster prion proteins. Eur J Biochem (1988) 2.35
Prion protein selectively binds copper(II) ions. Biochemistry (1998) 2.32
A hypothalamic neuronal cell line persistently infected with scrapie prions exhibits apoptosis. J Virol (1997) 2.32
Changes in the localization of brain prion proteins during scrapie infection. Neurology (1987) 2.27
Molecular properties, partial purification, and assay by incubation period measurements of the hamster scrapie agent. Biochemistry (1980) 2.27
Synthesis and trafficking of prion proteins in cultured cells. Mol Biol Cell (1992) 2.14
Genetics and polymorphism of the mouse prion gene complex: control of scrapie incubation time. Mol Cell Biol (1988) 2.14
Ultrastructural localization of scrapie prion proteins in cytoplasmic vesicles of infected cultured cells. Lab Invest (1991) 2.13
Monoclonal antibodies to the cellular and scrapie prion proteins. J Infect Dis (1986) 2.13
Recombinant scrapie-like prion protein of 106 amino acids is soluble. Proc Natl Acad Sci U S A (1996) 2.12
Scrapie prion rod formation in vitro requires both detergent extraction and limited proteolysis. J Virol (1991) 2.11
Insertion in prion protein gene in familial Creutzfeldt-Jakob disease. Lancet (1989) 2.11
Perturbation of the secondary structure of the scrapie prion protein under conditions that alter infectivity. Proc Natl Acad Sci U S A (1993) 2.10
Mutation of the prion protein in Libyan Jews with Creutzfeldt-Jakob disease. N Engl J Med (1991) 2.05
Prion protein conformation in a patient with sporadic fatal insomnia. N Engl J Med (1999) 2.04
Creutzfeldt-Jakob disease prion proteins in human brains. N Engl J Med (1985) 2.04
Identification of the Cu2+ binding sites in the N-terminal domain of the prion protein by EPR and CD spectroscopy. Biochemistry (2000) 2.02
Characterization of cloned cDNA representing rat myelin basic protein: absence of expression in brain of shiverer mutant mice. Cell (1983) 2.02
Homozygosity for prion protein alleles encoding glutamine-171 renders sheep susceptible to natural scrapie. Genes Dev (1994) 2.00
Cloning and characterization of the myelin basic protein gene from mouse: one gene can encode both 14 kd and 18.5 kd MBPs by alternate use of exons. Cell (1985) 1.98
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
High-level expression and characterization of a purified 142-residue polypeptide of the prion protein. Biochemistry (1996) 1.87
Prion protein of 106 residues creates an artifical transmission barrier for prion replication in transgenic mice. Cell (1999) 1.87
Conversion of truncated and elongated prion proteins into the scrapie isoform in cultured cells. Proc Natl Acad Sci U S A (1993) 1.87
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
Epitope mapping of the Syrian hamster prion protein utilizing chimeric and mutant genes in a vaccinia virus expression system. J Immunol (1991) 1.87
Solution structure of Syrian hamster prion protein rPrP(90-231). Biochemistry (1999) 1.86
Induction of distinct [URE3] yeast prion strains. Mol Cell Biol (2001) 1.85
Purification and properties of the cellular prion protein from Syrian hamster brain. Protein Sci (1992) 1.85
Amyloid plaques in Creutzfeldt-Jakob disease stain with prion protein antibodies. Ann Neurol (1986) 1.85
Scrapie prion liposomes and rods exhibit target sizes of 55,000 Da. Virology (1988) 1.85
Folding of prion protein to its native alpha-helical conformation is under kinetic control. J Biol Chem (2001) 1.84
Heritable disorder resembling neuronal storage disease in mice expressing prion protein with deletion of an alpha-helix. Nat Med (1997) 1.83
A mouse prion protein transgene rescues mice deficient for the prion protein gene from purkinje cell degeneration and demyelination. Lab Invest (1999) 1.83
Selective neuronal targeting in prion disease. Neuron (1997) 1.82
Chemical chaperones interfere with the formation of scrapie prion protein. EMBO J (1996) 1.81
Amyloid protein of Gerstmann-Sträussler-Scheinker disease (Indiana kindred) is an 11 kd fragment of prion protein with an N-terminal glycine at codon 58. EMBO J (1991) 1.74
Quantitative trait loci affecting prion incubation time in mice. Genomics (2000) 1.73