Published in Nature on July 25, 1991
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
A kinetic model for amyloid formation in the prion diseases: importance of seeding. Proc Natl Acad Sci U S A (1993) 2.63
Genetic influence on the structural variations of the abnormal prion protein. Proc Natl Acad Sci U S A (2000) 2.46
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
Ex vivo propagation of infectious sheep scrapie agent in heterologous epithelial cells expressing ovine prion protein. Proc Natl Acad Sci U S A (2001) 2.16
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
The genetic epidemiology of neurodegenerative disease. J Clin Invest (2005) 2.08
Prion protein NMR structure and familial human spongiform encephalopathies. Proc Natl Acad Sci U S A (1998) 2.02
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
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
Entry versus blockade of brain infection following oral or intraperitoneal scrapie administration: role of prion protein expression in peripheral nerves and spleen. J Virol (2000) 1.93
A single hamster PrP amino acid blocks conversion to protease-resistant PrP in scrapie-infected mouse neuroblastoma cells. J Virol (1995) 1.87
A naturally occurring variant of the human prion protein completely prevents prion disease. Nature (2015) 1.76
Prevalence of disease related prion protein in anonymous tonsil specimens in Britain: cross sectional opportunistic survey. BMJ (2009) 1.75
Genetic and environmental factors modify bovine spongiform encephalopathy incubation period in mice. Proc Natl Acad Sci U S A (2001) 1.69
The myth of maternal transmission of spongiform encephalopathy. BMJ (1995) 1.63
Sporadic--but not variant--Creutzfeldt-Jakob disease is associated with polymorphisms upstream of PRNP exon 1. Am J Hum Genet (2001) 1.50
How now mad cow? BMJ (1992) 1.49
Endogenous proteolytic cleavage of normal and disease-associated isoforms of the human prion protein in neural and non-neural tissues. Am J Pathol (1998) 1.48
Inactivation of prions by acidic sodium dodecyl sulfate. J Virol (2006) 1.43
Phenotype-genotype studies in kuru: implications for new variant Creutzfeldt-Jakob disease. Proc Natl Acad Sci U S A (1998) 1.43
The Distribution of Prion Protein Allotypes Differs Between Sporadic and Iatrogenic Creutzfeldt-Jakob Disease Patients. PLoS Pathog (2016) 1.40
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
Sheep and goat BSE propagate more efficiently than cattle BSE in human PrP transgenic mice. PLoS Pathog (2011) 1.38
A major genetic component of BSE susceptibility. BMC Biol (2006) 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
Etiology and pathogenesis of prion diseases. Am J Pathol (1995) 1.35
Mutation and polymorphism of the prion protein gene in Libyan Jews with Creutzfeldt-Jakob disease (CJD). Am J Hum Genet (1993) 1.34
Mechanism of inhibition of Psi+ prion determinant propagation by a mutation of the N-terminus of the yeast Sup35 protein. EMBO J (1998) 1.31
Protein inheritance (prions) based on parallel in-register beta-sheet amyloid structures. Bioessays (2008) 1.31
Phenotypic heterogeneity and genetic modification of P102L inherited prion disease in an international series. Brain (2008) 1.30
Changing a single amino acid in the N-terminus of murine PrP alters TSE incubation time across three species barriers. EMBO J (2001) 1.30
Crystal structure of human prion protein bound to a therapeutic antibody. Proc Natl Acad Sci U S A (2009) 1.29
Significant association of a M129V independent polymorphism in the 5' UTR of the PRNP gene with sporadic Creutzfeldt-Jakob disease in a large German case-control study. J Med Genet (2006) 1.28
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
Complete penetrance of Creutzfeldt-Jakob disease in Libyan Jews carrying the E200K mutation in the prion protein gene. Mol Med (1995) 1.19
Copper-dependent regulation of NMDA receptors by cellular prion protein: implications for neurodegenerative disorders. J Physiol (2012) 1.19
Inherited prion disease (PrP lysine 200) in Britain: two case reports. BMJ (1993) 1.18
Absence of spontaneous disease and comparative prion susceptibility of transgenic mice expressing mutant human prion proteins. J Gen Virol (2009) 1.16
Species-independent inhibition of abnormal prion protein (PrP) formation by a peptide containing a conserved PrP sequence. J Virol (1999) 1.16
CJD discrepancy. Nature (1991) 1.16
Genome-wide association study in multiple human prion diseases suggests genetic risk factors additional to PRNP. Hum Mol Genet (2011) 1.12
Review: contribution of transgenic models to understanding human prion disease. Neuropathol Appl Neurobiol (2010) 1.11
A clinical study of kuru patients with long incubation periods at the end of the epidemic in Papua New Guinea. Philos Trans R Soc Lond B Biol Sci (2008) 1.10
Attempts to convert the cellular prion protein into the scrapie isoform in cell-free systems. J Virol (1992) 1.09
An overview of human prion diseases. Virol J (2011) 1.08
Prion protein misfolding and disease. Curr Opin Struct Biol (2009) 1.06
Molecular biology and pathology of prion strains in sporadic human prion diseases. Acta Neuropathol (2010) 1.06
Evolution, revolution and heresy in the genetics of infectious disease susceptibility. Philos Trans R Soc Lond B Biol Sci (2012) 1.04
Molecular pathology of human prion disease. Acta Neuropathol (2010) 1.01
Crystallographic studies of prion protein (PrP) segments suggest how structural changes encoded by polymorphism at residue 129 modulate susceptibility to human prion disease. J Biol Chem (2010) 1.01
Lack of TAR-DNA binding protein-43 (TDP-43) pathology in human prion diseases. Neuropathol Appl Neurobiol (2008) 0.99
Delimiting the location of the scrapie prion incubation time gene on chromosome 2 of the mouse. Genetics (1993) 0.99
Human prion diseases in the Netherlands (1998-2009): clinical, genetic and molecular aspects. PLoS One (2012) 0.98
Association of sporadic Creutzfeldt-Jakob disease with homozygous genotypes at PRNP codons 129 and 219 in the Korean population. Neurogenetics (2005) 0.98
Cellular prion protein mediates the toxicity of beta-amyloid oligomers: implications for Alzheimer disease. Arch Neurol (2009) 0.96
Genome-wide search for markers associated with bovine spongiform encephalopathy. Mamm Genome (2002) 0.96
Investigating the role of rare coding variability in Mendelian dementia genes (APP, PSEN1, PSEN2, GRN, MAPT, and PRNP) in late-onset Alzheimer's disease. Neurobiol Aging (2014) 0.95
Iatrogenic CJD due to pituitary-derived growth hormone with genetically determined incubation times of up to 40 years. Brain (2015) 0.95
The primary structure of the prion protein influences the distribution of abnormal prion protein in the central nervous system. Am J Pathol (1992) 0.94
Genetics of prion diseases. Curr Opin Genet Dev (2013) 0.94
Dissecting trait heterogeneity: a comparison of three clustering methods applied to genotypic data. BMC Bioinformatics (2006) 0.92
Regional brain metabolite abnormalities in inherited prion disease and asymptomatic gene carriers demonstrated in vivo by quantitative proton magnetic resonance spectroscopy. Neuroradiology (2006) 0.92
Genetic susceptibility, evolution and the kuru epidemic. Philos Trans R Soc Lond B Biol Sci (2008) 0.91
Prion Disease Induces Alzheimer Disease-Like Neuropathologic Changes. J Neuropathol Exp Neurol (2015) 0.91
Experimental transmission of bovine spongiform encephalopathy to European red deer (Cervus elaphus elaphus). BMC Vet Res (2008) 0.91
Prion protein gene M129 allele is a risk factor for Alzheimer's disease. J Neural Transm (Vienna) (2006) 0.91
Inherited prion diseases. Proc Natl Acad Sci U S A (1994) 0.91
Genetic studies in human prion diseases. J Korean Med Sci (2014) 0.90
Review. The origin of the prion agent of kuru: molecular and biological strain typing. Philos Trans R Soc Lond B Biol Sci (2008) 0.90
Critical significance of the region between Helix 1 and 2 for efficient dominant-negative inhibition by conversion-incompetent prion protein. PLoS Pathog (2013) 0.89
Identification of new quantitative trait Loci (other than the PRNP gene) modulating the scrapie incubation period in sheep. Genetics (2008) 0.89
Burial of the polymorphic residue 129 in amyloid fibrils of prion stop mutants. J Biol Chem (2012) 0.89
Genetic cross-interaction between APOE and PRNP in sporadic Alzheimer's and Creutzfeldt-Jakob diseases. PLoS One (2011) 0.89
The prion protein gene in humans revisited: lessons from a worldwide resequencing study. Genome Res (2005) 0.89
Prion protein-detergent micelle interactions studied by NMR in solution. J Biol Chem (2009) 0.89
Epidemiological characteristics of human prion diseases. Infect Dis Poverty (2016) 0.89
In vivo comparison of chronic wasting disease infectivity from deer with variation at prion protein residue 96. J Virol (2011) 0.88
Strain specific resistance to murine scrapie associated with a naturally occurring human prion protein polymorphism at residue 171. PLoS Pathog (2011) 0.88
Conformational pH dependence of intermediate states during oligomerization of the human prion protein. Protein Sci (2008) 0.88
Novel mutation of the PRNP gene of a clinical CJD case. BMC Infect Dis (2006) 0.86
Identification of I137M and other mutations that modulate incubation periods for two human prion strains. J Virol (2012) 0.86
Modifications of transthyretin in amyloid fibrils: analysis of amyloid from homozygous and heterozygous individuals with the Met30 mutation. EMBO J (1993) 0.86
Prion diseases. BMJ (1993) 0.86
Multiple substitutions of methionine 129 in human prion protein reveal its importance in the amyloid fibrillation pathway. J Biol Chem (2012) 0.85
The retinoic acid receptor beta (Rarb) region of Mmu14 is associated with prion disease incubation time in mouse. PLoS One (2010) 0.85
Clinical features of genetic Creutzfeldt-Jakob disease with V180I mutation in the prion protein gene. BMJ Open (2014) 0.85
The molecular epidemiology of variant CJD. Int J Mol Epidemiol Genet (2011) 0.84
Kuru: genes, cannibals and neuropathology. J Neuropathol Exp Neurol (2012) 0.84
Sod1 deficiency reduces incubation time in mouse models of prion disease. PLoS One (2013) 0.84
Creutzfeldt-Jakob disease and cerebral amyloid angiopathy. Acta Neuropathol (1994) 0.84
Genotype patterns and characteristics of PRNP in the Korean population. Prion (2012) 0.84
Inherited prion disease A117V is not simply a proteinopathy but produces prions transmissible to transgenic mice expressing homologous prion protein. PLoS Pathog (2013) 0.83
Conservation of a glycine-rich region in the prion protein is required for uptake of prion infectivity. J Biol Chem (2010) 0.83
Heterozygous inhibition in prion infection: the stone fence model. Prion (2009) 0.82
PRNP M129V homozygosity in multiple system atrophy vs. Parkinson's disease. Clin Auton Res (2008) 0.82
The same prion strain causes vCJD and BSE. Nature (1997) 12.49
Meta-analysis of 74,046 individuals identifies 11 new susceptibility loci for Alzheimer's disease. Nat Genet (2013) 9.46
Tissue distribution of protease resistant prion protein in variant Creutzfeldt-Jakob disease using a highly sensitive immunoblotting assay. Lancet (2001) 5.57
The central nervous system in motor neurone disease. J Neurol Neurosurg Psychiatry (1970) 4.50
Diagnosis of new variant Creutzfeldt-Jakob disease by tonsil biopsy. Lancet (1997) 4.28
Investigation of variant Creutzfeldt-Jakob disease and other human prion diseases with tonsil biopsy samples. Lancet (1999) 4.02
Genetic predisposition to iatrogenic Creutzfeldt-Jakob disease. Lancet (1991) 3.22
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
Strain-specific prion-protein conformation determined by metal ions. Nat Cell Biol (1999) 2.61
Muscular dystrophy in an X; 1 translocation female suggests that Duchenne locus is on X chromosome short arm. J Med Genet (1979) 2.57
Species-barrier-independent prion replication in apparently resistant species. Proc Natl Acad Sci U S A (2000) 2.50
Post-natal knockout of prion protein alters hippocampal CA1 properties, but does not result in neurodegeneration. EMBO J (2002) 2.26
Location and properties of metal-binding sites on the human prion protein. Proc Natl Acad Sci U S A (2001) 2.16
Post-exposure prophylaxis after accidental prion inoculation. Lancet (1997) 2.15
Insertion in prion protein gene in familial Creutzfeldt-Jakob disease. Lancet (1989) 2.11
Choroid plexus papillomas: an immunohistological study of 16 cases. Histopathology (1989) 2.06
Rapid cell-surface prion protein conversion revealed using a novel cell system. Nat Commun (2011) 2.04
ALS phenotypes with mutations in CHMP2B (charged multivesicular body protein 2B). Neurology (2006) 2.00
Identification of multiple quantitative trait loci linked to prion disease incubation period in mice. Proc Natl Acad Sci U S A (2001) 1.96
Reversible conversion of monomeric human prion protein between native and fibrilogenic conformations. Science (1999) 1.95
Inherited prion disease with 144 base pair gene insertion. 1. Genealogical and molecular studies. Brain (1992) 1.86
Tissue handling in suspected Creutzfeldt-Jakob disease (CJD) and other human spongiform encephalopathies (prion diseases) Brain Pathol (1995) 1.86
Sporadic Creutzfeldt-Jakob disease in a 16-year-old in the UK. Lancet (1995) 1.78
Sporadic Creutzfeldt-Jakob disease in a 18-year-old in the UK. Lancet (1995) 1.76
Molecular screening of sheep for bovine spongiform encephalopathy. Neurosci Lett (1998) 1.74
Miraculous deliverance of Anne Green: an Oxford case of resuscitation in the seventeenth century. Br Med J (Clin Res Ed) (1983) 1.74
Irregular presence of abnormal prion protein in appendix in variant Creutzfeldt-Jakob disease. J Neurol Neurosurg Psychiatry (2002) 1.72
Early onset familial Alzheimer's disease: Mutation frequency in 31 families. Neurology (2003) 1.71
Transmission of scrapie by steel-surface-bound prions. Mol Med (2001) 1.63
Chloroquine myopathy. Q J Med (1971) 1.56
Transmission of Creutzfeldt-Jakob disease by handling of dura mater. Lancet (1993) 1.52
Codon 129 changes in the prion protein gene in Caucasians. Am J Hum Genet (1990) 1.52
Relief of severe intractable pain by barbotage of cerebrospinal fluid. Lancet (1972) 1.50
Sporadic--but not variant--Creutzfeldt-Jakob disease is associated with polymorphisms upstream of PRNP exon 1. Am J Hum Genet (2001) 1.50
Lymphocyte contamination of laryngoscope blades--a possible vector for transmission of variant Creutzfeldt-Jakob disease. Anaesthesia (2005) 1.45
Superficial siderosis of the central nervous system. A report on nine cases with autopsy. Acta Neuropathol (1969) 1.43
Structural mobility of the human prion protein probed by backbone hydrogen exchange. Nat Struct Biol (1999) 1.41
Multiparameter MR imaging in the 6-OPRI variant of inherited prion disease. AJNR Am J Neuroradiol (2013) 1.39
Prion protein gene analysis in new variant cases of Creutzfeldt-Jakob disease. Lancet (1996) 1.37
Transmission of fatal familial insomnia to laboratory animals. Lancet (1995) 1.37
Genetic basis of Creutzfeldt-Jakob disease in the United Kingdom: a systematic analysis of predisposing mutations and allelic variation in the PRNP gene. Hum Genet (1996) 1.36
Spinal cord ischemia due to arteriosclerosis. Arch Neurol (1966) 1.33
Thrombosis of the posterior spinal arteries. A complication of an intrathecal injection of phenol. Neurology (1970) 1.31
Cerebral artery spasm. A histological study at necropsy of the blood vessels in cases of subarachnoid hemorrhage. J Neurosurg (1978) 1.30
Phenotypic heterogeneity and genetic modification of P102L inherited prion disease in an international series. Brain (2008) 1.30
Hippocampal slices from prion protein null mice: disrupted Ca(2+)-activated K+ currents. Neurosci Lett (1996) 1.29
Multiple folding pathways for heterologously expressed human prion protein. Biochim Biophys Acta (1999) 1.29
Crystal structure of human prion protein bound to a therapeutic antibody. Proc Natl Acad Sci U S A (2009) 1.29
Ponto-bulbar palsy with deafness (Brown-Vialetto-Van Laere syndrome). J Neurol Sci (1981) 1.29
Intracranial reticulum cell sarcoma associated with immunoglobulin A deficiency. J Neurol Neurosurg Psychiatry (1973) 1.28
Ultrastructure of anterior horn motor neurones in the Hirano-Kurland-Sayre type of combined neurological system degeneration. J Neurol Sci (1971) 1.27
Fatal familial insomnia: a new Austrian family. Brain (1999) 1.24
Protease-resistant prion protein produced in vitro lacks detectable infectivity. J Gen Virol (1999) 1.24
Neuroimaging findings in human prion disease. J Neurol Neurosurg Psychiatry (2006) 1.21
Rescue of neurophysiological phenotype seen in PrP null mice by transgene encoding human prion protein. Nat Genet (1995) 1.20
Is the neuropathology of new variant Creutzfeldt-Jakob disease and kuru similar? Lancet (1997) 1.14
Immunocytochemical study of the cellular immune response in meningiomas. J Clin Pathol (1988) 1.13
Review: contribution of transgenic models to understanding human prion disease. Neuropathol Appl Neurobiol (2010) 1.11
Inherited prion disease with 5-OPRI: phenotype modification by repeat length and codon 129. Neurology (2007) 1.11
XAFS study of the high-affinity copper-binding site of human PrP(91-231) and its low-resolution structure in solution. J Mol Biol (2001) 1.10
An in-frame insertion in the prion protein gene in familial Creutzfeldt-Jakob disease. Brain Res Mol Brain Res (1990) 1.09
Incomplete penetrance of familial Alzheimer's disease in a pedigree with a novel presenilin-1 gene mutation. Lancet (1996) 1.07
Spinal cord degeneration in divers. Lancet (1987) 1.07
Immunohistological study of mononuclear cell infiltrate in malignant gliomas. Acta Neuropathol (1987) 1.07
A dementing illness associated with a novel insertion in the prion protein gene. Brain Res Mol Brain Res (1992) 1.04
Pathology of amyotrophic lateral sclerosis. Adv Neurol (1982) 1.03
Association of a null allele of SPRN with variant Creutzfeldt-Jakob disease. J Med Genet (2008) 1.02
The mononuclear cell infiltrate compared with survival in high-grade astrocytomas. Acta Neuropathol (1989) 1.02
Childhood mitochondrial myopathy with ophthalmoplegia. J Neurol Sci (1981) 1.01
Lack of TAR-DNA binding protein-43 (TDP-43) pathology in human prion diseases. Neuropathol Appl Neurobiol (2008) 0.99
Chromosome 3 linked frontotemporal dementia (FTD-3). Neurology (2002) 0.98
Seven-year discordance in age at onset in monozygotic twins with inherited prion disease (P102L). Neuropathol Appl Neurobiol (2009) 0.98
Pathology of peroneal muscular atrophy (Charcot-Marie-Tooth disease). J Neurol Neurosurg Psychiatry (1972) 0.97
Conformational properties of the prion octa-repeat and hydrophobic sequences. FEBS Lett (1997) 0.96
Prion infectivity in variant Creutzfeldt-Jakob disease rectum. Gut (2006) 0.96
Presenilin-1 mutation (E280G), spastic paraparesis, and cranial MRI white-matter abnormalities. Neurology (2002) 0.95
Mossy fibre reorganization in the hippocampus of prion protein null mice. Brain Res (1997) 0.95
Deletions in the prion protein gene are not associated with CJD. Hum Mol Genet (1993) 0.94
The molecular biology of prion propagation. Philos Trans R Soc Lond B Biol Sci (2001) 0.94
High-b-value diffusion MR imaging and basal nuclei apparent diffusion coefficient measurements in variant and sporadic Creutzfeldt-Jakob disease. AJNR Am J Neuroradiol (2009) 0.94
A presenilin 1 R278I mutation presenting with language impairment. Neurology (2004) 0.94
High levels of disease related prion protein in the ileum in variant Creutzfeldt-Jakob disease. Gut (2005) 0.94
Traumatic thrombosis of the internal carotid artery in the neck. J Neurol Neurosurg Psychiatry (1968) 0.94
An antibody raised against a conserved sequence of the prion protein recognizes pathological isoforms in human and animal prion diseases, including Creutzfeldt-Jakob disease and bovine spongiform encephalopathy. Am J Pathol (1998) 0.93
A prion disease with a novel 96-base pair insertional mutation in the prion protein gene. Neurology (1996) 0.93
Ultrastructural studies in human polymyositis. J Neurol Sci (1975) 0.93
Examination of the human prion protein-like gene doppel for genetic susceptibility to sporadic and variant Creutzfeldt-Jakob disease. Neurosci Lett (2000) 0.93
Insertions in the prion protein gene in atypical dementias. Exp Neurol (1991) 0.92
The effect of varying protein quality and energy intake on the nitrogen metabolism of parenterally fed very low birthweight (less than 1600 g) infants. Pediatr Res (1981) 0.92
Inherited prion disease with an alanine to valine mutation at codon 117 in the prion protein gene. Brain (1999) 0.92
Regional brain metabolite abnormalities in inherited prion disease and asymptomatic gene carriers demonstrated in vivo by quantitative proton magnetic resonance spectroscopy. Neuroradiology (2006) 0.92
Thymoma, plasma cell myeloma, red cell aplasia and malabsorption syndrome. Am J Med (1968) 0.91
Phenotypic variability in the brains of a family with a prion disease characterized by a 144-base pair insertion in the prion protein gene. Neuropathol Appl Neurobiol (2003) 0.90
Two-octapeptide repeat deletion of prion protein associated with rapidly progressive dementia. Neurology (2001) 0.90
Familial non-specific dementia maps to chromosome 3. Hum Mol Genet (1995) 0.90
Age of onset and death in inherited prion disease are heritable. Am J Med Genet B Neuropsychiatr Genet (2009) 0.89