Published in FASEB J on February 09, 2009
Exacerbation of experimental autoimmune encephalomyelitis in prion protein (PrPc)-null mice: evidence for a critical role of the central nervous system. J Neuroinflammation (2012) 0.92
Pharmacological prion protein silencing accelerates central nervous system autoimmune disease via T cell receptor signalling. Brain (2010) 0.86
Endogenous prion protein attenuates experimentally induced colitis. Am J Pathol (2011) 0.82
Neuroprotective effects of the cellular prion protein in autoimmune optic neuritis. Am J Pathol (2011) 0.80
Beta-amyloid peptides enhance the proliferative response of activated CD4CD28 lymphocytes from Alzheimer disease patients and from healthy elderly. PLoS One (2012) 0.79
Increased interleukin-17 in the cerebrospinal fluid in sporadic Creutzfeldt-Jakob disease: a case-control study of rapidly progressive dementia. J Neuroinflammation (2013) 0.76
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
Balancing selection at the prion protein gene consistent with prehistoric kurulike epidemics. Science (2003) 3.59
Clinical presentation and pre-mortem diagnosis of variant Creutzfeldt-Jakob disease associated with blood transfusion: a case report. Lancet (2006) 3.12
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
Th1 not Th17 cells drive spontaneous MS-like disease despite a functional regulatory T cell response. Acta Neuropathol (2013) 2.80
Prion propagation and toxicity in vivo occur in two distinct mechanistic phases. Nature (2011) 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
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
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
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
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
Peptide immunotherapy in allergic asthma generates IL-10-dependent immunological tolerance associated with linked epitope suppression. J Exp Med (2009) 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
A novel prion disease associated with diarrhea and autonomic neuropathy. N Engl J Med (2013) 1.69
Proteomic profiling of plasma in Huntington's disease reveals neuroinflammatory activation and biomarker candidates. J Proteome Res (2007) 1.59
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
Sodium chloride inhibits the suppressive function of FOXP3+ regulatory T cells. J Clin Invest (2015) 1.43
Disease-related prion protein forms aggresomes in neuronal cells leading to caspase activation and apoptosis. J Biol Chem (2005) 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
Analysis of potential transcriptomic biomarkers for Huntington's disease in peripheral blood. Proc Natl Acad Sci U S A (2007) 1.35
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
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
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
Subclinical prion infection in humans and animals. Br Med Bull (2003) 1.25
The bacterial superantigen streptococcal mitogenic exotoxin Z is the major immunoactive agent of Streptococcus pyogenes. J Immunol (2002) 1.24
Characterization of two distinct prion strains derived from bovine spongiform encephalopathy transmissions to inbred mice. J Gen Virol (2004) 1.23
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
Parietal lobe deficits in frontotemporal lobar degeneration caused by a mutation in the progranulin gene. Arch Neurol (2008) 1.20
The mechanism of superantigen-mediated toxic shock: not a simple Th1 cytokine storm. J Immunol (2005) 1.20
CHMP2B C-truncating mutations in frontotemporal lobar degeneration are associated with an aberrant endosomal phenotype in vitro. Hum Mol Genet (2007) 1.20
Chronic wasting disease prions are not transmissible to transgenic mice overexpressing human prion protein. J Gen Virol (2010) 1.19
HECTD2 is associated with susceptibility to mouse and human prion disease. PLoS Genet (2009) 1.18
Rational targeting for prion therapeutics. Nat Rev Neurosci (2005) 1.18
Mapping the progression of progranulin-associated frontotemporal lobar degeneration. Nat Clin Pract Neurol (2008) 1.17
Genetic variability in CLU and its association with Alzheimer's disease. PLoS One (2010) 1.16
Absence of spontaneous disease and comparative prion susceptibility of transgenic mice expressing mutant human prion proteins. J Gen Virol (2009) 1.16
Efficient dissemination of prions through preferential transmission to nearby cells. J Gen Virol (2007) 1.15
Inherited prion disease with six octapeptide repeat insertional mutation--molecular analysis of phenotypic heterogeneity. Brain (2006) 1.15
The residue 129 polymorphism in human prion protein does not confer susceptibility to Creutzfeldt-Jakob disease by altering the structure or global stability of PrPC. J Biol Chem (2004) 1.14
An enzyme-detergent method for effective prion decontamination of surgical steel. J Gen Virol (2005) 1.14
HLA-C and killer cell immunoglobulin-like receptor genes in idiopathic bronchiectasis. Am J Respir Crit Care Med (2005) 1.14
Misfolded PrP impairs the UPS by interaction with the 20S proteasome and inhibition of substrate entry. EMBO J (2011) 1.13
CD27 expression discriminates between regulatory and non-regulatory cells after expansion of human peripheral blood CD4+ CD25+ cells. Immunology (2007) 1.13
Genome-wide association study in multiple human prion diseases suggests genetic risk factors additional to PRNP. Hum Mol Genet (2011) 1.12
Molecular diagnosis of human prion disease. Methods Mol Biol (2008) 1.12
Genetics of prion disease. Top Curr Chem (2011) 1.11
Elongated oligomers assemble into mammalian PrP amyloid fibrils. J Mol Biol (2006) 1.10
PrP glycoforms are associated in a strain-specific ratio in native PrPSc. J Gen Virol (2005) 1.09
Modified amino acid copolymers suppress myelin basic protein 85-99-induced encephalomyelitis in humanized mice through different effects on T cells. Proc Natl Acad Sci U S A (2004) 1.08
PRNP allelic series from 19 years of prion protein gene sequencing at the MRC Prion Unit. Hum Mutat (2010) 1.07
Isolation of proteinase K-sensitive prions using pronase E and phosphotungstic acid. PLoS One (2010) 1.06
No association of PGRN 3'UTR rs5848 in frontotemporal lobar degeneration. Neurobiol Aging (2009) 1.05
Validation of next-generation sequencing technologies in genetic diagnosis of dementia. Neurobiol Aging (2013) 1.05
Modification of superoxide dismutase 1 (SOD1) properties by a GFP tag--implications for research into amyotrophic lateral sclerosis (ALS). PLoS One (2010) 1.05
Duplication of amyloid precursor protein (APP), but not prion protein (PRNP) gene is a significant cause of early onset dementia in a large UK series. Neurobiol Aging (2010) 1.04
Highly sensitive, quantitative cell-based assay for prions adsorbed to solid surfaces. Proc Natl Acad Sci U S A (2009) 1.04
High incidence of spontaneous disease in an HLA-DR15 and TCR transgenic multiple sclerosis model. J Immunol (2005) 1.04