Published in Acta Neuropathol on January 06, 2010
A test for Creutzfeldt-Jakob disease using nasal brushings. N Engl J Med (2014) 3.58
Mammalian prions generated from bacterially expressed prion protein in the absence of any mammalian cofactors. J Biol Chem (2010) 2.57
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
Conformational transformation and selection of synthetic prion strains. J Mol Biol (2011) 1.66
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
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
Highly efficient protein misfolding cyclic amplification. PLoS Pathog (2011) 1.46
Prion hypothesis: the end of the controversy? Trends Biochem Sci (2010) 1.40
Coinfecting prion strains compete for a limiting cellular resource. J Virol (2010) 1.32
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
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
Two amyloid States of the prion protein display significantly different folding patterns. J Mol Biol (2010) 1.16
Stabilization of a prion strain of synthetic origin requires multiple serial passages. J Biol Chem (2012) 1.13
Relationship between conformational stability and amplification efficiency of prions. Biochemistry (2011) 1.11
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
Biochemical properties of highly neuroinvasive prion strains. PLoS Pathog (2012) 1.07
Cellular factors implicated in prion replication. FEBS Lett (2010) 1.07
Oxidation of Helix-3 methionines precedes the formation of PK resistant PrP. PLoS Pathog (2010) 1.06
Molecular biology and pathology of prion strains in sporadic human prion diseases. Acta Neuropathol (2010) 1.06
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
Rapid transepithelial transport of prions following inhalation. 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
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
Cellular mechanisms of protein aggregate propagation. Curr Opin Neurol (2012) 1.01
Changes in prion replication environment cause prion strain mutation. FASEB J (2013) 0.99
The role of cofactors in prion propagation and infectivity. PLoS Pathog (2012) 0.99
Comparative profiling of highly enriched 22L and Chandler mouse scrapie prion protein preparations. Proteomics (2010) 0.99
Convergent replication of mouse synthetic prion strains. Am J Pathol (2013) 0.98
Dynamic diagnosis of familial prion diseases supports the β2-α2 loop as a universal interference target. PLoS One (2011) 0.98
In vitro generation of high-titer prions. J Virol (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
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
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
Low density subcellular fractions enhance disease-specific prion protein misfolding. J Biol Chem (2010) 0.94
Conformational stability of mammalian prion protein amyloid fibrils is dictated by a packing polymorphism within the core region. J Biol Chem (2013) 0.92
Synthesis of high titer infectious prions with cofactor molecules. J Biol Chem (2014) 0.92
The evolution of transmissible prions: the role of deformed templating. PLoS Pathog (2013) 0.91
Recombinant prion protein refolded with lipid and RNA has the biochemical hallmarks of a prion but lacks in vivo infectivity. PLoS One (2013) 0.90
Sialylation of prion protein controls the rate of prion amplification, the cross-species barrier, the ratio of PrPSc glycoform and prion infectivity. PLoS Pathog (2014) 0.90
Distinct stability states of disease-associated human prion protein identified by conformation-dependent immunoassay. J Virol (2010) 0.89
Molecular structure of amyloid fibrils controls the relationship between fibrillar size and toxicity. PLoS One (2011) 0.89
Generation of prions in vitro and the protein-only hypothesis. Prion (2010) 0.88
Dissociation of prion protein amyloid seeding from transmission of a spongiform encephalopathy. J Virol (2013) 0.88
Prion-like propagation of protein aggregation and related therapeutic strategies. Neurotherapeutics (2013) 0.88
Pathology of SSLOW, a transmissible and fatal synthetic prion protein disorder, and comparison with naturally occurring classical transmissible spongiform encephalopathies. Neuropathol Appl Neurobiol (2014) 0.87
Detection of Atypical H-Type Bovine Spongiform Encephalopathy and Discrimination of Bovine Prion Strains by Real-Time Quaking-Induced Conversion. J Clin Microbiol (2016) 0.87
Dissecting structure of prion amyloid fibrils by hydrogen-deuterium exchange ultraviolet Raman spectroscopy. J Phys Chem B (2012) 0.87
Structural requirements for efficient prion protein conversion: cofactors may promote a conversion-competent structure for PrP(C). Prion (2010) 0.86
NLRP3 inflammasome activation in macrophage cell lines by prion protein fibrils as the source of IL-1β and neuronal toxicity. Cell Mol Life Sci (2012) 0.86
De novo prion aggregates trigger autophagy in skeletal muscle. J Virol (2013) 0.86
Conformational properties of prion strains can be transmitted to recombinant prion protein fibrils in real-time quaking-induced conversion. J Virol (2014) 0.86
Amyloid features and neuronal toxicity of mature prion fibrils are highly sensitive to high pressure. J Biol Chem (2011) 0.85
Prion nucleation site unmasked by transient interaction with phospholipid cofactor. Biochemistry (2014) 0.85
Strain typing of classical scrapie by transgenic mouse bioassay using protein misfolding cyclic amplification to replace primary passage. PLoS One (2013) 0.84
Genesis of tramsmissible protein states via deformed templating. Prion (2012) 0.84
Ethics in prion disease. Prog Neurobiol (2013) 0.83
Mammalian prions and their wider relevance in neurodegenerative diseases. Nature (2016) 0.83
Infectious particles, stress, and induced prion amyloids: a unifying perspective. Virulence (2013) 0.82
The many shades of prion strain adaptation. Prion (2014) 0.82
Recombinant human prion protein inhibits prion propagation in vitro. Sci Rep (2013) 0.82
Synthetic Aβ peptides acquire prion-like properties in the brain. Oncotarget (2015) 0.82
Kosmotropic anions promote conversion of recombinant prion protein into a PrPSc-like misfolded form. PLoS One (2012) 0.81
Plasminogen: A cellular protein cofactor for PrPSc propagation. Prion (2011) 0.81
Synthetic scrapie infectivity: interaction between recombinant PrP and scrapie brain-derived RNA. Virulence (2015) 0.81
A Structural and Functional Comparison Between Infectious and Non-Infectious Autocatalytic Recombinant PrP Conformers. PLoS Pathog (2015) 0.81
From prion diseases to prion-like propagation mechanisms of neurodegenerative diseases. Int J Cell Biol (2013) 0.81
Proper calibration of ultrasonic power enabled the quantitative analysis of the ultrasonication-induced amyloid formation process. Protein Sci (2011) 0.80
Two alternative pathways for generating transmissible prion disease de novo. Acta Neuropathol Commun (2015) 0.80
Disulfide mapping reveals the domain swapping as the crucial process of the structural conversion of prion protein. Prion (2011) 0.80
The diversity and relationship of prion protein self-replicating states. Virus Res (2014) 0.80
Atypical and classical forms of the disease-associated state of the prion protein exhibit distinct neuronal tropism, deposition patterns, and lesion profiles. Am J Pathol (2013) 0.79
A systematic investigation of production of synthetic prions from recombinant prion protein. Open Biol (2015) 0.79
PrP aggregation can be seeded by pre-formed recombinant PrP amyloid fibrils without the replication of infectious prions. Acta Neuropathol (2016) 0.79
The effect of β2-α2 loop mutation on amyloidogenic properties of the prion protein. FEBS Lett (2013) 0.79
Reversible off and on switching of prion infectivity via removing and reinstalling prion sialylation. Sci Rep (2016) 0.79
Intraperitoneal Infection of Wild-Type Mice with Synthetically Generated Mammalian Prion. PLoS Pathog (2015) 0.79
Photodegradation illuminates the role of polyanions in prion infectivity. Prion (2011) 0.78
Amyloid fibrils from the N-terminal prion protein fragment are infectious. Proc Natl Acad Sci U S A (2016) 0.78
N-terminal domain of prion protein directs its oligomeric association. J Biol Chem (2014) 0.78
Progress towards structural understanding of infectious sheep PrP-amyloid. Prion (2014) 0.78
Strain-dependent profile of misfolded prion protein aggregates. Sci Rep (2016) 0.78
Transmission of prions within the gut and towards the central nervous system. Prion (2011) 0.78
De novo prions. F1000 Biol Rep (2010) 0.78
Elongation of mouse prion protein amyloid-like fibrils: effect of temperature and denaturant concentration. PLoS One (2014) 0.78
Novel proteinaceous infectious particles cause scrapie. Science (1982) 24.09
Adapting proteostasis for disease intervention. Science (2008) 13.22
Eight prion strains have PrP(Sc) molecules with different conformations. Nat Med (1998) 9.01
Synthetic mammalian prions. Science (2004) 7.35
Classification of sporadic Creutzfeldt-Jakob disease based on molecular and phenotypic analysis of 300 subjects. Ann Neurol (1999) 6.98
In vitro generation of infectious scrapie prions. Cell (2005) 6.86
Formation of native prions from minimal components in vitro. Proc Natl Acad Sci U S A (2007) 5.94
RNA molecules stimulate prion protein conversion. Nature (2003) 4.06
Pathway complexity of prion protein assembly into amyloid. J Biol Chem (2002) 2.96
A 'unified theory' of prion propagation. Nature (1991) 2.87
In vitro conversion of full-length mammalian prion protein produces amyloid form with physical properties of PrP(Sc). J Mol Biol (2004) 2.59
Species-barrier-independent prion replication in apparently resistant species. Proc Natl Acad Sci U S A (2000) 2.50
Design and construction of diverse mammalian prion strains. Proc Natl Acad Sci U S A (2009) 2.41
PrP expression in B lymphocytes is not required for prion neuroinvasion. Nat Med (1998) 2.40
Strain-specified characteristics of mouse synthetic prions. Proc Natl Acad Sci U S A (2005) 2.25
Natural and synthetic prion structure from X-ray fiber diffraction. Proc Natl Acad Sci U S A (2009) 1.97
Variant CJD in an individual heterozygous for PRNP codon 129. Lancet (2009) 1.84
De novo generation of infectious prions in vitro produces a new disease phenotype. PLoS Pathog (2009) 1.83
The same primary structure of the prion protein yields two distinct self-propagating states. J Biol Chem (2008) 1.64
The stoichiometry of host PrPC glycoforms modulates the efficiency of PrPSc formation in vitro. Biochemistry (2006) 1.56
Annealing prion protein amyloid fibrils at high temperature results in extension of a proteinase K-resistant core. J Biol Chem (2005) 1.44
Subclinical prion infection. Trends Microbiol (2003) 1.36
Conformational switching within individual amyloid fibrils. J Biol Chem (2009) 1.31
Scrapie pathogenesis in subclinically infected B-cell-deficient mice. J Virol (1999) 1.31
Subclinical prion infection in humans and animals. Br Med Bull (2003) 1.25
Scrapie incubation time can exceed natural lifespan. Nature (1975) 1.24
Converting the prion protein: what makes the protein infectious. Biochim Biophys Acta (2006) 1.15
The effects of prion protein proteolysis and disaggregation on the strain properties of hamster scrapie. J Gen Virol (2008) 1.05
Synthetic mammalian prions. Science (2004) 7.35
Nomenclature and nosology for neuropathologic subtypes of frontotemporal lobar degeneration: an update. Acta Neuropathol (2009) 4.73
Nomenclature for neuropathologic subtypes of frontotemporal lobar degeneration: consensus recommendations. Acta Neuropathol (2008) 4.73
Primary age-related tauopathy (PART): a common pathology associated with human aging. Acta Neuropathol (2014) 3.75
Genetic prion disease: the EUROCJD experience. Hum Genet (2005) 2.64
In vitro conversion of full-length mammalian prion protein produces amyloid form with physical properties of PrP(Sc). J Mol Biol (2004) 2.59
Molecular classification of sporadic Creutzfeldt-Jakob disease. Brain (2003) 2.42
Design and construction of diverse mammalian prion strains. Proc Natl Acad Sci U S A (2009) 2.41
Strain-specified characteristics of mouse synthetic prions. Proc Natl Acad Sci U S A (2005) 2.25
Mutations of the prion protein gene phenotypic spectrum. J Neurol (2002) 2.20
Fibulin-5 mutations link inherited neuropathies, age-related macular degeneration and hyperelastic skin. Brain (2011) 2.15
Protease-sensitive synthetic prions. PLoS Pathog (2010) 2.14
Rosette-forming glioneuronal tumor of the fourth ventricle. Acta Neuropathol (2003) 1.95
TARDBP variation associated with frontotemporal dementia, supranuclear gaze palsy, and chorea. Mov Disord (2009) 1.95
Reduction in infectivity of endogenous transmissible spongiform encephalopathies present in blood by adsorption to selective affinity resins. Lancet (2006) 1.77
Effectiveness of leucoreduction for removal of infectivity of transmissible spongiform encephalopathies from blood. Lancet (2004) 1.70
The same primary structure of the prion protein yields two distinct self-propagating states. J Biol Chem (2008) 1.64
Synthetic prions generated in vitro are similar to a newly identified subpopulation of PrPSc from sporadic Creutzfeldt-Jakob Disease. Protein Sci (2005) 1.62
Severe depletion of mitochondrial DNA in spinal muscular atrophy. Acta Neuropathol (2002) 1.60
A peculiar constellation of tau pathology defines a subset of dementia in the elderly. Acta Neuropathol (2011) 1.60
Interlaboratory comparison of assessments of Alzheimer disease-related lesions: a study of the BrainNet Europe Consortium. J Neuropathol Exp Neurol (2006) 1.56
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
A pan-European study of the C9orf72 repeat associated with FTLD: geographic prevalence, genomic instability, and intermediate repeats. Hum Mutat (2013) 1.53
Dopamine transporter imaging in autopsy-confirmed Parkinson's disease and multiple system atrophy. Mov Disord (2011) 1.53
Genesis of mammalian prions: from non-infectious amyloid fibrils to a transmissible prion disease. PLoS Pathog (2011) 1.53
Genetic Creutzfeldt-Jakob disease with R208H mutation presenting as progressive supranuclear palsy. Mov Disord (2012) 1.51
Management of a twenty-first century brain bank: experience in the BrainNet Europe consortium. Acta Neuropathol (2008) 1.51
Endoplasmic reticulum stress features are prominent in Alzheimer disease but not in prion diseases in vivo. J Neuropathol Exp Neurol (2006) 1.49
Amyloid fibrils of mammalian prion protein are highly toxic to cultured cells and primary neurons. J Biol Chem (2006) 1.47
Highly efficient protein misfolding cyclic amplification. PLoS Pathog (2011) 1.46
Polymorphism and ultrastructural organization of prion protein amyloid fibrils: an insight from high resolution atomic force microscopy. J Mol Biol (2006) 1.44
Annealing prion protein amyloid fibrils at high temperature results in extension of a proteinase K-resistant core. J Biol Chem (2005) 1.44
Immunohistochemical analysis of INI1 protein in malignant pediatric CNS tumors: Lack of INI1 in atypical teratoid/rhabdoid tumors and in a fraction of primitive neuroectodermal tumors without rhabdoid phenotype. Am J Surg Pathol (2006) 1.39
Brain protein preservation largely depends on the postmortem storage temperature: implications for study of proteins in human neurologic diseases and management of brain banks: a BrainNet Europe Study. J Neuropathol Exp Neurol (2007) 1.38
Effects of formalin fixation, paraffin embedding, and time of storage on DNA preservation in brain tissue: a BrainNet Europe study. Brain Pathol (2007) 1.36
D18G transthyretin is monomeric, aggregation prone, and not detectable in plasma and cerebrospinal fluid: a prescription for central nervous system amyloidosis? Biochemistry (2003) 1.36
Non-Alzheimer neurodegenerative pathologies and their combinations are more frequent than commonly believed in the elderly brain: a community-based autopsy series. Acta Neuropathol (2013) 1.35
Phenotypic heterogeneity in inherited prion disease (P102L) is associated with differential propagation of protease-resistant wild-type and mutant prion protein. Brain (2006) 1.34
An antibody with high reactivity for disease-associated α-synuclein reveals extensive brain pathology. Acta Neuropathol (2012) 1.33
Conformational switching within individual amyloid fibrils. J Biol Chem (2009) 1.31
Serotonin receptor subtype and p11 mRNA expression in stress-relevant brain regions of suicide and control subjects. J Psychiatry Neurosci (2008) 1.30
Consensus classification of human prion disease histotypes allows reliable identification of molecular subtypes: an inter-rater study among surveillance centres in Europe and USA. Acta Neuropathol (2012) 1.29
JC virus granule cell neuronopathy and GCN-IRIS under natalizumab treatment. Ann Neurol (2013) 1.29
Copper(II) inhibits in vitro conversion of prion protein into amyloid fibrils. Biochemistry (2005) 1.28
Conformational stability of PrP amyloid fibrils controls their smallest possible fragment size. J Mol Biol (2008) 1.27
A new mechanism for transmissible prion diseases. J Neurosci (2012) 1.25
Methionine oxidation interferes with conversion of the prion protein into the fibrillar proteinase K-resistant conformation. Biochemistry (2005) 1.25
Probing the conformation of the prion protein within a single amyloid fibril using a novel immunoconformational assay. J Biol Chem (2006) 1.22
White matter tauopathy with globular glial inclusions: a distinct sporadic frontotemporal lobar degeneration. J Neuropathol Exp Neurol (2008) 1.21
Excretion of transmissible spongiform encephalopathy infectivity in urine. Emerg Infect Dis (2008) 1.21
Visualization of Central European tick-borne encephalitis infection in fatal human cases. J Neuropathol Exp Neurol (2005) 1.20
Neuropathology of white matter disease in Leber's hereditary optic neuropathy. Brain (2004) 1.17
PART, a distinct tauopathy, different from classical sporadic Alzheimer disease. Acta Neuropathol (2015) 1.17
Mixed brain pathologies in dementia: the BrainNet Europe consortium experience. Dement Geriatr Cogn Disord (2008) 1.17
Two amyloid States of the prion protein display significantly different folding patterns. J Mol Biol (2010) 1.16
Site-specific conformational studies of prion protein (PrP) amyloid fibrils revealed two cooperative folding domains within amyloid structure. J Biol Chem (2007) 1.14
Inter-laboratory comparison of neuropathological assessments of beta-amyloid protein: a study of the BrainNet Europe consortium. Acta Neuropathol (2008) 1.13
Stabilization of a prion strain of synthetic origin requires multiple serial passages. J Biol Chem (2012) 1.13
Highly promiscuous nature of prion polymerization. J Biol Chem (2007) 1.12
Immunohistochemistry for the prion protein: comparison of different monoclonal antibodies in human prion disease subtypes. Brain Pathol (2002) 1.12
Optical coherence tomography in the diagnosis and treatment of neurological disorders. J Biomed Opt (2005) 1.11
Vascular patterns in glioblastoma influence clinical outcome and associate with variable expression of angiogenic proteins: evidence for distinct angiogenic subtypes. Brain Pathol (2003) 1.11
Subcellular localization of disease-associated prion protein in the human brain. Am J Pathol (2005) 1.11
14-3-3 proteins in Lewy bodies in Parkinson disease and diffuse Lewy body disease brains. J Neuropathol Exp Neurol (2002) 1.11
Relationship between conformational stability and amplification efficiency of prions. Biochemistry (2011) 1.11
Cerebrospinal fluid collection tubes: a critical issue for Alzheimer disease diagnosis. Clin Chem (2012) 1.11
The monomer/dimer transition of enzyme I of the Escherichia coli phosphotransferase system. J Biol Chem (2006) 1.10
Ki-67 immunolabeling index is an accurate predictor of outcome in patients with intracranial ependymoma. Am J Surg Pathol (2004) 1.10
Genetic Creutzfeldt-Jakob disease associated with the E200K mutation: characterization of a complex proteinopathy. Acta Neuropathol (2010) 1.10
Assessment of alpha-synuclein pathology: a study of the BrainNet Europe Consortium. J Neuropathol Exp Neurol (2008) 1.08
Spontaneous mutations in the prion protein gene causing transmissible spongiform encephalopathy. Ann Neurol (2002) 1.07
Peroxisomal alterations in Alzheimer's disease. Acta Neuropathol (2011) 1.06