Accelerated, spleen-based titration of variant Creutzfeldt-Jakob disease infectivity in transgenic mice expressing human prion protein with sensitivity comparable to that of survival time bioassay.

Emergence of two prion subtypes in ovine PrP transgenic mice infected with human MM2-cortical Creutzfeldt-Jakob disease prions. Acta Neuropathol Commun (2016) 0.84

Post-conversion sialylation of prions in lymphoid tissues. Proc Natl Acad Sci U S A (2015) 0.83

Multifaceted Role of Sialylation in Prion Diseases. Front Neurosci (2016) 0.79

Sialylation Controls Prion Fate in Vivo. J Biol Chem (2016) 0.78

Absence of Evidence for a Causal Link between Bovine Spongiform Encephalopathy Strain Variant L-BSE and Known Forms of Sporadic Creutzfeldt-Jakob Disease in Human PrP Transgenic Mice. J Virol (2016) 0.75

Rapid and Highly Sensitive Detection of Variant Creutzfeldt - Jakob Disease Abnormal Prion Protein on Steel Surfaces by Protein Misfolding Cyclic Amplification: Application to Prion Decontamination Studies. PLoS One (2016) 0.75

Tissue distribution of protease resistant prion protein in variant Creutzfeldt-Jakob disease using a highly sensitive immunoblotting assay. Lancet (2001) 5.57

Human spongiform encephalopathy: the National Institutes of Health series of 300 cases of experimentally transmitted disease. Ann Neurol (1994) 5.20

Preclinical vCJD after blood transfusion in a PRNP codon 129 heterozygous patient. Lancet (2004) 4.59

Measurement of the scrapie agent using an incubation time interval assay. Ann Neurol (1982) 3.68

Natural infection of Suffolk sheep with scrapie virus. J Infect Dis (1982) 3.42

Pathogenesis of scrapie virus infection in the mouse. J Infect Dis (1967) 3.34

Prions. Cold Spring Harb Perspect Biol (2011) 3.17

Clinical presentation and pre-mortem diagnosis of variant Creutzfeldt-Jakob disease associated with blood transfusion: a case report. Lancet (2006) 3.12

Prevalent abnormal prion protein in human appendixes after bovine spongiform encephalopathy epizootic: large scale survey. BMJ (2013) 2.74

Detection of prion infection in variant Creutzfeldt-Jakob disease: a blood-based assay. Lancet (2011) 2.60

Prevalence of lymphoreticular prion protein accumulation in UK tissue samples. J Pathol (2004) 2.47

Facilitated cross-species transmission of prions in extraneural tissue. Science (2012) 2.26

Asymptomatic deer excrete infectious prions in faeces. Nature (2009) 2.10

Preliminary observations on the pathogenesis of experimental bovine spongiform encephalopathy (BSE): an update. Vet Rec (1998) 2.05

Pathogenesis of scrapie (strain 263K) in hamsters infected intracerebrally, intraperitoneally or intraocularly. J Gen Virol (1986) 1.87

Scrapie infectivity and proteinase K-resistant prion protein in sheep placenta, brain, spleen, and lymph node: implications for transmission and antemortem diagnosis. J Infect Dis (1998) 1.86

Detection of variant Creutzfeldt-Jakob disease infectivity in extraneural tissues. Lancet (2001) 1.84

Prion disease blood test using immunoprecipitation and improved quaking-induced conversion. MBio (2011) 1.82

Prion agent diversity and species barrier. Vet Res (2008) 1.80

Creutzfeldt-Jakob disease and blood transfusion: results of the UK Transfusion Medicine Epidemiological Review study. Vox Sang (2006) 1.76

Highly bovine spongiform encephalopathy-sensitive transgenic mice confirm the essential restriction of infectivity to the nervous system in clinically diseased cattle. J Infect Dis (2005) 1.70

Pathogenesis of mouse scrapie: dynamics of agent replication in spleen, spinal cord and brain after infection by different routes. J Comp Pathol (1979) 1.69

The sequential development of abnormal prion protein accumulation in mice with Creutzfeldt-Jakob disease. Am J Pathol (1992) 1.66

The physical relationship between infectivity and prion protein aggregates is strain-dependent. PLoS Pathog (2010) 1.59

Update on human prion disease. Biochim Biophys Acta (2007) 1.46

Variant CJD infection in the spleen of a neurologically asymptomatic UK adult patient with haemophilia. Haemophilia (2010) 1.45

Molecular and clinical classification of human prion disease. Br Med Bull (2003) 1.45

Transmission of atypical bovine prions to mice transgenic for human prion protein. Emerg Infect Dis (2008) 1.38

Primary isolation of the bovine spongiform encephalopathy agent in mice: agent definition based on a review of 150 transmissions. J Comp Pathol (2005) 1.34

Highly efficient prion transmission by blood transfusion. PLoS Pathog (2012) 1.30

Atypical/Nor98 scrapie infectivity in sheep peripheral tissues. PLoS Pathog (2011) 1.26

Prominent and persistent extraneural infection in human PrP transgenic mice infected with variant CJD. PLoS One (2008) 1.24

Similar levels of infectivity in the blood of mice infected with human-derived vCJD and GSS strains of transmissible spongiform encephalopathy. Transfusion (2003) 1.23

Studies of the transmissibility of the agent of bovine spongiform encephalopathy to pigs. J Gen Virol (2003) 1.20

In vitro detection of prionemia in TSE-infected cervids and hamsters. PLoS One (2013) 1.17

Resistance of bovine spongiform encephalopathy (BSE) prions to inactivation. PLoS Pathog (2008) 1.14

Marked influence of the route of infection on prion strain apparent phenotype in a scrapie transgenic mouse model. Neurobiol Dis (2010) 1.04

Inactivation of prions by physical and chemical means. J Hosp Infect (1999) 1.04

Prions and the potential transmissibility of protein misfolding diseases. Annu Rev Microbiol (2013) 1.04

The effects of host age on follicular dendritic cell status dramatically impair scrapie agent neuroinvasion in aged mice. J Immunol (2009) 0.99

Genetical control of the concentration of ME7 scrapie agent in mouse spleen. J Comp Pathol (1969) 0.98

Opposite effects of dextran sulfate 500, the polyene antibiotic MS-8209, and Congo red on accumulation of the protease-resistant isoform of PrP in the spleens of mice inoculated intraperitoneally with the scrapie agent. J Virol (2000) 0.97

Effect of fixation on brain and lymphoreticular vCJD prions and bioassay of key positive specimens from a retrospective vCJD prevalence study. J Pathol (2010) 0.94

Detection of infectivity in blood of persons with variant and sporadic Creutzfeldt-Jakob disease. Emerg Infect Dis (2014) 0.91

Highly infectious prions generated by a single round of microplate-based protein misfolding cyclic amplification. MBio (2013) 0.89

The sympathetic nervous system is involved in variant Creutzfeldt-Jakob disease. Nat Med (2003) 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

Prions and peripheral nerves: a deadly rendezvous. J Neurosci Res (2007) 0.86

Impact of leucocyte depletion and prion reduction filters on TSE blood borne transmission. PLoS One (2012) 0.84

PrP expression level and sensitivity to prion infection. J Virol (2014) 0.81

A highly specific blood test for vCJD. Blood (2014) 0.80

White blood cell-based detection of asymptomatic scrapie infection by ex vivo assays. PLoS One (2014) 0.81

Transgenic Rabbits Expressing Ovine PrP Are Susceptible to Scrapie. PLoS Pathog (2015) 0.78

Prion protein localizes at the ciliary base during neural and cardiovascular development, and its depletion affects α-tubulin post-translational modifications. Sci Rep (2015) 0.75