Published in Nature on October 02, 1997
Prions. Proc Natl Acad Sci U S A (1998) 27.80
Human BSE. Nature (1997) 11.79
Compelling transgenetic evidence for transmission of bovine spongiform encephalopathy prions to humans. Proc Natl Acad Sci U S A (1999) 3.43
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
New variant Creutzfeldt-Jakob disease: the epidemic that never was. BMJ (2001) 3.25
BSE prions propagate as either variant CJD-like or sporadic CJD-like prion strains in transgenic mice expressing human prion protein. EMBO J (2002) 2.98
Distinct molecular phenotypes in bovine prion diseases. EMBO Rep (2004) 2.90
Bovine spongiform encephalopathy and variant Creutzfeldt-Jakob disease: background, evolution, and current concerns. Emerg Infect Dis (2001) 2.62
NMR structure of the bovine prion protein. Proc Natl Acad Sci U S A (2000) 2.56
Species-barrier-independent prion replication in apparently resistant species. Proc Natl Acad Sci U S A (2000) 2.50
Genetic influence on the structural variations of the abnormal prion protein. Proc Natl Acad Sci U S A (2000) 2.46
Evidence of a molecular barrier limiting susceptibility of humans, cattle and sheep to chronic wasting disease. EMBO J (2000) 2.42
The prion hypothesis: from biological anomaly to basic regulatory mechanism. Nat Rev Mol Cell Biol (2010) 2.22
Bovine spongiform encephalopathy and variant Creutzfeldt-Jakob disease. BMJ (2001) 2.17
Molecular neurology of prion disease. J Neurol Neurosurg Psychiatry (2005) 2.15
Population screening for variant Creutzfeldt-Jakob disease using a novel blood test: diagnostic accuracy and feasibility study. JAMA Neurol (2014) 2.08
Rapid prion neuroinvasion following tongue infection. J Virol (2003) 2.06
Abbreviated incubation times for human prions in mice expressing a chimeric mouse-human prion protein transgene. Proc Natl Acad Sci U S A (2003) 2.01
Conversion of the BASE prion strain into the BSE strain: the origin of BSE? PLoS Pathog (2007) 1.97
Identification of multiple quantitative trait loci linked to prion disease incubation period in mice. Proc Natl Acad Sci U S A (2001) 1.96
Genetic risk factors for variant Creutzfeldt-Jakob disease: a genome-wide association study. Lancet Neurol (2009) 1.94
The paraffin-embedded tissue blot detects PrP(Sc) early in the incubation time in prion diseases. Am J Pathol (2000) 1.91
Infectious prions in pre-clinical deer and transmission of chronic wasting disease solely by environmental exposure. PLoS One (2009) 1.90
Accelerated high fidelity prion amplification within and across prion species barriers. PLoS Pathog (2008) 1.89
Chronic wasting disease and potential transmission to humans. Emerg Infect Dis (2004) 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
Detection of type 1 prion protein in variant Creutzfeldt-Jakob disease. Am J Pathol (2006) 1.80
Rapid and discriminatory diagnosis of scrapie and BSE in retro-pharyngeal lymph nodes of sheep. BMC Vet Res (2006) 1.77
Chronic wasting disease prions in elk antler velvet. Emerg Infect Dis (2009) 1.77
A naturally occurring variant of the human prion protein completely prevents prion disease. Nature (2015) 1.76
Prions in the urine of patients with variant Creutzfeldt-Jakob disease. N Engl J Med (2014) 1.74
Early spread of scrapie from the gastrointestinal tract to the central nervous system involves autonomic fibers of the splanchnic and vagus nerves. J Virol (2001) 1.73
Elimination of prions by branched polyamines and implications for therapeutics. Proc Natl Acad Sci U S A (1999) 1.73
Interactions between heterologous forms of prion protein: binding, inhibition of conversion, and species barriers. Proc Natl Acad Sci U S A (2000) 1.73
Efficient transmission and characterization of Creutzfeldt-Jakob disease strains in bank voles. PLoS Pathog (2006) 1.70
Adaptation and selection of prion protein strain conformations following interspecies transmission of transmissible mink encephalopathy. J Virol (2000) 1.66
Epidemiological determinants of the pattern and magnitude of the vCJD epidemic in Great Britain. Proc Biol Sci (1998) 1.64
Discrimination between scrapie and bovine spongiform encephalopathy in sheep by molecular size, immunoreactivity, and glycoprofile of prion protein. J Clin Microbiol (2004) 1.60
Disease dynamics over very different time-scales: foot-and-mouth disease and scrapie on the network of livestock movements in the UK. J R Soc Interface (2007) 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
Isolation from cattle of a prion strain distinct from that causing bovine spongiform encephalopathy. PLoS Pathog (2006) 1.55
Defining sporadic Creutzfeldt-Jakob disease strains and their transmission properties. Proc Natl Acad Sci U S A (2010) 1.51
Sporadic--but not variant--Creutzfeldt-Jakob disease is associated with polymorphisms upstream of PRNP exon 1. Am J Hum Genet (2001) 1.50
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
Cellular biology of prion diseases. Clin Microbiol Rev (1999) 1.48
Chronic subclinical prion disease induced by low-dose inoculum. J Virol (2002) 1.47
Adaptation of the bovine spongiform encephalopathy agent to primates and comparison with Creutzfeldt-- Jakob disease: implications for human health. Proc Natl Acad Sci U S A (2001) 1.46
The expanding universe of prion diseases. PLoS Pathog (2006) 1.45
Possible underascertainment of variant Creutzfeldt-Jakob disease: a systematic study. J Neurol Neurosurg Psychiatry (2002) 1.44
Analysis of sequence variability of the bovine prion protein gene (PRNP) in German cattle breeds. Neurogenetics (2004) 1.43
Transmission barriers for bovine, ovine, and human prions in transgenic mice. J Virol (2005) 1.43
Analysis of prion strains by PrPSc profiling in sporadic Creutzfeldt-Jakob disease. PLoS Med (2005) 1.43
Antibody to DNA detects scrapie but not normal prion protein. Proc Natl Acad Sci U S A (2004) 1.43
Prions in milk from ewes incubating natural scrapie. PLoS Pathog (2008) 1.43
A molecular switch controls interspecies prion disease transmission in mice. J Clin Invest (2010) 1.41
Characterization of truncated forms of abnormal prion protein in Creutzfeldt-Jakob disease. J Biol Chem (2008) 1.40
The prion strain phenomenon: molecular basis and unprecedented features. Biochim Biophys Acta (2006) 1.39
Tetracyclines affect prion infectivity. Proc Natl Acad Sci U S A (2002) 1.39
Sheep and goat BSE propagate more efficiently than cattle BSE in human PrP transgenic mice. PLoS Pathog (2011) 1.38
Atypical scrapie prions from sheep and lack of disease in transgenic mice overexpressing human prion protein. Emerg Infect Dis (2013) 1.38
Follicular dendritic cell dedifferentiation by treatment with an inhibitor of the lymphotoxin pathway dramatically reduces scrapie susceptibility. J Virol (2003) 1.37
Kuru prions and sporadic Creutzfeldt-Jakob disease prions have equivalent transmission properties in transgenic and wild-type mice. Proc Natl Acad Sci U S A (2008) 1.37
Phenotypic similarity of transmissible mink encephalopathy in cattle and L-type bovine spongiform encephalopathy in a mouse model. Emerg Infect Dis (2007) 1.37
Motif-grafted antibodies containing the replicative interface of cellular PrP are specific for PrPSc. Proc Natl Acad Sci U S A (2004) 1.37
Tumor necrosis factor alpha-deficient, but not interleukin-6-deficient, mice resist peripheral infection with scrapie. J Virol (2000) 1.34
Scrapie infections initiated at varying doses: an analysis of 117 titration experiments. Philos Trans R Soc Lond B Biol Sci (2000) 1.34
Updated projections of future vCJD deaths in the UK. BMC Infect Dis (2003) 1.33
PrPTSE distribution in a primate model of variant, sporadic, and iatrogenic Creutzfeldt-Jakob disease. J Virol (2005) 1.33
Coinfecting prion strains compete for a limiting cellular resource. J Virol (2010) 1.32
Widespread PrPSc accumulation in muscles of hamsters orally infected with scrapie. EMBO Rep (2003) 1.31
Highly efficient prion transmission by blood transfusion. PLoS Pathog (2012) 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
Molecular analysis of the abnormal prion protein during coinfection of mice by bovine spongiform encephalopathy and a scrapie agent. J Virol (2001) 1.28
A descriptive study of the prevalence of atypical and classical scrapie in sheep in 20 European countries. BMC Vet Res (2008) 1.27
Neuropathology of non-Alzheimer degenerative disorders. Int J Clin Exp Pathol (2009) 1.27
Atypical/Nor98 scrapie infectivity in sheep peripheral tissues. PLoS Pathog (2011) 1.26
Specificity of lymphoreticular accumulation of prion protein for variant Creutzfeldt-Jakob disease. J Clin Pathol (2004) 1.26
Sheep-passaged bovine spongiform encephalopathy agent exhibits altered pathobiological properties in bovine-PrP transgenic mice. J Virol (2006) 1.25
Estimation of the basic reproduction number of BSE: the intensity of transmission in British cattle. Proc Biol Sci (1999) 1.25
Early identification of variant Creutzfeldt-Jakob disease. BMJ (1998) 1.23
Intraspecies transmission of BASE induces clinical dullness and amyotrophic changes. PLoS Pathog (2008) 1.23
Decision support tools for clinical diagnosis of disease in cows with suspected bovine spongiform encephalopathy. J Clin Microbiol (2004) 1.23
Demographic risk factors for classical and atypical scrapie in Great Britain. J Gen Virol (2007) 1.22
Dissociation of pathological and molecular phenotype of variant Creutzfeldt-Jakob disease in transgenic human prion protein 129 heterozygous mice. Proc Natl Acad Sci U S A (2006) 1.22
Oral prion infection requires normal numbers of Peyer's patches but not of enteric lymphocytes. Am J Pathol (2003) 1.21
Molecular analysis of the protease-resistant prion protein in scrapie and bovine spongiform encephalopathy transmitted to ovine transgenic and wild-type mice. J Virol (2004) 1.20
Similar signature of the prion protein in natural sheep scrapie and bovine spongiform encephalopathy-linked diseases. J Clin Microbiol (1999) 1.19
Chronic wasting disease prions are not transmissible to transgenic mice overexpressing human prion protein. J Gen Virol (2010) 1.19
Variant Creutzfeldt-Jakob disease: a summary of current scientific knowledge in relation to public health. CMAJ (2001) 1.18
Ultra-efficient PrP(Sc) amplification highlights potentialities and pitfalls of PMCA technology. PLoS Pathog (2011) 1.17
Florid plaques in ovine PrP transgenic mice infected with an experimental ovine BSE. EMBO Rep (2001) 1.17
Influence of the N-terminal domain on the aggregation properties of the prion protein. Protein Sci (2005) 1.16
Review. Lessons of kuru research: background to recent studies with some personal reflections. Philos Trans R Soc Lond B Biol Sci (2008) 1.15
Molecular, biochemical and genetic characteristics of BSE in Canada. PLoS One (2010) 1.15
Emergence of classical BSE strain properties during serial passages of H-BSE in wild-type mice. PLoS One (2011) 1.15
PET-blot analysis contributes to BSE strain recognition in C57Bl/6 mice. J Histochem Cytochem (2006) 1.15
Informed consent should be obtained from patients to use products (skin substitutes) and dressings containing biological material. J Med Ethics (2005) 1.14
Pathogenesis of bovine spongiform encephalopathy in sheep. Arch Virol (2007) 1.14
Genome-wide association study in multiple human prion diseases suggests genetic risk factors additional to PRNP. Hum Mol Genet (2011) 1.12
Prevalence of the prion protein gene E211K variant in U.S. cattle. BMC Vet Res (2008) 1.12
A new variant of Creutzfeldt-Jakob disease in the UK. Lancet (1996) 20.15
The prevalence of hypertension in seven populations of west African origin. Am J Public Health (1997) 5.55
Transmission of BSE by blood transfusion in sheep. Lancet (2000) 5.16
Updated clinical diagnostic criteria for sporadic Creutzfeldt-Jakob disease. Brain (2009) 5.03
Possible transmission of variant Creutzfeldt-Jakob disease by blood transfusion. Lancet (2004) 4.85
Phenotypic differentiation of Streptococcus intermedius, Streptococcus constellatus, and Streptococcus anginosus strains within the "Streptococcus milleri group". J Clin Microbiol (1990) 4.03
Investigation of variant Creutzfeldt-Jakob disease and other human prion diseases with tonsil biopsy samples. Lancet (1999) 4.02
Using serial registered brain magnetic resonance imaging to measure disease progression in Alzheimer disease: power calculations and estimates of sample size to detect treatment effects. Arch Neurol (2000) 3.99
"Friendly fire" in medicine: hormones, homografts, and Creutzfeldt-Jakob disease. Lancet (1992) 3.74
129/Ola mice carrying a null mutation in PrP that abolishes mRNA production are developmentally normal. Mol Neurobiol (1995) 3.60
The sequential development of the brain lesion of scrapie in three strains of mice. J Comp Pathol (1968) 3.60
The disease characteristics of different strains of scrapie in Sinc congenic mouse lines: implications for the nature of the agent and host control of pathogenesis. J Gen Virol (1991) 3.47
A community-based programme to provide prompt and adequate treatment of presumptive malaria in children. Trans R Soc Trop Med Hyg (1998) 3.42
Geographical distribution of variant CJD in the UK (excluding Northern Ireland). Lancet (1999) 3.39
Identification of a gene which controls the incubation period of some strains of scrapie agent in mice. J Comp Pathol (1968) 3.31
Predicting susceptibility and incubation time of human-to-human transmission of vCJD. Lancet Neurol (2006) 3.29
DNA synthesis in the fission yeast Schizosaccharomyces pombe. Exp Cell Res (1970) 3.29
Iatrogenic Creutzfeldt-Jakob disease at the millennium. Neurology (2000) 3.23
Alternatives to randomisation in the evaluation of public health interventions: design challenges and solutions. J Epidemiol Community Health (2009) 3.15
Prion immunoreactivity in appendix before clinical onset of variant Creutzfeldt-Jakob disease. Lancet (1998) 3.08
Predicting the CJD epidemic in humans. Nature (1997) 3.04
Analysis of EEG and CSF 14-3-3 proteins as aids to the diagnosis of Creutzfeldt-Jakob disease. Neurology (2000) 3.03
Studies on actively allergized cells. I. The cyto-dynamics and morphology of rosete-forming lymph node cells in mice and inhibition of rosette-formation with antibody to mouse immunoglobulins. Int Arch Allergy Appl Immunol (1969) 3.00
Epidemic of fatal encephalopathy in preschool children in Burkina Faso and consumption of unripe ackee (Blighia sapida) fruit. Lancet (1999) 2.98
Creutzfeldt-Jakob disease and blood transfusion. Lancet (1993) 2.97
The pulvinar sign on magnetic resonance imaging in variant Creutzfeldt-Jakob disease. Lancet (2000) 2.92
N-WASP deficiency reveals distinct pathways for cell surface projections and microbial actin-based motility. Nat Cell Biol (2001) 2.91
Molecular assessment of the potential transmissibilities of BSE and scrapie to humans. Nature (1997) 2.80
Transmission of bovine spongiform encephalopathy and scrapie to mice: strain variation and the species barrier. Philos Trans R Soc Lond B Biol Sci (1994) 2.76
A retrospective study of Creutzfeldt-Jakob disease in England and Wales 1970-79. I: Clinical features. J Neurol Neurosurg Psychiatry (1984) 2.68
The genomic identity of different strains of mouse scrapie is expressed in hamsters and preserved on reisolation in mice. J Gen Virol (1989) 2.64
PrP genotype and agent effects in scrapie: change in allelic interaction with different isolates of agent in sheep, a natural host of scrapie. J Gen Virol (1994) 2.63
Bovine spongiform encephalopathy and variant Creutzfeldt-Jakob disease: background, evolution, and current concerns. Emerg Infect Dis (2001) 2.62
Retrospective study of prion-protein accumulation in tonsil and appendix tissues. Lancet (2000) 2.59
Determinants of diagnostic investigation sensitivities across the clinical spectrum of sporadic Creutzfeldt-Jakob disease. Brain (2006) 2.54
Evaluation of a nurse-run hypertension clinic in general practice. Practitioner (1988) 2.49
Relationship between spectral components of cardiovascular variabilities and direct measures of muscle sympathetic nerve activity in humans. Circulation (1997) 2.49
Evidence of behaviour change following a hygiene promotion programme in Burkina Faso. Bull World Health Organ (2001) 2.47
Distribution and aetiology of blindness and visual impairment in mesoendemic onchocercal communities, Kaduna State, Nigeria. Kaduna Collaboration for Research on Onchocerciasis. Br J Ophthalmol (1994) 2.47
Genetic influence on the structural variations of the abnormal prion protein. Proc Natl Acad Sci U S A (2000) 2.46
Diagnosis of new variant Creutzfeldt-Jakob disease. Ann Neurol (2000) 2.42
Inhibition of the late sodium current as a potential cardioprotective principle: effects of the late sodium current inhibitor ranolazine. Heart (2006) 2.33
The role of cerebrospinal fluid 14-3-3 and other proteins in the diagnosis of sporadic Creutzfeldt-Jakob disease in the UK: a 10-year review. J Neurol Neurosurg Psychiatry (2010) 2.29
Distribution of cell-associated prion protein in normal adult blood determined by flow cytometry. Br J Haematol (1999) 2.29
Scrapie in mice. Agent-strain differences in the distribution and intensity of grey matter vacuolation. J Comp Pathol (1973) 2.28
Mice with gene targetted prion protein alterations show that Prnp, Sinc and Prni are congruent. Nat Genet (1998) 2.23
PrP gene dosage determines the timing but not the final intensity or distribution of lesions in scrapie pathology. Neurodegeneration (1994) 2.21
Structured observations of hygiene behaviours in Burkina Faso: validity, variability, and utility. Bull World Health Organ (1993) 2.19
Evidence for case-to-case transmission of Creutzfeldt-Jakob disease. J Neurol Neurosurg Psychiatry (1982) 2.15
Scrapie replication in lymphoid tissues depends on prion protein-expressing follicular dendritic cells. Nat Med (1999) 2.14
Natural scrapie in a closed flock of Cheviot sheep occurs only in specific PrP genotypes. Arch Virol (1996) 2.14
Neuropathological diagnostic criteria for Creutzfeldt-Jakob disease (CJD) and other human spongiform encephalopathies (prion diseases). Brain Pathol (1995) 2.10
Lymphocyte receptors. II. Receptors for rabbit IgM on human T lymphocytes. Immunology (1976) 2.06
Deaths from variant Creutzfeldt-Jakob disease. Lancet (1999) 2.04
Risk reduction strategies for variant Creutzfeldt-Jakob disease transmission by UK plasma products and their impact on patients with inherited bleeding disorders. Haemophilia (2010) 2.03
High frequency of apolipoprotein E epsilon 2 allele in hemorrhage due to cerebral amyloid angiopathy. Ann Neurol (1997) 2.00
Standardization of blood pressure measurement in an international comparative study. J Clin Epidemiol (1996) 1.99
Measuring hygiene practices: a comparison of questionnaires with direct observations in rural Zaïre. Trop Med Int Health (1997) 1.99
CD4+ T-lymphocyte counts in HIV infection: are European standards applicable to African patients? J Acquir Immune Defic Syndr Hum Retrovirol (1997) 1.99
Geographical distribution of variant Creutzfeldt-Jakob disease in Great Britain, 1994-2000. Lancet (2001) 1.97
Transmission of bovine spongiform encephalopathy to sheep and goats. Vet Rec (1993) 1.97
Characterization of sheep afferent lymph dendritic cells and their role in antigen carriage. J Exp Med (1989) 1.97
A single amino acid alteration (101L) introduced into murine PrP dramatically alters incubation time of transmissible spongiform encephalopathy. EMBO J (1999) 1.96
Predictors of survival in sporadic Creutzfeldt-Jakob disease and other human transmissible spongiform encephalopathies. Brain (2004) 1.96
Potties, pits and pipes: explaining hygiene behaviour in Burkina Faso. Soc Sci Med (1995) 1.95
Transmission of bovine spongiform encephalopathy and scrapie to mice. J Gen Virol (1992) 1.90
Molecular composition of mitochondrial ATP-sensitive potassium channels probed by viral Kir gene transfer. J Mol Cell Cardiol (2000) 1.88
Mortality from Creutzfeldt-Jakob disease and related disorders in Europe, Australia, and Canada. Neurology (2005) 1.88
Different forms of the bovine PrP gene have five or six copies of a short, G-C-rich element within the protein-coding exon. J Gen Virol (1991) 1.88
The prion protein gene: a role in mouse embryogenesis? Development (1992) 1.87
Creutzfeldt-Jakob disease in England and Wales, 1980-1984: a case-control study of potential risk factors. J Neurol Neurosurg Psychiatry (1988) 1.87
Two alleles of a neural protein gene linked to scrapie in sheep. Proc Natl Acad Sci U S A (1990) 1.84
Detection of variant Creutzfeldt-Jakob disease infectivity in extraneural tissues. Lancet (2001) 1.84
An evaluation of the double thymidine block for synchronizing mammalian cells at the G1-S border. Exp Cell Res (1971) 1.83
PrP protein is associated with follicular dendritic cells of spleens and lymph nodes in uninfected and scrapie-infected mice. J Pathol (1992) 1.79
Creutzfeldt-Jakob disease and blood transfusion: results of the UK Transfusion Medicine Epidemiological Review study. Vox Sang (2006) 1.76
Genetic apparatus of Stylonychia sp. Nature (1973) 1.72
New variant Creutzfeldt-Jakob disease: neurological features and diagnostic tests. Lancet (1997) 1.70
Homologous subfamilies of human alphoid repetitive DNA on different nucleolus organizing chromosomes. Proc Natl Acad Sci U S A (1987) 1.69
Genetic and environmental factors modify bovine spongiform encephalopathy incubation period in mice. Proc Natl Acad Sci U S A (2001) 1.69
Amount of research interest in rare and common neurological conditions: bibliometric study. BMJ (2001) 1.68