Published in PLoS Pathog on March 08, 2016
Preclinical vCJD after blood transfusion in a PRNP codon 129 heterozygous patient. Lancet (2004) 4.59
Ultrasensitive detection of scrapie prion protein using seeded conversion of recombinant prion protein. Nat Methods (2007) 3.74
A test for Creutzfeldt-Jakob disease using nasal brushings. N Engl J Med (2014) 3.58
Identification of a second bovine amyloidotic spongiform encephalopathy: molecular similarities with sporadic Creutzfeldt-Jakob disease. Proc Natl Acad Sci U S A (2004) 3.29
Prevalent abnormal prion protein in human appendixes after bovine spongiform encephalopathy epizootic: large scale survey. BMJ (2013) 2.74
Simplified ultrasensitive prion detection by recombinant PrP conversion with shaking. Nat Methods (2008) 2.56
Prevalence of lymphoreticular prion protein accumulation in UK tissue samples. J Pathol (2004) 2.47
Prion protein heterogeneity in sporadic but not variant Creutzfeldt-Jakob disease: UK cases 1991-2002. Ann Neurol (2004) 2.44
Molecular classification of sporadic Creutzfeldt-Jakob disease. Brain (2003) 2.42
Mutations of the prion protein gene phenotypic spectrum. J Neurol (2002) 2.20
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Validation of diagnostic criteria for variant Creutzfeldt-Jakob disease. Ann Neurol (2010) 2.09
Diagnosing variant Creutzfeldt-Jakob disease with the pulvinar sign: MR imaging findings in 86 neuropathologically confirmed cases. AJNR Am J Neuroradiol (2003) 2.05
Conversion of the BASE prion strain into the BSE strain: the origin of BSE? PLoS Pathog (2007) 1.97
Detection of type 1 prion protein in variant Creutzfeldt-Jakob disease. Am J Pathol (2006) 1.80
Prevalence of disease related prion protein in anonymous tonsil specimens in Britain: cross sectional opportunistic survey. BMJ (2009) 1.75
Progressive multifocal leukoencephalopathy in a patient with Good's syndrome. Int J Infect Dis (2009) 1.67
Rabbits are not resistant to prion infection. Proc Natl Acad Sci U S A (2012) 1.63
Nonpsychoactive cannabidiol prevents prion accumulation and protects neurons against prion toxicity. J Neurosci (2007) 1.61
Nodule formation and desmoplasia in medulloblastomas-defining the nodular/desmoplastic variant and its biological behavior. Brain Pathol (2007) 1.59
Evaluation of the human transmission risk of an atypical bovine spongiform encephalopathy prion strain. J Virol (2008) 1.59
Atypical BSE (BASE) transmitted from asymptomatic aging cattle to a primate. PLoS One (2008) 1.56
Peripheral tissue involvement in sporadic, iatrogenic, and variant Creutzfeldt-Jakob disease: an immunohistochemical, quantitative, and biochemical study. Am J Pathol (2004) 1.56
Prion protein accumulation and neuroprotection in hypoxic brain damage. Am J Pathol (2004) 1.52
Management of a twenty-first century brain bank: experience in the BrainNet Europe consortium. Acta Neuropathol (2008) 1.51
Immunohistochemical localization of aspartoacylase in the rat central nervous system. J Comp Neurol (2004) 1.47
A multivariate analysis of factors determining tumor progression in childhood low-grade glioma: a population-based cohort study (CCLG CNS9702). Neuro Oncol (2010) 1.46
Inherited prion disease with 4-octapeptide repeat insertion: disease requires the interaction of multiple genetic risk factors. Brain (2011) 1.45
Global democratic consensus on neuropathological disease criteria. Lancet Neurol (2002) 1.44
Accumulation of prion protein in tonsil and appendix: review of tissue samples. BMJ (2002) 1.44
Prion protein accumulation in eyes of patients with sporadic and variant Creutzfeldt-Jakob disease. Invest Ophthalmol Vis Sci (2003) 1.42
Sensitive and specific detection of sporadic Creutzfeldt-Jakob disease brain prion protein using real-time quaking-induced conversion. J Gen Virol (2011) 1.40
The Distribution of Prion Protein Allotypes Differs Between Sporadic and Iatrogenic Creutzfeldt-Jakob Disease Patients. PLoS Pathog (2016) 1.40
Tetracyclines affect prion infectivity. Proc Natl Acad Sci U S A (2002) 1.39
Transplantation of ocular tissue from a donor with sporadic Creutzfeldt-Jakob disease. Clin Experiment Ophthalmol (2006) 1.39
Multiple amino acid residues within the rabbit prion protein inhibit formation of its abnormal isoform. J Virol (2003) 1.35
Susceptibilities of nonhuman primates to chronic wasting disease. Emerg Infect Dis (2009) 1.33
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
Variant Creutzfeldt-Jakob disease: prion protein genotype analysis of positive appendix tissue samples from a retrospective prevalence study. BMJ (2006) 1.28
Assessment of beta-amyloid deposits in human brain: a study of the BrainNet Europe Consortium. Acta Neuropathol (2009) 1.27
Detection and localization of PrPSc in the skeletal muscle of patients with variant, iatrogenic, and sporadic forms of Creutzfeldt-Jakob disease. Am J Pathol (2006) 1.26
Susceptibility of common fibroblast cell lines to transmissible spongiform encephalopathy agents. J Infect Dis (2004) 1.25
On the question of sporadic or atypical bovine spongiform encephalopathy and Creutzfeldt-Jakob disease. Emerg Infect Dis (2006) 1.25
Intraspecies transmission of BASE induces clinical dullness and amyotrophic changes. PLoS Pathog (2008) 1.23
Acute formation of protease-resistant prion protein does not always lead to persistent scrapie infection in vitro. J Biol Chem (2004) 1.21
PRNP contains both intronic and upstream regulatory regions that may influence susceptibility to Creutzfeldt-Jakob Disease. Gene (2002) 1.17
Staging/typing of Lewy body related alpha-synuclein pathology: a study of the BrainNet Europe Consortium. Acta Neuropathol (2009) 1.17
Humanized knock-in mice expressing chimeric prion protein showed varied susceptibility to different human prions. Am J Pathol (2003) 1.16
Molecular basis of scrapie strain glycoform variation. J Biol Chem (2002) 1.16
Beyond PrP res type 1/type 2 dichotomy in Creutzfeldt-Jakob disease. PLoS Pathog (2008) 1.14
Immunohistochemistry for the prion protein: comparison of different monoclonal antibodies in human prion disease subtypes. Brain Pathol (2002) 1.12
Acute cellular uptake of abnormal prion protein is cell type and scrapie-strain independent. Virology (2008) 1.10
Cells expressing anchorless prion protein are resistant to scrapie infection. J Virol (2009) 1.07
Activation of the innate signaling molecule MAVS by bunyavirus infection upregulates the adaptor protein SARM1, leading to neuronal death. Immunity (2013) 1.07
Variant Creutzfeldt-Jakob disease in France and the United Kingdom: Evidence for the same agent strain. Ann Neurol (2009) 1.07
Association of a presenilin 1 S170F mutation with a novel Alzheimer disease molecular phenotype. Arch Neurol (2007) 1.07
Octapeptide repeat insertions increase the rate of protease-resistant prion protein formation. Protein Sci (2006) 1.07
Prion protein misfolding and disease. Curr Opin Struct Biol (2009) 1.06
Heparin enhances the cell-protein misfolding cyclic amplification efficiency of variant Creutzfeldt-Jakob disease. Neurosci Lett (2011) 1.05
Human prion protein (PrP) 219K is converted to PrPSc but shows heterozygous inhibition in variant Creutzfeldt-Jakob disease infection. J Biol Chem (2008) 1.04
Glioblastoma-derived spheroid cultures as an experimental model for analysis of EGFR anomalies. J Neurooncol (2010) 1.03
Association of an 11-12 kDa protease-resistant prion protein fragment with subtypes of dura graft-associated Creutzfeldt-Jakob disease and other prion diseases. J Gen Virol (2003) 1.03
Human platelets as a substrate source for the in vitro amplification of the abnormal prion protein (PrP) associated with variant Creutzfeldt-Jakob disease. Transfusion (2008) 1.03
Correlative studies support lipid peroxidation is linked to PrP(res) propagation as an early primary pathogenic event in prion disease. Brain Res Bull (2005) 1.03
A structural basis for the mechanism of aspartate-beta-semialdehyde dehydrogenase from Vibrio cholerae. Protein Sci (2003) 1.02
Flexible N-terminal region of prion protein influences conformation of protease-resistant prion protein isoforms associated with cross-species scrapie infection in vivo and in vitro. J Biol Chem (2004) 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
Capture of an intermediate in the catalytic cycle of L-aspartate-beta-semialdehyde dehydrogenase. Proc Natl Acad Sci U S A (2003) 1.01
Variably protease-sensitive prionopathy in a PRNP codon 129 heterozygous UK patient with co-existing tau, α synuclein and Aβ pathology. Acta Neuropathol (2010) 0.99
Comparative profiling of highly enriched 22L and Chandler mouse scrapie prion protein preparations. Proteomics (2010) 0.99
Creutzfeldt-Jakob disease. Adv Exp Med Biol (2012) 0.99
Effects of human PrPSc type and PRNP genotype in an in-vitro conversion assay. Neuroreport (2008) 0.98
DNA aptamers that bind to PrP(C) and not PrP(Sc) show sequence and structure specificity. Exp Biol Med (Maywood) (2006) 0.98
Redox metals and oxidative abnormalities in human prion diseases. Acta Neuropathol (2005) 0.98
Brain banking for neurological disorders. Lancet Neurol (2013) 0.97
Prion deposition in olfactory biopsy of sporadic Creutzfeldt-Jakob disease. Ann Neurol (2004) 0.97
Proteome analysis in the clinical chemistry laboratory: myth or reality? Clin Chim Acta (2005) 0.97
Neuroinvasion of the 263K scrapie strain after intranasal administration occurs through olfactory-unrelated pathways. Acta Neuropathol (2008) 0.97
Endocytosis of prion protein is required for ERK1/2 signaling induced by stress-inducible protein 1. J Neurosci (2008) 0.96
vCJD prion acquires altered virulence through trans-species infection. Biochem Biophys Res Commun (2006) 0.96
Assessment of outcome predictors in first-episode acute myelitis: a retrospective study of 53 cases. Arch Neurol (2010) 0.96
Inter-laboratory assessment of PrPSc typing in creutzfeldt-jakob disease: a Western blot study within the NeuroPrion Consortium. Brain Pathol (2008) 0.95