Prion protein gene variation among primates.

Prions. Proc Natl Acad Sci U S A (1998) 27.80

NMR solution structure of the human prion protein. Proc Natl Acad Sci U S A (2000) 4.80

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

Substrate-specific translocational attenuation during ER stress defines a pre-emptive quality control pathway. Cell (2006) 3.26

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

NMR structure of the bovine prion protein. Proc Natl Acad Sci U S A (2000) 2.56

The 37-kDa/67-kDa laminin receptor acts as the cell-surface receptor for the cellular prion protein. EMBO J (2001) 2.16

De novo generation of a transmissible spongiform encephalopathy by mouse transgenesis. Proc Natl Acad Sci U S A (2008) 2.10

Prion protein NMR structure and familial human spongiform encephalopathies. Proc Natl Acad Sci U S A (1998) 2.02

Prion protein NMR structure and species barrier for prion diseases. Proc Natl Acad Sci U S A (1997) 1.86

Prion protein expression in different species: analysis with a panel of new mAbs. Proc Natl Acad Sci U S A (1998) 1.80

Prion protein NMR structures of cats, dogs, pigs, and sheep. Proc Natl Acad Sci U S A (2005) 1.66

A scrapie-like unfolding intermediate of the prion protein domain PrP(121-231) induced by acidic pH. Proc Natl Acad Sci U S A (1998) 1.55

Overexpression of nonconvertible PrPc delta114-121 in scrapie-infected mouse neuroblastoma cells leads to trans-dominant inhibition of wild-type PrP(Sc) accumulation. J Virol (1998) 1.49

RNA aptamers specifically interact with the prion protein PrP. J Virol (1997) 1.43

A molecular switch controls interspecies prion disease transmission in mice. J Clin Invest (2010) 1.41

Susceptibilities of nonhuman primates to chronic wasting disease. Emerg Infect Dis (2009) 1.33

Intracellular re-routing of prion protein prevents propagation of PrP(Sc) and delays onset of prion disease. EMBO J (2001) 1.29

NMR structures of three single-residue variants of the human prion protein. Proc Natl Acad Sci U S A (2000) 1.28

Comparative genomic analysis of prion genes. BMC Genomics (2007) 1.11

The polybasic N-terminal region of the prion protein controls the physical properties of both the cellular and fibrillar forms of PrP. J Mol Biol (2008) 1.01

Disruption of Doppel prevents neurodegeneration in mice with extensive Prnp deletions. Proc Natl Acad Sci U S A (2004) 1.01

Cellular prion protein conformation and function. Proc Natl Acad Sci U S A (2011) 0.97

Cellular aspects of prion replication in vitro. Viruses (2013) 0.95

PrPSc-like prion protein peptide inhibits the function of cellular prion protein. Biochem J (2000) 0.93

Genetic susceptibility, evolution and the kuru epidemic. Philos Trans R Soc Lond B Biol Sci (2008) 0.91

Altered toxicity of the prion protein peptide PrP106-126 carrying the Ala(117)-->Val mutation. Biochem J (2000) 0.91

Characteristics of 263K scrapie agent in multiple hamster species. Emerg Infect Dis (2009) 0.90

Prion protein-detergent micelle interactions studied by NMR in solution. J Biol Chem (2009) 0.89

PrionHome: a database of prions and other sequences relevant to prion phenomena. PLoS One (2012) 0.89

Strain specific resistance to murine scrapie associated with a naturally occurring human prion protein polymorphism at residue 171. PLoS Pathog (2011) 0.88

Novel aspects of prions, their receptor molecules, and innovative approaches for TSE therapy. Cell Mol Neurobiol (2006) 0.85

Prion protein (PrP) gene-knockout cell lines: insight into functions of the PrP. Front Cell Dev Biol (2015) 0.85

Structural plasticity of the cellular prion protein and implications in health and disease. Proc Natl Acad Sci U S A (2013) 0.85

NMR experiments for resonance assignments of 13C, 15N doubly-labeled flexible polypeptides: application to the human prion protein hPrP(23-230). J Biomol NMR (2000) 0.84

Conservation of a glycine-rich region in the prion protein is required for uptake of prion infectivity. J Biol Chem (2010) 0.83

Endogenous prion protein attenuates experimentally induced colitis. Am J Pathol (2011) 0.82

Antiaggregating antibody raised against human PrP 106-126 recognizes pathological and normal isoforms of the whole prion protein. Cell Mol Neurobiol (2001) 0.82

Prion protein in milk. PLoS One (2006) 0.82

Evidence for a pathogenic role of different mutations at codon 188 of PRNP. PLoS One (2008) 0.81

Transmission of scrapie prions to primate after an extended silent incubation period. Sci Rep (2015) 0.80

Pathologic evidence that the T188R mutation in PRNP is associated with prion disease. J Neuropathol Exp Neurol (2010) 0.80

Characterization of the genomic region containing the Shadow of Prion Protein (SPRN) gene in sheep. BMC Genomics (2007) 0.80

Molecular origin of Gerstmann-Sträussler-Scheinker syndrome: insight from computer simulation of an amyloidogenic prion peptide. Biophys J (2011) 0.79

Transmissible spongiform encephalopathies and tissue cell culture. Cytotechnology (2002) 0.79

Increased infectivity of anchorless mouse scrapie prions in transgenic mice overexpressing human prion protein. J Virol (2015) 0.79

Function of PrPC (1-OPRD) in biological activities of gastric cancer cell lines. J Cell Mol Med (2009) 0.79

Pathogenic mutations within the hydrophobic domain of the prion protein lead to the formation of protease-sensitive prion species with increased lethality. J Virol (2013) 0.78

Positive selection in prion protein. J Mol Evol (2009) 0.77

Enzymatic degradation of PrPSc by a protease secreted from Aeropyrum pernix K1. PLoS One (2012) 0.77

Spectroscopic and Theoretical Study of Cu(I) Binding to His111 in the Human Prion Protein Fragment 106-115. Inorg Chem (2016) 0.77

Negative purifying selection drives prion and doppel protein evolution. J Mol Evol (2014) 0.76

Conditional modulation of membrane protein expression in cultured cells mediated by prion protein recognition of short phosphorothioate oligodeoxynucleotides. J Biol Chem (2010) 0.76

Assessing proteinase K resistance of fish prion proteins in a scrapie-infected mouse neuroblastoma cell line. Viruses (2014) 0.75

From missiles to malaria. Lab Anim (NY) (2015) 0.75

Disparate Modes of Evolution Shaped Modern Prion (PRNP) and Prion-Related Doppel (PRND) Variation in Domestic Cattle. PLoS One (2016) 0.75

Cellular Prion Protein (PrP(c)) and Hypoxia: True to Each Other in Good Times and in Bad, in Sickness, and in Health. Front Cell Neurosci (2016) 0.75

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

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

The epic project: developing national evidence-based guidelines for preventing healthcare associated infections. Phase I: Guidelines for preventing hospital-acquired infections. Department of Health (England). J Hosp Infect (2001) 4.95

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

Demographics of the gay and lesbian population in the United States: evidence from available systematic data sources. Demography (2000) 4.23

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

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

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

Leber hereditary optic neuropathy: identification of the same mitochondrial ND1 mutation in six pedigrees. Am J Hum Genet (1991) 2.70

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

The rate and cost of hospital-acquired infections occurring in patients admitted to selected specialties of a district general hospital in England and the national burden imposed. J Hosp Infect (2001) 2.69

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

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

Collecting and harvesting biological data: the GPCRDB and NucleaRDB information systems. Nucleic Acids Res (2001) 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

Monoclonal antibodies to the cellular and scrapie prion proteins. J Infect Dis (1986) 2.13

Ultrastructural localization of scrapie prion proteins in cytoplasmic vesicles of infected cultured cells. Lab Invest (1991) 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