Published in EMBO J on January 24, 2007
Cellular prion protein mediates impairment of synaptic plasticity by amyloid-beta oligomers. Nature (2009) 6.99
Memory impairment in transgenic Alzheimer mice requires cellular prion protein. J Neurosci (2010) 3.36
Protein aggregation diseases: pathogenicity and therapeutic perspectives. Nat Rev Drug Discov (2010) 2.58
A systems approach to prion disease. Mol Syst Biol (2009) 2.49
The two faces of protein misfolding: gain- and loss-of-function in neurodegenerative diseases. EMBO J (2008) 2.43
Axonal prion protein is required for peripheral myelin maintenance. Nat Neurosci (2010) 2.25
The prion hypothesis: from biological anomaly to basic regulatory mechanism. Nat Rev Mol Cell Biol (2010) 2.22
De novo generation of a transmissible spongiform encephalopathy by mouse transgenesis. Proc Natl Acad Sci U S A (2008) 2.10
In vitro and in vivo neurotoxicity of prion protein oligomers. PLoS Pathog (2007) 1.56
The prion protein knockout mouse: a phenotype under challenge. Prion (2007) 1.51
The CNS glycoprotein Shadoo has PrP(C)-like protective properties and displays reduced levels in prion infections. EMBO J (2007) 1.49
The toxicity of antiprion antibodies is mediated by the flexible tail of the prion protein. Nature (2013) 1.27
Prion protein at the crossroads of physiology and disease. Trends Neurosci (2011) 1.26
Stress-protective signalling of prion protein is corrupted by scrapie prions. EMBO J (2008) 1.18
The comprehensive native interactome of a fully functional tagged prion protein. PLoS One (2009) 1.13
Retrotranslocation of prion proteins from the endoplasmic reticulum by preventing GPI signal transamidation. Mol Biol Cell (2008) 1.12
Neurotoxic mutants of the prion protein induce spontaneous ionic currents in cultured cells. J Biol Chem (2010) 1.12
Prions, prionoids and pathogenic proteins in Alzheimer disease. Prion (2012) 1.11
Review: contribution of transgenic models to understanding human prion disease. Neuropathol Appl Neurobiol (2010) 1.11
A novel, drug-based, cellular assay for the activity of neurotoxic mutants of the prion protein. J Biol Chem (2009) 1.11
Zinc drives a tertiary fold in the prion protein with familial disease mutation sites at the interface. Structure (2013) 1.08
Dissociation of infectivity from seeding ability in prions with alternate docking mechanism. PLoS Pathog (2011) 1.07
A naturally occurring C-terminal fragment of the prion protein (PrP) delays disease and acts as a dominant-negative inhibitor of PrPSc formation. J Biol Chem (2011) 1.07
An N-terminal polybasic domain and cell surface localization are required for mutant prion protein toxicity. J Biol Chem (2011) 1.03
Early onset prion disease from octarepeat expansion correlates with copper binding properties. PLoS Pathog (2009) 1.03
Prion neurotoxicity: insights from prion protein mutants. Curr Issues Mol Biol (2009) 1.02
Beta-amyloid oligomers and cellular prion protein in Alzheimer's disease. J Mol Med (Berl) (2009) 0.99
A nine amino acid domain is essential for mutant prion protein toxicity. J Neurosci (2011) 0.98
Prion protein with an octapeptide insertion has impaired neuroprotective activity in transgenic mice. EMBO J (2007) 0.98
Prion protein biosynthesis and its emerging role in neurodegeneration. Trends Biochem Sci (2009) 0.97
Fishing for prion protein function. PLoS Biol (2009) 0.97
Endocytosis of prion protein is required for ERK1/2 signaling induced by stress-inducible protein 1. J Neurosci (2008) 0.96
Cellular prion protein mediates the toxicity of beta-amyloid oligomers: implications for Alzheimer disease. Arch Neurol (2009) 0.96
Protease-resistant prions selectively decrease Shadoo protein. PLoS Pathog (2011) 0.95
Context dependent neuroprotective properties of prion protein (PrP). Prion (2009) 0.95
Proteasomal dysfunction and endoplasmic reticulum stress enhance trafficking of prion protein aggregates through the secretory pathway and increase accumulation of pathologic prion protein. J Biol Chem (2011) 0.94
Functionally relevant domains of the prion protein identified in vivo. PLoS One (2009) 0.94
Unexpected tolerance of alpha-cleavage of the prion protein to sequence variations. PLoS One (2010) 0.94
A new paradigm for enzymatic control of α-cleavage and β-cleavage of the prion protein. J Biol Chem (2013) 0.92
The N-terminal, polybasic region is critical for prion protein neuroprotective activity. PLoS One (2011) 0.91
Dominant-negative effects of the N-terminal half of prion protein on neurotoxicity of prion protein-like protein/doppel in mice. J Biol Chem (2008) 0.90
Conserved stress-protective activity between prion protein and Shadoo. J Biol Chem (2011) 0.90
Perturbation of T-cell development by insertional mutation of a PrP transgene. Immunology (2008) 0.89
The elusive role of the prion protein and the mechanism of toxicity in prion disease. PLoS Pathog (2015) 0.89
Ion channels induced by the prion protein: mediators of neurotoxicity. Prion (2012) 0.87
Cellular prion protein and NMDA receptor modulation: protecting against excitotoxicity. Front Cell Dev Biol (2014) 0.87
Five questions on prion diseases. PLoS Pathog (2012) 0.86
ZyFISH: a simple, rapid and reliable zygosity assay for transgenic mice. PLoS One (2012) 0.86
Cellular prion protein: from physiology to pathology. Viruses (2012) 0.85
Doppel induces degeneration of cerebellar Purkinje cells independently of Bax. Am J Pathol (2007) 0.85
Prion protein (PrP) gene-knockout cell lines: insight into functions of the PrP. Front Cell Dev Biol (2015) 0.85
Is, indeed, the prion protein a Harlequin servant of "many" masters? Prion (2009) 0.84
A highly toxic cellular prion protein induces a novel, nonapoptotic form of neuronal death. Am J Pathol (2010) 0.83
A Drug-Based Cellular Assay (DBCA) for studying cytotoxic and cytoprotective activities of the prion protein: A practical guide. Methods (2010) 0.83
The sheddase ADAM10 is a potent modulator of prion disease. Elife (2015) 0.83
Integrity of helix 2-helix 3 domain of the PrP protein is not mandatory for prion replication. J Biol Chem (2012) 0.82
Prion protein lacks robust cytoprotective activity in cultured cells. Mol Neurodegener (2008) 0.82
The toxicity of a mutant prion protein is cell-autonomous, and can be suppressed by wild-type prion protein on adjacent cells. PLoS One (2012) 0.82
Conserved roles of the prion protein domains on subcellular localization and cell-cell adhesion. PLoS One (2013) 0.81
Neurodegeneration and unfolded-protein response in mice expressing a membrane-tethered flexible tail of PrP. PLoS One (2015) 0.81
Generating Bona Fide Mammalian Prions with Internal Deletions. J Virol (2016) 0.81
A C-terminal membrane anchor affects the interactions of prion proteins with lipid membranes. J Biol Chem (2014) 0.80
PrP overdrive: does inhibition of α-cleavage contribute to PrP(C) toxicity and prion disease? Prion (2014) 0.80
De novo mammalian prion synthesis. Prion (2009) 0.79
Doppel and PrPC co-immunoprecipitate in detergent-resistant membrane domains of epithelial FRT cells. Biochem J (2009) 0.79
Neurotoxicity of prion peptides mimicking the central domain of the cellular prion protein. PLoS One (2013) 0.79
Antagonistic roles of the N-terminal domain of prion protein to doppel. Prion (2008) 0.78
Prion protein on astrocytes or in extracellular fluid impedes neurodegeneration induced by truncated prion protein. Exp Neurol (2009) 0.78
Involvement of PrP(C) in kainate-induced excitotoxicity in several mouse strains. Sci Rep (2015) 0.78
The non-octarepeat copper binding site of the prion protein is a key regulator of prion conversion. Sci Rep (2015) 0.78
Role of prions in neuroprotection and neurodegeneration: a mechanism involving glutamate receptors? Prion (2009) 0.78
Proteolysis of prion protein by cathepsin S generates a soluble beta-structured intermediate oligomeric form, with potential implications for neurotoxic mechanisms. Eur Biophys J (2008) 0.77
Mutated but Not Deleted Ovine PrPC N-Terminal Polybasic Region Strongly Interferes with Prion Propagation in Transgenic Mice. J Virol (2015) 0.77
The biological function of the cellular prion protein: an update. BMC Biol (2017) 0.76
Evolutionary implications of metal binding features in different species' prion protein: an inorganic point of view. Biomolecules (2014) 0.76
Disease-associated mutations in the prion protein impair laminin-induced process outgrowth and survival. J Biol Chem (2012) 0.76
A cationic tetrapyrrole inhibits toxic activities of the cellular prion protein. Sci Rep (2016) 0.76
Expression of the Prion Protein Family Member Shadoo Causes Drug Hypersensitivity That Is Diminished by the Coexpression of the Wild Type Prion Protein. J Biol Chem (2015) 0.76
Mammalian prions: tolerance to sequence changes-how far? Prion (2012) 0.76
The Cellular Prion Protein: A Player in Immunological Quiescence. Front Immunol (2015) 0.76
Structural transitions in full-length human prion protein detected by xenon as probe and spin labeling of the N-terminal domain. Sci Rep (2016) 0.75
Identification of Anti-prion Compounds using a Novel Cellular Assay. J Biol Chem (2016) 0.75
Effects of FlAsH/tetracysteine (TC) Tag on PrP proteolysis and PrPres formation by TC-scanning. Chembiochem (2013) 0.75
Regulation of RhoA activity by the cellular prion protein. Cell Death Dis (2017) 0.75
Unique structural properties associated with mouse prion Δ105-125 protein. Prion (2013) 0.75
The prion protein family member Shadoo induces spontaneous ionic currents in cultured cells. Sci Rep (2016) 0.75
Myelin damage due to local quantitative abnormalities in normal prion levels: evidence from subacute combined degeneration and multiple sclerosis. J Neurol (2013) 0.75
Dual role of cellular prion protein in normal host and Alzheimer's disease. Proc Jpn Acad Ser B Phys Biol Sci (2017) 0.75
Novel proteinaceous infectious particles cause scrapie. Science (1982) 24.09
Mice devoid of PrP are resistant to scrapie. Cell (1993) 11.46
Normal development and behaviour of mice lacking the neuronal cell-surface PrP protein. Nature (1992) 9.01
A cellular gene encodes scrapie PrP 27-30 protein. Cell (1985) 8.86
Prion protein (PrP) with amino-proximal deletions restoring susceptibility of PrP knockout mice to scrapie. EMBO J (1996) 6.66
Normal host prion protein necessary for scrapie-induced neurotoxicity. Nature (1996) 4.25
A transmembrane form of the prion protein in neurodegenerative disease. Science (1998) 3.76
Expression of amino-terminally truncated PrP in the mouse leading to ataxia and specific cerebellar lesions. Cell (1998) 3.59
Mammalian prion biology: one century of evolving concepts. Cell (2004) 3.46
A vector for expressing foreign genes in the brains and hearts of transgenic mice. Genet Anal (1996) 3.18
Signal transduction through prion protein. Science (2000) 3.09
Neurotoxicity and neurodegeneration when PrP accumulates in the cytosol. Science (2002) 3.05
Ataxia in prion protein (PrP)-deficient mice is associated with upregulation of the novel PrP-like protein doppel. J Mol Biol (1999) 2.92
Coexistence of multiple PrPSc types in individuals with Creutzfeldt-Jakob disease. Lancet Neurol (2005) 2.91
Characterization of detergent-insoluble complexes containing the cellular prion protein and its scrapie isoform. J Biol Chem (1997) 2.48
Prions prevent neuronal cell-line death. Nature (1999) 2.13
Cross-linking cellular prion protein triggers neuronal apoptosis in vivo. Science (2004) 2.00
Nearly ubiquitous tissue distribution of the scrapie agent precursor protein. Neurology (1992) 2.00
COOH-terminal sequence of the cellular prion protein directs subcellular trafficking and controls conversion into the scrapie isoform. Proc Natl Acad Sci U S A (1997) 1.89
Onset of ataxia and Purkinje cell loss in PrP null mice inversely correlated with Dpl level in brain. EMBO J (2001) 1.85
A mouse prion protein transgene rescues mice deficient for the prion protein gene from purkinje cell degeneration and demyelination. Lab Invest (1999) 1.83
Prion protein-deficient cells show altered response to oxidative stress due to decreased SOD-1 activity. Exp Neurol (1997) 1.78
Prion protein protects human neurons against Bax-mediated apoptosis. J Biol Chem (2001) 1.77
Stress-inducible protein 1 is a cell surface ligand for cellular prion that triggers neuroprotection. EMBO J (2002) 1.58
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 prion protein transduces neuroprotective signals. EMBO J (2002) 1.47
Three-dimensional structures of prion proteins. Adv Protein Chem (2001) 1.46
Truncated prion protein and Doppel are myelinotoxic in the absence of oligodendrocytic PrPC. J Neurosci (2005) 1.25
Prion protein is a component of the multimolecular signaling complex involved in T cell activation. FEBS Lett (2004) 1.12
Cellular prion protein sensitizes neurons to apoptotic stimuli through Mdm2-regulated and p53-dependent caspase 3-like activation. J Biol Chem (2003) 1.08
No superoxide dismutase activity of cellular prion protein in vivo. Biol Chem (2003) 1.05
Perspectives: neurobiology. PrP's double causes trouble. Science (1999) 1.04
Pronuclear microinjection of mouse zygotes. Methods Mol Biol (2004) 1.00
The role of PrP in health and disease. Curr Mol Med (2004) 0.98
Overexpression of PrPc triggers caspase 3 activation: potentiation by proteasome inhibitors and blockade by anti-PrP antibodies. J Neurochem (2002) 0.98
Primary cultured neurons devoid of cellular prion display lower responsiveness to staurosporine through the control of p53 at both transcriptional and post-transcriptional levels. J Biol Chem (2003) 0.97
Neuropathologic diagnostic and nosologic criteria for frontotemporal lobar degeneration: consensus of the Consortium for Frontotemporal Lobar Degeneration. Acta Neuropathol (2007) 8.10
Transmission and spreading of tauopathy in transgenic mouse brain. Nat Cell Biol (2009) 7.50
Microglia in the adult brain arise from Ly-6ChiCCR2+ monocytes only under defined host conditions. Nat Neurosci (2007) 6.23
Senescence surveillance of pre-malignant hepatocytes limits liver cancer development. Nature (2011) 6.14
Specific and nonhepatotoxic degradation of nuclear hepatitis B virus cccDNA. Science (2014) 5.47
Experimental autoimmune encephalomyelitis repressed by microglial paralysis. Nat Med (2005) 4.42
IFIT1 is an antiviral protein that recognizes 5'-triphosphate RNA. Nat Immunol (2011) 4.18
Peripherally applied Abeta-containing inoculates induce cerebral beta-amyloidosis. Science (2010) 3.98
ATGL-mediated fat catabolism regulates cardiac mitochondrial function via PPAR-α and PGC-1. Nat Med (2011) 3.96
An analytical solution to the kinetics of breakable filament assembly. Science (2009) 3.87
p62 Is a common component of cytoplasmic inclusions in protein aggregation diseases. Am J Pathol (2002) 3.80
Reversible microbial colonization of germ-free mice reveals the dynamics of IgA immune responses. Science (2010) 3.53
The AP-1 transcription factor c-Jun is required for efficient axonal regeneration. Neuron (2004) 3.33
TAK1 suppresses a NEMO-dependent but NF-kappaB-independent pathway to liver cancer. Cancer Cell (2010) 3.25
The Salmonella pathogenicity island (SPI)-2 and SPI-1 type III secretion systems allow Salmonella serovar typhimurium to trigger colitis via MyD88-dependent and MyD88-independent mechanisms. J Immunol (2005) 3.14
Microglia emerge from erythromyeloid precursors via Pu.1- and Irf8-dependent pathways. Nat Neurosci (2013) 3.11
Bi-directional interconversion of brite and white adipocytes. Nat Cell Biol (2013) 3.08
Disseminated and sustained HIV infection in CD34+ cord blood cell-transplanted Rag2-/-gamma c-/- mice. Proc Natl Acad Sci U S A (2006) 3.07
Coexistence of multiple PrPSc types in individuals with Creutzfeldt-Jakob disease. Lancet Neurol (2005) 2.91
Formation and maintenance of Alzheimer's disease beta-amyloid plaques in the absence of microglia. Nat Neurosci (2009) 2.85
Extraneural pathologic prion protein in sporadic Creutzfeldt-Jakob disease. N Engl J Med (2003) 2.79
Triggering TLR7 in mice induces immune activation and lymphoid system disruption, resembling HIV-mediated pathology. Blood (2008) 2.65
Genetic prion disease: the EUROCJD experience. Hum Genet (2005) 2.64
A lymphotoxin-driven pathway to hepatocellular carcinoma. Cancer Cell (2009) 2.64
Immunoprivileged status of the liver is controlled by Toll-like receptor 3 signaling. J Clin Invest (2006) 2.63
Prion strain discrimination using luminescent conjugated polymers. Nat Methods (2007) 2.62
Mutations in the gene encoding PDGF-B cause brain calcifications in humans and mice. Nat Genet (2013) 2.62
Prevalence of Fabry disease in patients with cryptogenic stroke: a prospective study. Lancet (2005) 2.59
Follicular dendritic cells emerge from ubiquitous perivascular precursors. Cell (2012) 2.50
Insights into prion strains and neurotoxicity. Nat Rev Mol Cell Biol (2007) 2.34
Brain homogenates from human tauopathies induce tau inclusions in mouse brain. Proc Natl Acad Sci U S A (2013) 2.27
Induction of cerebral beta-amyloidosis: intracerebral versus systemic Abeta inoculation. Proc Natl Acad Sci U S A (2009) 2.27
Axonal prion protein is required for peripheral myelin maintenance. Nat Neurosci (2010) 2.25
Prion protein and Abeta-related synaptic toxicity impairment. EMBO Mol Med (2010) 2.25
The microbiota mediates pathogen clearance from the gut lumen after non-typhoidal Salmonella diarrhea. PLoS Pathog (2010) 2.17
Lymphoid follicle destruction and immunosuppression after repeated CpG oligodeoxynucleotide administration. Nat Med (2004) 2.13
De novo generation of a transmissible spongiform encephalopathy by mouse transgenesis. Proc Natl Acad Sci U S A (2008) 2.10
The propagation of prion-like protein inclusions in neurodegenerative diseases. Trends Neurosci (2010) 2.10
Chronic lymphocytic inflammation specifies the organ tropism of prions. Science (2005) 2.07
An essential function for NBS1 in the prevention of ataxia and cerebellar defects. Nat Med (2005) 2.06
A new type of microglia gene targeting shows TAK1 to be pivotal in CNS autoimmune inflammation. Nat Neurosci (2013) 2.03
Nonredundant function of soluble LTα3 produced by innate lymphoid cells in intestinal homeostasis. Science (2013) 2.03
Coincident scrapie infection and nephritis lead to urinary prion excretion. Science (2005) 1.94
Microbe sampling by mucosal dendritic cells is a discrete, MyD88-independent step in DeltainvG S. Typhimurium colitis. J Exp Med (2008) 1.92
Maturation of lymph node fibroblastic reticular cells from myofibroblastic precursors is critical for antiviral immunity. Immunity (2013) 1.89
E6 and E7 from beta HPV38 cooperate with ultraviolet light in the development of actinic keratosis-like lesions and squamous cell carcinoma in mice. PLoS Pathog (2011) 1.89
T cells expressing a chimeric antigen receptor that binds hepatitis B virus envelope proteins control virus replication in mice. Gastroenterology (2013) 1.77
Induction of tau pathology by intracerebral infusion of amyloid-beta -containing brain extract and by amyloid-beta deposition in APP x Tau transgenic mice. Am J Pathol (2007) 1.77
Hepatocyte-specific deletion of the antiapoptotic protein myeloid cell leukemia-1 triggers proliferation and hepatocarcinogenesis in mice. Hepatology (2010) 1.75
Deposition of Alzheimer's beta-amyloid is inversely correlated with P-glycoprotein expression in the brains of elderly non-demented humans. Pharmacogenetics (2002) 1.75
IL-6 is required for glioma development in a mouse model. Oncogene (2004) 1.74
The S. Typhimurium effector SopE induces caspase-1 activation in stromal cells to initiate gut inflammation. Cell Host Microbe (2009) 1.74
Quantitative analysis of the binding affinity of poly(ADP-ribose) to specific binding proteins as a function of chain length. Nucleic Acids Res (2007) 1.73
Aerosols transmit prions to immunocompetent and immunodeficient mice. PLoS Pathog (2011) 1.71
Neonatal hepatic steatosis by disruption of the adenosine kinase gene. Proc Natl Acad Sci U S A (2002) 1.70
Cell biology: Beyond the prion principle. Nature (2009) 1.70
The POM monoclonals: a comprehensive set of antibodies to non-overlapping prion protein epitopes. PLoS One (2008) 1.70
Hippocampal sclerosis dementia: a reappraisal. Acta Neuropathol (2007) 1.68
PrPSc in mammary glands of sheep affected by scrapie and mastitis. Nat Med (2005) 1.66
Follicular dendritic cells control engulfment of apoptotic bodies by secreting Mfge8. J Exp Med (2008) 1.65
Sorafenib perpetuates cellular anticancer effector functions by modulating the crosstalk between macrophages and natural killer cells. Hepatology (2013) 1.62
Pathogenesis of prion diseases: current status and future outlook. Nat Rev Microbiol (2006) 1.60
Chemokine-driven lymphocyte infiltration: an early intratumoural event determining long-term survival in resectable hepatocellular carcinoma. Gut (2011) 1.56
Endothelial CCR2 signaling induced by colon carcinoma cells enables extravasation via the JAK2-Stat5 and p38MAPK pathway. Cancer Cell (2012) 1.55
Stimulation of autophagy reduces neurodegeneration in a mouse model of human tauopathy. Brain (2012) 1.55
The transcriptional regulator PLZF induces the development of CD44 high memory phenotype T cells. Proc Natl Acad Sci U S A (2008) 1.54
Loss of hypocretin (orexin) neurons with traumatic brain injury. Ann Neurol (2009) 1.54
Comparison of a new self-gripping mesh with other fixation methods for laparoscopic hernia repair in a rat model. J Am Coll Surg (2009) 1.53
Parthenogenetic embryo-like structures in the human ovarian surface epithelium cell culture in postmenopausal women with no naturally present follicles and oocytes. Stem Cells Dev (2009) 1.52
The prion protein knockout mouse: a phenotype under challenge. Prion (2007) 1.51
Positioning of follicular dendritic cells within the spleen controls prion neuroinvasion. Nature (2003) 1.51
Astrogliosis in epilepsy leads to overexpression of adenosine kinase, resulting in seizure aggravation. Brain (2005) 1.51
The complex PrP(c)-Fyn couples human oligomeric Aβ with pathological tau changes in Alzheimer's disease. J Neurosci (2012) 1.50
A novel Ncr1-Cre mouse reveals the essential role of STAT5 for NK-cell survival and development. Blood (2010) 1.50
Characterization of the interferon-producing cell in mice infected with Listeria monocytogenes. PLoS Pathog (2009) 1.50
Human prion diseases: molecular and clinical aspects. Arch Neurol (2005) 1.49
The strain-encoded relationship between PrP replication, stability and processing in neurons is predictive of the incubation period of disease. PLoS Pathog (2011) 1.49
Chronic subclinical prion disease induced by low-dose inoculum. J Virol (2002) 1.47
Global democratic consensus on neuropathological disease criteria. Lancet Neurol (2002) 1.44
Microglial repopulation model reveals a robust homeostatic process for replacing CNS myeloid cells. Proc Natl Acad Sci U S A (2012) 1.43
Lymphotoxin β receptor signaling promotes development of autoimmune pancreatitis. Gastroenterology (2012) 1.43
An impaired neocortical Ih is associated with enhanced excitability and absence epilepsy. Eur J Neurosci (2004) 1.43
Manganese superoxide dismutase and aldehyde dehydrogenase deficiency increase mitochondrial oxidative stress and aggravate age-dependent vascular dysfunction. Cardiovasc Res (2008) 1.43
Analysis of prion strains by PrPSc profiling in sporadic Creutzfeldt-Jakob disease. PLoS Med (2005) 1.43
Novel pentameric thiophene derivatives for in vitro and in vivo optical imaging of a plethora of protein aggregates in cerebral amyloidoses. ACS Chem Biol (2009) 1.42