Published in Lancet on December 09, 2006
Safety and efficacy of quinacrine in human prion disease (PRION-1 study): a patient-preference trial. Lancet Neurol (2009) 2.41
Transmission and detection of prions in feces. J Infect Dis (2008) 2.20
Population screening for variant Creutzfeldt-Jakob disease using a novel blood test: diagnostic accuracy and feasibility study. JAMA Neurol (2014) 2.08
Genetic risk factors for variant Creutzfeldt-Jakob disease: a genome-wide association study. Lancet Neurol (2009) 1.94
Infectious prions in pre-clinical deer and transmission of chronic wasting disease solely by environmental exposure. PLoS One (2009) 1.90
Prion disease blood test using immunoprecipitation and improved quaking-induced conversion. MBio (2011) 1.82
Prevalence of disease related prion protein in anonymous tonsil specimens in Britain: cross sectional opportunistic survey. BMJ (2009) 1.75
Prions in the urine of patients with variant Creutzfeldt-Jakob disease. N Engl J Med (2014) 1.74
Surgery and risk of sporadic Creutzfeldt-Jakob disease in Denmark and Sweden: registry-based case-control studies. Neuroepidemiology (2008) 1.56
Prion proteins in subpopulations of white blood cells from patients with sporadic Creutzfeldt-Jakob disease. Lab Invest (2009) 1.48
Sensitive and specific detection of sporadic Creutzfeldt-Jakob disease brain prion protein using real-time quaking-induced conversion. J Gen Virol (2011) 1.40
Multiparameter MR imaging in the 6-OPRI variant of inherited prion disease. AJNR Am J Neuroradiol (2013) 1.39
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
Crystal structure of human prion protein bound to a therapeutic antibody. Proc Natl Acad Sci U S A (2009) 1.29
Is there a risk of prion-like disease transmission by Alzheimer- or Parkinson-associated protein particles? Acta Neuropathol (2014) 1.24
Neuroimaging findings in human prion disease. J Neurol Neurosurg Psychiatry (2006) 1.21
In vitro detection of prionemia in TSE-infected cervids and hamsters. PLoS One (2013) 1.17
Intraepithelial and interstitial deposition of pathological prion protein in kidneys of scrapie-affected sheep. PLoS One (2007) 1.17
Review. Lessons of kuru research: background to recent studies with some personal reflections. Philos Trans R Soc Lond B Biol Sci (2008) 1.15
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
Protein misfolding cyclic amplification of infectious prions. Nat Protoc (2012) 1.10
An overview of human prion diseases. Virol J (2011) 1.08
All clinically-relevant blood components transmit prion disease following a single blood transfusion: a sheep model of vCJD. PLoS One (2011) 1.08
Characterization of prion protein (PrP)-derived peptides that discriminate full-length PrPSc from PrPC. Proc Natl Acad Sci U S A (2007) 1.07
PRNP variation in UK sporadic and variant Creutzfeldt Jakob disease highlights genetic risk factors and a novel non-synonymous polymorphism. BMC Med Genet (2009) 1.06
Emerging trends in plasma-free manufacturing of recombinant protein therapeutics expressed in mammalian cells. Biotechnol J (2009) 1.05
Emergence and natural selection of drug-resistant prions. Mol Biosyst (2010) 1.05
Highly sensitive, quantitative cell-based assay for prions adsorbed to solid surfaces. Proc Natl Acad Sci U S A (2009) 1.04
Uncertainty in the tail of the variant Creutzfeldt-Jakob disease epidemic in the UK. PLoS One (2010) 1.04
Central and peripheral pathology of kuru: pathological analysis of a recent case and comparison with other forms of human prion disease. Philos Trans R Soc Lond B Biol Sci (2008) 1.03
AA-amyloidosis can be transferred by peripheral blood monocytes. PLoS One (2008) 1.03
Detection of PrPsc in blood from sheep infected with the scrapie and bovine spongiform encephalopathy agents. J Virol (2009) 1.01
Molecular pathology of human prion disease. Acta Neuropathol (2010) 1.01
Anti-PrP Mab 6D11 suppresses PrP(Sc) replication in prion infected myeloid precursor line FDC-P1/22L and in the lymphoreticular system in vivo. Neurobiol Dis (2009) 1.00
Lack of TAR-DNA binding protein-43 (TDP-43) pathology in human prion diseases. Neuropathol Appl Neurobiol (2008) 0.99
Sheep with scrapie and mastitis transmit infectious prions through the milk. J Virol (2010) 0.98
Variant CJD. 18 years of research and surveillance. Prion (2014) 0.93
Rapid and Quantitative Assay of Amyloid-Seeding Activity in Human Brains Affected with Prion Diseases. PLoS One (2015) 0.93
Effective gene therapy in a mouse model of prion diseases. PLoS One (2008) 0.92
Re-assessment of PrP(Sc) distribution in sporadic and variant CJD. PLoS One (2013) 0.92
Mother to offspring transmission of chronic wasting disease in reeves' muntjac deer. PLoS One (2013) 0.92
Prion infectivity in the spleen of a PRNP heterozygous individual with subclinical variant Creutzfeldt-Jakob disease. Brain (2013) 0.91
Nosocomial transmission of sporadic Creutzfeldt-Jakob disease: results from a risk-based assessment of surgical interventions. J Neurol Neurosurg Psychiatry (2010) 0.91
Pharmacological chaperone for the structured domain of human prion protein. Proc Natl Acad Sci U S A (2010) 0.90
A standardized comparison of commercially available prion decontamination reagents using the Standard Steel-Binding Assay. J Gen Virol (2010) 0.90
Epidemiological characteristics of human prion diseases. Infect Dis Poverty (2016) 0.89
Conformational pH dependence of intermediate states during oligomerization of the human prion protein. Protein Sci (2008) 0.88
The effects of host age on the transport of complement-bound complexes to the spleen and the pathogenesis of intravenous scrapie infection. J Virol (2011) 0.88
B cell-specific S1PR1 deficiency blocks prion dissemination between secondary lymphoid organs. J Immunol (2012) 0.87
A new prion disease: relationship with central and peripheral amyloidoses. Nat Rev Neurol (2015) 0.87
A critical appraisal of the pathogenic protein spread hypothesis of neurodegeneration. Nat Rev Neurosci (2016) 0.86
The molecular epidemiology of variant CJD. Int J Mol Epidemiol Genet (2011) 0.84
Neuroinvasion in prion diseases: the roles of ascending neural infection and blood dissemination. Interdiscip Perspect Infect Dis (2010) 0.83
Chemical and biophysical insights into the propagation of prion strains. HFSP J (2008) 0.83
Post-conversion sialylation of prions in lymphoid tissues. Proc Natl Acad Sci U S A (2015) 0.83
Brain-water diffusion coefficients reflect the severity of inherited prion disease. Neurology (2010) 0.83
Prion pathogenesis and secondary lymphoid organs (SLO): tracking the SLO spread of prions to the brain. Prion (2012) 0.83
Role of prion protein aggregation in neurotoxicity. Int J Mol Sci (2012) 0.83
MRI and clinical syndrome in dura mater-related Creutzfeldt-Jakob disease. J Neurol (2009) 0.82
Cathepsin D SNP associated with increased risk of variant Creutzfeldt-Jakob disease. BMC Med Genet (2008) 0.82
Detection of prions in blood from patients with variant Creutzfeldt-Jakob disease. Sci Transl Med (2016) 0.82
No major change in vCJD agent strain after secondary transmission via blood transfusion. PLoS One (2008) 0.81
Prion replication in the hematopoietic compartment is not required for neuroinvasion in scrapie mouse model. PLoS One (2010) 0.81
White blood cell-based detection of asymptomatic scrapie infection by ex vivo assays. PLoS One (2014) 0.81
From prion diseases to prion-like propagation mechanisms of neurodegenerative diseases. Int J Cell Biol (2013) 0.81
Therapy for prion diseases: Insights from the use of RNA interference. Prion (2009) 0.80
Comparative Study of Prions in Iatrogenic and Sporadic Creutzfeldt-Jakob Disease. J Clin Cell Immunol (2014) 0.80
A third episode of transfusion-derived vCJD. Lancet (2006) 0.80
Variant Creutzfeldt-Jakob disease with extremely low lymphoreticular deposition of prion protein. JAMA Neurol (2014) 0.79
Multifaceted Role of Sialylation in Prion Diseases. Front Neurosci (2016) 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
Variably protease-sensitive prionopathy, a unique prion variant with inefficient transmission properties. Emerg Infect Dis (2014) 0.78
Mechanism of scrapie prion precipitation with phosphotungstate anions. ACS Chem Biol (2015) 0.78
Lack of prion infectivity in fixed heart tissue from patients with Creutzfeldt-Jakob disease or amyloid heart disease. J Virol (2013) 0.78
Characterization of variant Creutzfeldt-Jakob disease prions in prion protein-humanized mice carrying distinct codon 129 genotypes. J Biol Chem (2013) 0.77
Application of Atomic Dielectric Resonance Spectroscopy for the screening of blood samples from patients with clinical variant and sporadic CJD. J Transl Med (2007) 0.77
Emerging roles of extracellular vesicles in neurodegenerative disorders: focus on HIV-associated neurological complications. Cell Death Dis (2016) 0.76
Preclinical detection of infectivity and disease-specific PrP in blood throughout the incubation period of prion disease. Sci Rep (2015) 0.76
The prion protein preference of sporadic Creutzfeldt-Jakob disease subtypes. J Biol Chem (2012) 0.75
Infectious disease - developments in the field of Creutzfeldt-Jakob disease. Rev Neurol Dis (2007) 0.75
Neuroimaging in human prion disease: Searching in the mist. World J Radiol (2009) 0.75
Prion pathogenesis is unaltered following down-regulation of SIGN-R1. Virology (2016) 0.75
Diagnostic value of terminal ileum biopsies in patients with abnormal terminal ileum mucosal appearance. Ulus Cerrahi Derg (2015) 0.75
Characterisation of radioiodinated flavonoid derivatives for SPECT imaging of cerebral prion deposits. Sci Rep (2015) 0.75
Porcine prion protein amyloid. Prion (2015) 0.75
Guinea Pig Prion Protein Supports Rapid Propagation of Bovine Spongiform Encephalopathy and Variant Creutzfeldt-Jakob Disease Prions. J Virol (2016) 0.75
Genome-wide association study identifies variants at CLU and PICALM associated with Alzheimer's disease. Nat Genet (2009) 15.15
TREM2 variants in Alzheimer's disease. N Engl J Med (2012) 11.35
Common variants at ABCA7, MS4A6A/MS4A4E, EPHA1, CD33 and CD2AP are associated with Alzheimer's disease. Nat Genet (2011) 9.23
Depleting neuronal PrP in prion infection prevents disease and reverses spongiosis. Science (2003) 4.57
Mutations in the endosomal ESCRTIII-complex subunit CHMP2B in frontotemporal dementia. Nat Genet (2005) 4.21
An aneuploid mouse strain carrying human chromosome 21 with Down syndrome phenotypes. Science (2005) 3.61
Balancing selection at the prion protein gene consistent with prehistoric kurulike epidemics. Science (2003) 3.59
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
Human prion protein with valine 129 prevents expression of variant CJD phenotype. Science (2004) 2.82
Prion propagation and toxicity in vivo occur in two distinct mechanistic phases. Nature (2011) 2.74
Prevalent abnormal prion protein in human appendixes after bovine spongiform encephalopathy epizootic: large scale survey. BMJ (2013) 2.74
Frontotemporal dementia with the C9ORF72 hexanucleotide repeat expansion: clinical, neuroanatomical and neuropathological features. Brain (2012) 2.65
Detection of prion infection in variant Creutzfeldt-Jakob disease: a blood-based assay. Lancet (2011) 2.60
Targeting cellular prion protein reverses early cognitive deficits and neurophysiological dysfunction in prion-infected mice. Neuron (2007) 2.58
Genetic evidence implicates the immune system and cholesterol metabolism in the aetiology of Alzheimer's disease. PLoS One (2010) 2.57
Autoantibodies in sporadic Creutzfeldt-Jakob disease. JAMA Neurol (2013) 2.42
Molecular classification of sporadic Creutzfeldt-Jakob disease. Brain (2003) 2.42
Safety and efficacy of quinacrine in human prion disease (PRION-1 study): a patient-preference trial. Lancet Neurol (2009) 2.41
Monoclonal antibodies inhibit prion replication and delay the development of prion disease. Nature (2003) 2.31
FUS pathology defines the majority of tau- and TDP-43-negative frontotemporal lobar degeneration. Acta Neuropathol (2010) 2.30
A systematic review of prion therapeutics in experimental models. Brain (2006) 2.25
Fbw7 controls neural stem cell differentiation and progenitor apoptosis via Notch and c-Jun. Nat Neurosci (2010) 2.23
Neuroprotective role of the Reaper-related serine protease HtrA2/Omi revealed by targeted deletion in mice. Mol Cell Biol (2004) 2.15
A distinct clinical, neuropsychological and radiological phenotype is associated with progranulin gene mutations in a large UK series. Brain (2008) 2.13
Large C9orf72 hexanucleotide repeat expansions are seen in multiple neurodegenerative syndromes and are more frequent than expected in the UK population. Am J Hum Genet (2013) 2.09
Magnetic resonance imaging evidence for presymptomatic change in thalamus and caudate in familial Alzheimer's disease. Brain (2013) 2.05
Disease-associated prion protein oligomers inhibit the 26S proteasome. Mol Cell (2007) 2.01
Analysis of 2000 consecutive UK tonsillectomy specimens for disease-related prion protein. Lancet (2004) 1.96
Interaction between prion protein and toxic amyloid β assemblies can be therapeutically targeted at multiple sites. Nat Commun (2011) 1.96
Genetic risk factors for variant Creutzfeldt-Jakob disease: a genome-wide association study. Lancet Neurol (2009) 1.94
Treatable childhood neuronopathy caused by mutations in riboflavin transporter RFVT2. Brain (2013) 1.93
Amyloid beta-protein dimers rapidly form stable synaptotoxic protofibrils. J Neurosci (2010) 1.92
Variant CJD in an individual heterozygous for PRNP codon 129. Lancet (2009) 1.84
Detection and characterization of proteinase K-sensitive disease-related prion protein with thermolysin. Biochem J (2008) 1.77
Suburothelial myofibroblasts in the human overactive bladder and the effect of botulinum neurotoxin type A treatment. Eur Urol (2008) 1.73
Single treatment with RNAi against prion protein rescues early neuronal dysfunction and prolongs survival in mice with prion disease. Proc Natl Acad Sci U S A (2008) 1.71
Neonatal hepatic steatosis by disruption of the adenosine kinase gene. Proc Natl Acad Sci U S A (2002) 1.70
A novel prion disease associated with diarrhea and autonomic neuropathy. N Engl J Med (2013) 1.69
Combinations of genetic mutations in the adult neural stem cell compartment determine brain tumour phenotypes. EMBO J (2009) 1.66
PTEN is essential for cell migration but not for fate determination and tumourigenesis in the cerebellum. Development (2002) 1.64
Early-onset L-dopa-responsive parkinsonism with pyramidal signs due to ATP13A2, PLA2G6, FBXO7 and spatacsin mutations. Mov Disord (2010) 1.56
A novel protective prion protein variant that colocalizes with kuru exposure. N Engl J Med (2009) 1.53
Update on human prion disease. Biochim Biophys Acta (2007) 1.46
Inherited prion disease with 4-octapeptide repeat insertion: disease requires the interaction of multiple genetic risk factors. Brain (2011) 1.45
Molecular and clinical classification of human prion disease. Br Med Bull (2003) 1.45
Disease-related prion protein forms aggresomes in neuronal cells leading to caspase activation and apoptosis. J Biol Chem (2005) 1.42
High field (9.4 Tesla) magnetic resonance imaging of cortical grey matter lesions in multiple sclerosis. Brain (2010) 1.42
Homozygosity for the C9orf72 GGGGCC repeat expansion in frontotemporal dementia. Acta Neuropathol (2013) 1.41
Atypical scrapie prions from sheep and lack of disease in transgenic mice overexpressing human prion protein. Emerg Infect Dis (2013) 1.38
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
Regional heterogeneity of cellular prion protein isoforms in the mouse brain. Brain (2003) 1.37
Subclinical prion infection. Trends Microbiol (2003) 1.36
Disruption of endocytic trafficking in frontotemporal dementia with CHMP2B mutations. Hum Mol Genet (2010) 1.35
The role of CHMP2B in frontotemporal dementia. Biochem Soc Trans (2009) 1.34
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
Wnt signalling inhibits neural differentiation of embryonic stem cells by controlling bone morphogenetic protein expression. Mol Cell Neurosci (2003) 1.32
Mitochondria and quality control defects in a mouse model of Gaucher disease--links to Parkinson's disease. Cell Metab (2013) 1.32
Identification of genetic loci affecting mouse-adapted bovine spongiform encephalopathy incubation time in mice. Neurogenetics (2002) 1.31
Distinct glycoform ratios of protease resistant prion protein associated with PRNP point mutations. Brain (2006) 1.31
Subclinical prion infection in humans and animals. Br Med Bull (2003) 1.25
Superoxide dismutase 1 and tgSOD1 mouse spinal cord seed fibrils, suggesting a propagative cell death mechanism in amyotrophic lateral sclerosis. PLoS One (2010) 1.25