Published in Science on January 28, 2010
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
Prions. Cold Spring Harb Perspect Biol (2011) 3.17
The tip of the iceberg: RNA-binding proteins with prion-like domains in neurodegenerative disease. Brain Res (2012) 3.00
Pathogenic protein seeding in Alzheimer disease and other neurodegenerative disorders. Ann Neurol (2011) 2.94
Mammalian prions generated from bacterially expressed prion protein in the absence of any mammalian cofactors. J Biol Chem (2010) 2.57
Prion-like disorders: blurring the divide between transmissibility and infectivity. J Cell Sci (2010) 2.29
Rapid cell-surface prion protein conversion revealed using a novel cell system. Nat Commun (2011) 2.04
The seeds of neurodegeneration: prion-like spreading in ALS. Cell (2011) 2.03
Isolation of phosphatidylethanolamine as a solitary cofactor for prion formation in the absence of nucleic acids. Proc Natl Acad Sci U S A (2012) 1.97
Structural organization of brain-derived mammalian prions examined by hydrogen-deuterium exchange. Nat Struct Mol Biol (2011) 1.90
Cell-to-cell transmission of pathogenic proteins in neurodegenerative diseases. Nat Med (2014) 1.85
Cofactor molecules maintain infectious conformation and restrict strain properties in purified prions. Proc Natl Acad Sci U S A (2012) 1.77
Species-dependent differences in cofactor utilization for formation of the protease-resistant prion protein in vitro. Biochemistry (2010) 1.68
Exosomes: vehicles for the transfer of toxic proteins associated with neurodegenerative diseases? Front Physiol (2012) 1.65
Rabbits are not resistant to prion infection. Proc Natl Acad Sci U S A (2012) 1.63
Biology and genetics of prions causing neurodegeneration. Annu Rev Genet (2013) 1.63
The α-helical C-terminal domain of full-length recombinant PrP converts to an in-register parallel β-sheet structure in PrP fibrils: evidence from solid state nuclear magnetic resonance. Biochemistry (2010) 1.53
Genesis of mammalian prions: from non-infectious amyloid fibrils to a transmissible prion disease. PLoS Pathog (2011) 1.53
Transmissible proteins: expanding the prion heresy. Cell (2012) 1.49
Prion-like spread of protein aggregates in neurodegeneration. J Exp Med (2012) 1.49
The strain-encoded relationship between PrP replication, stability and processing in neurons is predictive of the incubation period of disease. PLoS Pathog (2011) 1.49
Prions on the move. EMBO Rep (2011) 1.47
Highly efficient protein misfolding cyclic amplification. PLoS Pathog (2011) 1.46
A bovine cell line that can be infected by natural sheep scrapie prions. PLoS One (2015) 1.44
Prion hypothesis: the end of the controversy? Trends Biochem Sci (2010) 1.40
Increased Abundance of M Cells in the Gut Epithelium Dramatically Enhances Oral Prion Disease Susceptibility. PLoS Pathog (2016) 1.38
Occurrence, transmission, and zoonotic potential of chronic wasting disease. Emerg Infect Dis (2012) 1.34
Exosomes: vesicular carriers for intercellular communication in neurodegenerative disorders. Cell Tissue Res (2012) 1.31
Degradation of misfolded proteins in neurodegenerative diseases: therapeutic targets and strategies. Exp Mol Med (2015) 1.29
Globular domain of the prion protein needs to be unlocked by domain swapping to support prion protein conversion. J Biol Chem (2011) 1.29
Parallel in-register intermolecular β-sheet architectures for prion-seeded prion protein (PrP) amyloids. J Biol Chem (2014) 1.27
Lower specific infectivity of protease-resistant prion protein generated in cell-free reactions. Proc Natl Acad Sci U S A (2011) 1.26
A new mechanism for transmissible prion diseases. J Neurosci (2012) 1.25
Prion seeding activities of mouse scrapie strains with divergent PrPSc protease sensitivities and amyloid plaque content using RT-QuIC and eQuIC. PLoS One (2012) 1.25
Prion propagation, toxicity and degradation. Nat Neurosci (2012) 1.25
Neurodegenerative diseases: expanding the prion concept. Annu Rev Neurosci (2015) 1.24
Ultra-efficient PrP(Sc) amplification highlights potentialities and pitfalls of PMCA technology. PLoS Pathog (2011) 1.17
De novo generation of prion strains. Nat Rev Microbiol (2011) 1.17
Stabilization of a prion strain of synthetic origin requires multiple serial passages. J Biol Chem (2012) 1.13
The immunobiology of prion diseases. Nat Rev Immunol (2013) 1.13
Biochemistry. What makes a prion infectious? Science (2010) 1.12
Review: contribution of transgenic models to understanding human prion disease. Neuropathol Appl Neurobiol (2010) 1.11
Relationship between conformational stability and amplification efficiency of prions. Biochemistry (2011) 1.11
Role of the highly conserved middle region of prion protein (PrP) in PrP-lipid interaction. Biochemistry (2010) 1.11
Replication efficiency of soil-bound prions varies with soil type. J Virol (2011) 1.11
Intermolecular alignment in Y145Stop human prion protein amyloid fibrils probed by solid-state NMR spectroscopy. J Am Chem Soc (2011) 1.10
Protein misfolding cyclic amplification of infectious prions. Nat Protoc (2012) 1.10
Genetic informational RNA is not required for recombinant prion infectivity. J Virol (2011) 1.10
High-resolution structure of infectious prion protein: the final frontier. Nat Struct Mol Biol (2012) 1.09
Prion-like domains as epigenetic regulators, scaffolds for subcellular organization, and drivers of neurodegenerative disease. Brain Res (2016) 1.08
Dissociation of infectivity from seeding ability in prions with alternate docking mechanism. PLoS Pathog (2011) 1.07
Cellular factors implicated in prion replication. FEBS Lett (2010) 1.07
Biochemical properties of highly neuroinvasive prion strains. PLoS Pathog (2012) 1.07
Small protease sensitive oligomers of PrPSc in distinct human prions determine conversion rate of PrP(C). PLoS Pathog (2012) 1.07
High CJD infectivity remains after prion protein is destroyed. J Cell Biochem (2011) 1.06
Protein-only mechanism induces self-perpetuating changes in the activity of neuronal Aplysia cytoplasmic polyadenylation element binding protein (CPEB). Proc Natl Acad Sci U S A (2011) 1.06
Molecular biology and pathology of prion strains in sporadic human prion diseases. Acta Neuropathol (2010) 1.06
Oxidation of Helix-3 methionines precedes the formation of PK resistant PrP. PLoS Pathog (2010) 1.06
Sulfated dextrans enhance in vitro amplification of bovine spongiform encephalopathy PrP(Sc) and enable ultrasensitive detection of bovine PrP(Sc). PLoS One (2010) 1.05
Quantitative detection and biological propagation of scrapie seeding activity in vitro facilitate use of prions as model pathogens for disinfection. PLoS One (2011) 1.05
Cofactor molecules induce structural transformation during infectious prion formation. Structure (2013) 1.05
Emergence and natural selection of drug-resistant prions. Mol Biosyst (2010) 1.05
Seeding specificity and ultrastructural characteristics of infectious recombinant prions. Biochemistry (2011) 1.04
Rapid transepithelial transport of prions following inhalation. J Virol (2012) 1.04
Intrinsically Disordered Proteins Drive Emergence and Inheritance of Biological Traits. Cell (2016) 1.04
Binding with nucleic acids or glycosaminoglycans converts soluble protein oligomers to amyloid. J Biol Chem (2011) 1.04
Strain-specific role of RNAs in prion replication. J Virol (2012) 1.04
Prions and the potential transmissibility of protein misfolding diseases. Annu Rev Microbiol (2013) 1.04
Spontaneous generation of rapidly transmissible prions in transgenic mice expressing wild-type bank vole prion protein. Proc Natl Acad Sci U S A (2012) 1.03
The core of Ure2p prion fibrils is formed by the N-terminal segment in a parallel cross-β structure: evidence from solid-state NMR. J Mol Biol (2011) 1.03
Targeting protein aggregation for the treatment of degenerative diseases. Nat Rev Drug Discov (2015) 1.02
Unique structural characteristics of the rabbit prion protein. J Biol Chem (2010) 1.02
Fast and ultrasensitive method for quantitating prion infectivity titre. Nat Commun (2012) 1.01
Prion amyloid structure explains templating: how proteins can be genes. FEMS Yeast Res (2010) 1.01
Isolation of novel synthetic prion strains by amplification in transgenic mice coexpressing wild-type and anchorless prion proteins. J Virol (2012) 1.01
Rapidly progressive dementias and the treatment of human prion diseases. Expert Opin Pharmacother (2010) 1.00
Spontaneous generation of mammalian prions. Proc Natl Acad Sci U S A (2010) 1.00
The role of cofactors in prion propagation and infectivity. PLoS Pathog (2012) 0.99
Spongiform encephalopathy in transgenic mice expressing a point mutation in the β2-α2 loop of the prion protein. J Neurosci (2011) 0.99
Changes in prion replication environment cause prion strain mutation. FASEB J (2013) 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
Generating extracellular amyloid aggregates using E. coli cells. Genes Dev (2012) 0.98
In situ photodegradation of incorporated polyanion does not alter prion infectivity. PLoS Pathog (2011) 0.97
De novo generation of infectious prions with bacterially expressed recombinant prion protein. FASEB J (2013) 0.97
Detection of prion protein particles in blood plasma of scrapie infected sheep. PLoS One (2012) 0.96
Selective amplification of classical and atypical prions using modified protein misfolding cyclic amplification. J Biol Chem (2012) 0.96
Down-regulation of Shadoo in prion infections traces a pre-clinical event inversely related to PrP(Sc) accumulation. PLoS Pathog (2011) 0.96
Acquisition of drug resistance and dependence by prions. PLoS Pathog (2013) 0.96
Assessment of strain-specific PrP(Sc) elongation rates revealed a transformation of PrP(Sc) properties during protein misfolding cyclic amplification. PLoS One (2012) 0.95
Conversion of bacterially expressed recombinant prion protein. Methods (2010) 0.95
Brain iron homeostasis: from molecular mechanisms to clinical significance and therapeutic opportunities. Antioxid Redox Signal (2013) 0.94
Charge neutralization of the central lysine cluster in prion protein (PrP) promotes PrP(Sc)-like folding of recombinant PrP amyloids. J Biol Chem (2014) 0.94
The N-terminal, polybasic region of PrP(C) dictates the efficiency of prion propagation by binding to PrP(Sc). J Neurosci (2012) 0.93
Highly neurotoxic monomeric α-helical prion protein. Proc Natl Acad Sci U S A (2012) 0.92
Conformational stability of mammalian prion protein amyloid fibrils is dictated by a packing polymorphism within the core region. J Biol Chem (2013) 0.92
Prion disease tempo determined by host-dependent substrate reduction. J Clin Invest (2014) 0.92
Synthesis of high titer infectious prions with cofactor molecules. J Biol Chem (2014) 0.92
The iron regulatory capability of the major protein participants in prevalent neurodegenerative disorders. Front Pharmacol (2014) 0.91
In vitro amplification of misfolded prion protein using lysate of cultured cells. PLoS One (2011) 0.91
Novel proteinaceous infectious particles cause scrapie. Science (1982) 24.09
Mice devoid of PrP are resistant to scrapie. Cell (1993) 11.46
Synthetic mammalian prions. Science (2004) 7.35
A general model of prion strains and their pathogenicity. Science (2007) 6.94
In vitro generation of infectious scrapie prions. Cell (2005) 6.86
Evidence for the conformation of the pathologic isoform of the prion protein enciphering and propagating prion diversity. Science (1996) 6.71
Cell-free formation of protease-resistant prion protein. Nature (1994) 6.07
Formation of native prions from minimal components in vitro. Proc Natl Acad Sci U S A (2007) 5.94
Non-genetic propagation of strain-specific properties of scrapie prion protein. Nature (1995) 4.22
RNA molecules stimulate prion protein conversion. Nature (2003) 4.06
The prion's elusive reason for being. Annu Rev Neurosci (2008) 3.23
Getting a grip on prions: oligomers, amyloids, and pathological membrane interactions. Annu Rev Biochem (2009) 2.80
Purified prion proteins and scrapie infectivity copartition into liposomes. Proc Natl Acad Sci U S A (1987) 2.61
Prions and their partners in crime. Nature (2006) 2.55
How membranes shape protein structure. J Biol Chem (2001) 2.48
Protease-resistant prion protein amplification reconstituted with partially purified substrates and synthetic polyanions. J Biol Chem (2005) 2.29
Cell-free propagation of prion strains. EMBO J (2008) 2.20
A 25 nm virion is the likely cause of transmissible spongiform encephalopathies. J Cell Biochem (2007) 2.04
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
Selective incorporation of polyanionic molecules into hamster prions. J Biol Chem (2007) 1.71
Lipid interaction converts prion protein to a PrPSc-like proteinase K-resistant conformation under physiological conditions. Biochemistry (2007) 1.62
Genes contributing to prion pathogenesis. J Gen Virol (2008) 1.51
Birth of a prion: spontaneous generation revisited. Cell (2005) 1.42
Mouse-adapted scrapie infection of SN56 cells: greater efficiency with microsome-associated versus purified PrP-res. J Virol (2006) 1.35
An aggregation-specific enzyme-linked immunosorbent assay: detection of conformational differences between recombinant PrP protein dimers and PrP(Sc) aggregates. J Virol (2005) 1.22
Foxm1b transcription factor is essential for development of hepatocellular carcinomas and is negatively regulated by the p19ARF tumor suppressor. Genes Dev (2004) 3.91
Transcription factors in liver development, differentiation, and regeneration. Hepatology (2003) 3.06
The mouse Forkhead Box m1 transcription factor is essential for hepatoblast mitosis and development of intrahepatic bile ducts and vessels during liver morphogenesis. Dev Biol (2004) 2.19
Isolation of phosphatidylethanolamine as a solitary cofactor for prion formation in the absence of nucleic acids. Proc Natl Acad Sci U S A (2012) 1.97
Growth hormone stimulates proliferation of old-aged regenerating liver through forkhead box m1b. Hepatology (2003) 1.94
Cofactor molecules maintain infectious conformation and restrict strain properties in purified prions. Proc Natl Acad Sci U S A (2012) 1.77
Ubiquitous expression of the forkhead box M1B transgene accelerates proliferation of distinct pulmonary cell types following lung injury. J Biol Chem (2003) 1.63
Lipid interaction converts prion protein to a PrPSc-like proteinase K-resistant conformation under physiological conditions. Biochemistry (2007) 1.62
Mechanism of arylating quinone toxicity involving Michael adduct formation and induction of endoplasmic reticulum stress. Proc Natl Acad Sci U S A (2006) 1.43
dELL is an essential RNA polymerase II elongation factor with a general role in development. Proc Natl Acad Sci U S A (2002) 1.34
Role of the highly conserved middle region of prion protein (PrP) in PrP-lipid interaction. Biochemistry (2010) 1.11
Genetic informational RNA is not required for recombinant prion infectivity. J Virol (2011) 1.10
Cofactor molecules induce structural transformation during infectious prion formation. Structure (2013) 1.05
Seeding specificity and ultrastructural characteristics of infectious recombinant prions. Biochemistry (2011) 1.04
De novo generation of infectious prions with bacterially expressed recombinant prion protein. FASEB J (2013) 0.97
Generation of goats lacking prion protein. Mol Reprod Dev (2009) 0.96
Conversion of bacterially expressed recombinant prion protein. Methods (2010) 0.95
The interaction between cytoplasmic prion protein and the hydrophobic lipid core of membrane correlates with neurotoxicity. J Biol Chem (2006) 0.91
Tocopherol metabolism using thermochemolysis: chemical and biological properties of gamma-tocopherol, gamma-carboxyethyl-hydroxychroman, and their quinones. Chem Res Toxicol (2005) 0.90
Calpain and other cytosolic proteases can contribute to the degradation of retro-translocated prion protein in the cytosol. J Biol Chem (2004) 0.88
Adenovirus-mediated increase in HNF-3beta or HNF-3alpha shows differences in levels of liver glycogen and gene expression. Hepatology (2002) 0.86
Cytoplasmic prion protein induces forebrain neurotoxicity. Biochim Biophys Acta (2009) 0.86
The important roles of RET, VEGFR2 and the RAF/MEK/ERK pathway in cancer treatment with sorafenib. Acta Pharmacol Sin (2012) 0.85
Nutritional benefit of olive oil: the biological effects of hydroxytyrosol and its arylating quinone adducts. J Agric Food Chem (2008) 0.84
Context-dependent perturbation of neural systems in transgenic mice expressing a cytosolic prion protein. Neuroimage (2009) 0.83
Quinoprotein adducts accumulate in the substantia nigra of aged rats and correlate with dopamine-induced toxicity in SH-SY5Y cells. Neurochem Res (2011) 0.81
Galanin in neuro(glio)genesis: expression of galanin and receptors by progenitor cells in vivo and in vitro and effects of galanin on neurosphere proliferation. Neuropeptides (2005) 0.80
Silencing prion protein in MDA-MB-435 breast cancer cells leads to pleiotropic cellular responses to cytotoxic stimuli. PLoS One (2012) 0.79
Searching for type 2 diabetes genes on chromosome 20. Diabetes (2002) 0.79
Studies in vitamin E: biochemistry and molecular biology of tocopherol quinones. Vitam Horm (2007) 0.78
[Effects of traditional Chinese herbal medicine on the neurobehavioral manifestations and the activity of dopamine D2 receptor in corpora striatum of rats with levodopa-induced dyskinesias]. Zhong Xi Yi Jie He Xue Bao (2008) 0.78
Role of lipid in forming an infectious prion? Acta Biochim Biophys Sin (Shanghai) (2013) 0.78
Galanin inhibits the proliferation of glial olfactory ensheathing cells. Neuropeptides (2005) 0.77
Differential effect of galanin on proliferation of PC12 and B104 cells. Neuroreport (2007) 0.76
How strong is the evidence that Parkinson's disease is a prion disorder? Curr Opin Neurol (2016) 0.75