The immunobiology of prion diseases

Novel Proteinaceous Infectious Particles Cause Scrapie

1982 • 4,972 citations

Immunization with amyloid-β attenuates Alzheimer-disease-like pathology in the PDAPP mouse

1999 • 3,112 citations

A new variant of Creutzfeldt-Jakob disease in the UK

1996 • 2,690 citations

Conversion of alpha-helices into beta-sheets features in the formation of the scrapie prion proteins.

1993 • 2,237 citations

Mice devoid of PrP are resistant to scrapie

1993 • 2,058 citations

Normal development and behaviour of mice lacking the neuronal cell-surface PrP protein

1992 • 1,632 citations

Cellular prion protein mediates impairment of synaptic plasticity by amyloid-β oligomers

2009 • 1,465 citations

Possible transmission of variant Creutzfeldt-Jakob disease by blood transfusion

2004 • 1,123 citations

An Analytical Solution to the Kinetics of Breakable Filament Assembly

2009 • 1,061 citations

Cells release prions in association with exosomes

2004 • 967 citations

Transgenetic studies implicate interactions between homologous PrP isoforms in scrapie prion replication

1990 • 874 citations

Prion protein (PrP) with amino-proximal deletions restoring susceptibility of PrP knockout mice to scrapie.

1996 • 872 citations

The most infectious prion protein particles

2005 • 871 citations

Prion propagation in mice expressing human and chimeric PrP transgenes implicates the interaction of cellular PrP with another protein

1995 • 835 citations

Normal host prion protein necessary for scrapie-induced neurotoxicity

1996 • 791 citations

Microglia: Scapegoat, Saboteur, or Something Else?

2013 • 788 citations

Depleting Neuronal PrP in Prion Infection Prevents Disease and Reverses Spongiosis

2003 • 728 citations

Transgenic mice expressing hamster prion protein produce species-specific scrapie infectivity and amyloid plaques

1989 • 696 citations

Generating a Prion with Bacterially Expressed Recombinant Prion Protein

2010 • 639 citations

Alzheimer amyloid-β oligomer bound to postsynaptic prion protein activates Fyn to impair neurons

2012 • 591 citations

Physiology of the Prion Protein

2008 • 587 citations

Prions hijack tunnelling nanotubes for intercellular spread

2009 • 572 citations

Degenerative Disease of the Central Nervous System in New Guinea

1957 • 570 citations

Expression of Amino-Terminally Truncated PrP in the Mouse Leading to Ataxia and Specific Cerebellar Lesions

1998 • 529 citations

Ataxia in prion protein (PrP)-deficient mice is associated with upregulation of the novel PrP-like protein doppel

1999 • 523 citations

Microglial Cx3cr1 knockout prevents neuron loss in a mouse model of Alzheimer's disease

2010 • 521 citations

Antibodies inhibit prion propagation and clear cell cultures of prion infectivity

2001 • 520 citations

Infectious Prions in the Saliva and Blood of Deer with Chronic Wasting Disease

2006 • 518 citations

A crucial role for B cells in neuroinvasive scrapie

1997 • 503 citations

Loss of cerebellar Purkinje cells in aged mice homozygous for a disrupted PrP gene

1996 • 483 citations

Monoclonal antibodies inhibit prion replication and delay the development of prion disease

2003 • 477 citations

Synthetic amyloid-β oligomers impair long-term memory independently of cellular prion protein

2010 • 451 citations

Lymphoid follicle destruction and immunosuppression after repeated CpG oligodeoxynucleotide administration

2004 • 437 citations

Diagnosis of new variant Creutzfeldt-Jakob disease by tonsil biopsy

1997 • 431 citations

Scrapie prion protein accumulation by scrapie-infected neuroblastoma cells abrogated by exposure to a prion protein antibody

2001 • 425 citations

Memory Impairment in Transgenic Alzheimer Mice Requires Cellular Prion Protein

2010 • 397 citations

Axonal prion protein is required for peripheral myelin maintenance

2010 • 375 citations

Scrapie replication in lymphoid tissues depends on prion protein-expressing follicular dendritic cells

1999 • 367 citations

Follicular Dendritic Cells Emerge from Ubiquitous Perivascular Precursors

2012 • 364 citations

Cross-Linking Cellular Prion Protein Triggers Neuronal Apoptosis in Vivo

2004 • 360 citations

Impaired Prion Replication in Spleens of Mice Lacking Functional Follicular Dendritic Cells

2000 • 354 citations

Prevention of Scrapie Pathogenesis by Transgenic Expression of Anti-Prion Protein Antibodies

2001 • 348 citations

Oral transmission and early lymphoid tropism of chronic wasting disease PrPres in mule deer fawns (Odocoileus hemionus )

1999 • 335 citations

Complement facilitates early prion pathogenesis

2001 • 320 citations

Abnormal isoform of prion protein accumulates in follicular dendritic cells in mice with Creutzfeldt-Jakob disease

1991 • 290 citations

PrP expression in B lymphocytes is not required for prion neuroinvasion

1998 • 271 citations

PrP-expressing tissue required for transfer of scrapie infectivity from spleen to brain

1997 • 269 citations

The Complex PrP^c-Fyn Couples Human Oligomeric Aβ with Pathological Tau Changes in Alzheimer's Disease

2012 • 259 citations

High Prion and PrPSc Levels but Delayed Onset of Disease in Scrapie-Inoculated Mice Heterozygous for a Disrupted PrP Gene

1994 • 245 citations

Prion protein and Aβ‐related synaptic toxicity impairment

2010 • 241 citations

Surgical treatment and risk of sporadic Creutzfeldt-Jakob disease: a case-control study

1999 • 236 citations

Expression of fractalkine (CX3CL1) and its receptor, CX3CR1, during acute and chronic inflammation in the rodent CNS

2002 • 233 citations

Sympathetic Innervation of Lymphoreticular Organs Is Rate Limiting for Prion Neuroinvasion

2001 • 229 citations

Temporary depletion of complement component C3 or genetic deficiency of C1q significantly delays onset of scrapie

2001 • 227 citations

Pathogenesis of scrapie in mice after intragastric infection

1989 • 216 citations

Positioning of follicular dendritic cells within the spleen controls prion neuroinvasion

2003 • 216 citations

Migrating intestinal dendritic cells transport PrPSc from the gut

2002 • 210 citations

Immunoaffinity purification and neutralization of scrapie prion infectivity.

1988 • 205 citations

The toxicity of antiprion antibodies is mediated by the flexible tail of the prion protein

2013 • 198 citations

Pathogenesis of natural scrapie in sheep

2000 • 193 citations

Chronic Lymphocytic Inflammation Specifies the Organ Tropism of Prions

2005 • 192 citations

Immunization Delays the Onset of Prion Disease in Mice

2002 • 190 citations

De novo generation of a transmissible spongiform encephalopathy by mouse transgenesis

2008 • 187 citations

Mice with gene targetted prion protein alterations show that Prnp, Sine and Prni are congruent

1998 • 185 citations

Coincident Scrapie Infection and Nephritis Lead to Urinary Prion Excretion

2005 • 182 citations

Pathogenesis of Scrapie in the Mouse: the Role of the Spleen

1970 • 170 citations

Follicular Dendritic Cell Dedifferentiation by Treatment with an Inhibitor of the Lymphotoxin Pathway Dramatically Reduces Scrapie Susceptibility

2003 • 152 citations

Role of spleen macrophages in the clearance of scrapie agent early in pathogenesis

2000 • 145 citations

PrPCWD in the myenteric plexus, vagosympathetic trunk and endocrine glands of deer with chronic wasting disease

2001 • 144 citations

Characterization of the microglial response in murine scrapie

1994 • 144 citations

Immune system-dependent and -independent replication of the scrapie agent

1996 • 144 citations

Lymph nodal prion replication and neuroinvasion in mice devoid of follicular dendritic cells

2002 • 143 citations

Oral Prion Infection Requires Normal Numbers of Peyer's Patches but Not of Enteric Lymphocytes

2003 • 139 citations

Postexposure prophylaxis against prion disease with a stimulator of innate immunity

2002 • 138 citations

A versatile prion replication assay in organotypic brain slices

2007 • 138 citations

Facilitated Cross-Species Transmission of Prions in Extraneural Tissue

2012 • 137 citations

Activation of cerebral cytokine gene expression and its correlation with onset of reactive astrocyte and acute-phase response gene expression in scrapie

1994 • 136 citations

Soluble Dimeric Prion Protein Binds PrPSc In Vivo and Antagonizes Prion Disease

2003 • 135 citations

Pathological PrP is abundant in sympathetic and sensory ganglia of hamsters fed with scrapie

1999 • 132 citations

Genes contributing to prion pathogenesis

2008 • 127 citations

The role of the spleen in the neuroinvasion of scrapie in mice

1989 • 127 citations

Tumor Necrosis Factor Alpha-Deficient, but Not Interleukin-6-Deficient, Mice Resist Peripheral Infection with Scrapie

2000 • 126 citations

Expression of cytokine genes and increased nuclear factor-kappa B activity in the brains of scrapie-infected mice

1999 • 122 citations

Microglia from Creutzfeldt-Jakob Disease-Infected Brains Are Infectious and Show Specific mRNA Activation Profiles

2002 • 121 citations

Prion Uptake in the Gut: Identification of the First Uptake and Replication Sites

2011 • 117 citations

The cellular prion protein modulates phagocytosis and inflammatory response

2004 • 114 citations

Anti-prion antibodies for prophylaxis following prion exposure in mice

2003 • 112 citations

Failure to Demonstrate a Humoral Immune Response to Scrapie Infection in Mice

1973 • 111 citations

Humoral immune response to native eukaryotic prion protein correlates with anti-prion protection

2004 • 110 citations

Engulfment of cerebral apoptotic bodies controls the course of prion disease in a mouse strain–dependent manner

2010 • 107 citations

Prions in Milk from Ewes Incubating Natural Scrapie

2008 • 100 citations

M cell-depletion blocks oral prion disease pathogenesis

2012 • 97 citations

Identification of Upregulated Genes in Scrapie-Infected Brain Tissue

2000 • 97 citations

Role of Interleukin-1 in Prion Disease-Associated Astrocyte Activation

2004 • 95 citations

Follicular Dendritic Cell-Specific Prion Protein (PrPc) Expression Alone Is Sufficient to Sustain Prion Infection in the Spleen

2011 • 95 citations

Microglia in the degenerating brain are capable of phagocytosis of beads and of apoptotic cells, but do not efficiently remove PrP^Sc, even upon LPS stimulation

2010 • 93 citations

Immunisation with a synthetic prion protein-derived peptide prolongs survival times of mice orally exposed to the scrapie agent

2003 • 93 citations

Heightened expression of tumor necrosis factor alpha, interleukin 1 alpha, and glial fibrillary acidic protein in experimental Creutzfeldt-Jakob disease in mice.

1996 • 90 citations

Microglial Activation Varies in Different Models of Creutzfeldt-Jakob Disease

1999 • 89 citations

Ionising radiation has no influence on scrapie incubation period in mice

1987 • 87 citations

Cutting Edge: Brucella abortus Exploits a Cellular Prion Protein on Intestinal M Cells as an Invasive Receptor

2012 • 84 citations

Multiplication of Scrapie Agent in Mouse Spleen

1971 • 80 citations

Immunohistochemical study of microglia in the Creutzfeldt-Jakob diseased brain

1993 • 80 citations

Prion pathogenesis in the absence of Toll‐like receptor signalling

2003 • 79 citations

Modulation of proteinase-K resistant prion protein by prion peptide immunization

2001 • 79 citations

The sympathetic nervous system is involved in variant Creutzfeldt-Jakob disease

2003 • 78 citations

Prion Pathogenesis Is Faithfully Reproduced in Cerebellar Organotypic Slice Cultures

2012 • 76 citations

Accelerated Prion Disease in the Absence of Interleukin-10

2004 • 75 citations

Efficient Lymphoreticular Prion Propagation Requires PrP^cin Stromal and Hematopoietic Cells

2001 • 75 citations

Antiprion immunotherapy: to suppress or to stimulate?

2004 • 75 citations

Pathogenesis of mouse scrapie: Distribution of agent in the pulp and stroma of infected spleens

1984 • 73 citations

Stromal Complement Receptor CD21/35 Facilitates Lymphoid Prion Colonization and Pathogenesis

2007 • 72 citations

Aerosols Transmit Prions to Immunocompetent and Immunodeficient Mice

2011 • 72 citations

B lymphocyte-restricted expression of prion protein does not enable prion replication in prion protein knockout mice

2001 • 71 citations

Disease-associated PrP in the enteric nervous system of scrapie-affected Suffolk sheep

2003 • 71 citations

SIRPα polymorphisms, but not the prion protein, control phagocytosis of apoptotic cells

2013 • 70 citations

Absence of Detectable IL-1β Production in Murine Prion Disease: A Model of Chronic Neurodegeneration

2001 • 70 citations

Retrograde Transport of Transmissible Mink Encephalopathy within Descending Motor Tracts

2002 • 68 citations

MCP-1 and murine prion disease: Separation of early behavioural dysfunction from overt clinical disease

2005 • 68 citations

Aerosol Transmission of Chronic Wasting Disease in White-Tailed Deer

2012 • 67 citations

Requirement of Cellular Prion Protein for Intestinal Barrier Function and Mislocalization in Patients With Inflammatory Bowel Disease

2012 • 66 citations

In Vivo Depletion of CD11c+ Cells Impairs Scrapie Agent Neuroinvasion from the Intestine

2007 • 65 citations

CNS Delivery of Vectored Prion-specific Single-chain Antibodies Delays Disease Onset

2008 • 64 citations

Further Observations on the Experimental Production of Scrapie in Goats and Sheep

1960 • 63 citations

High titers of mucosal and systemic anti-PrP antibodies abrogate oral prion infection in mucosal-vaccinated mice

2008 • 63 citations

In vitro Interaction of Scrapie Agent and Mouse Peritoneal Macrophages

1981 • 61 citations

Prion infectivity in the spleen of a PRNP heterozygous individual with subclinical variant Creutzfeldt–Jakob disease

2013 • 59 citations

Temporary Blockade of the Tumor Necrosis Factor Receptor Signaling Pathway Impedes the Spread of Scrapie to the Brain

2002 • 58 citations

PATHOGENESIS OF MOUSE SCRAPIE: DYNAMICS OF VACUOLATION IN BRAIN AND SPINAL CORD AFTER INTRAPERITONEAL INFECTION

1985 • 57 citations

Temporal distribution of transmissible mink encephalopathy virus in mink inoculated subcutaneously

1987 • 57 citations

Sheep with Scrapie and Mastitis Transmit Infectious Prions through the Milk

2010 • 56 citations

Lymphotoxin-Dependent Prion Replication in Inflammatory Stromal Cells of Granulomas

2008 • 55 citations

Accelerated Prion Disease Pathogenesis in Toll-Like Receptor 4 Signaling-Mutant Mice

2008 • 50 citations

Intranasal Inoculation of White-Tailed Deer (Odocoileus virginianus) with Lyophilized Chronic Wasting Disease Prion Particulate Complexed to Montmorillonite Clay

2013 • 49 citations

Heightened intrathecal release of proinflammatory cytokines in Creutzfeldt–Jakob disease

1999 • 48 citations

Isolation from Mouse Spleen of Cell Populations with High Specific Infectivity for Scrapie Virus

1972 • 47 citations

Interleukin 4 and Interleukin 10 Levels Are Elevated in the Cerebrospinal Fluid of Patients With Creutzfeldt-Jakob Disease

2005 • 47 citations

Murine Scrapie Infection Causes an Abnormal Germinal Centre Reaction in the Spleen

2004 • 46 citations

Prion Protein Expression Regulates Embryonic Stem Cell Pluripotency and Differentiation

2011 • 46 citations

Anti-PrP Mab 6D11 suppresses PrPSc replication in prion infected myeloid precursor line FDC-P1/22L and in the lymphoreticular system in vivo

2009 • 44 citations

Immunotherapy in prion disease

2012 • 43 citations

Evidence for Normal Cell-Mediated Immunity in Scrapie-Infected Mice

1981 • 42 citations

Accelerated Prion Replication in, but Prolonged Survival Times of, Prion-Infected CXCR3^−/−Mice

2008 • 41 citations

Scrapie transmission following exposure through the skin is dependent on follicular dendritic cells in lymphoid tissues

2004 • 41 citations

Abnormal Activation of Microglia Accompanied with Disrupted CX3CR1/CX3CL1 Pathway in the Brains of the Hamsters Infected with Scrapie Agent 263K

2013 • 39 citations

Evidence of effective scrapie transmission via colostrum and milk in sheep

2013 • 38 citations

Bacterial Colitis Increases Susceptibility to Oral Prion Disease

2008 • 38 citations

DNA Vaccination Can Break Immunological Tolerance to PrP in Wild-Type Mice and Attenuates Prion Disease after Intracerebral Challenge

2006 • 37 citations

Severely combined immunodeficient (SCID) mice resist infection with bovine spongiform encephalopathy.

1997 • 36 citations

Somatic mosaicism in a case of apparently sporadic Creutzfeldt‐Jakob disease carrying a de novo D178N mutation in the PRNP gene

2010 • 35 citations

Immunization with recombinant bovine but not mouse prion protein delays the onset of disease in mice inoculated with a mouse-adapted prion

2006 • 34 citations

Anti-prion activity generated by a novel vaccine formulation

2007 • 34 citations

Intranasal immunization of Balb/c mice against prion protein attenuates orally acquired transmissible spongiform encephalopathy

2006 • 33 citations

Abnormal Prion Protein in Ectopic Lymphoid Tissue in a Kidney of an Asymptomatic White-tailed Deer Experimentally Inoculated with the Agent of Chronic Wasting Disease

2006 • 33 citations

Biological Effects and Use of PrP ^Sc - and PrP-Specific Antibodies Generated by Immunization with Purified Full-Length Native Mouse Prions

2011 • 32 citations

Genetic Depletion of Complement Receptors CD21/35 Prevents Terminal Prion Disease in a Mouse Model of Chronic Wasting Disease

2012 • 31 citations

Alteration of NF-κB activity leads to mitochondrial apoptosis after infection with pathological prion protein

2007 • 30 citations

Protective Role of Interferon Regulatory Factor 3-Mediated Signaling against Prion Infection

2012 • 29 citations

Rapid Transepithelial Transport of Prions following Inhalation

2012 • 29 citations

Role of Erk1/2 activation in prion disease pathogenesis: Absence of CCR1 leads to increased Erk1/2 activation and accelerated disease progression

2008 • 29 citations

Toxic Effects of Intracerebral PrP Antibody Administration During the Course of BSE Infection in Mice

2007 • 28 citations

Follicular dendritic cell-specific prion protein (PrPc) expression is sufficient to sustain prion infection in the spleen

2011 • 27 citations

Mouse vaccination with dendritic cells loaded with prion protein peptides overcomes tolerance and delays scrapie

2009 • 27 citations

Investigation ofMcp1as a Quantitative Trait Gene for Prion Disease Incubation Time in Mouse

2008 • 26 citations

Sod1 Deficiency Reduces Incubation Time in Mouse Models of Prion Disease

2013 • 24 citations

Lack of Effect of Thymus and Spleen on the Incubation Period of Creutzfeldt-Jakob Disease in Mice

1987 • 23 citations

Role of the draining lymph node in scrapie agent transmission from the skin

2007 • 22 citations

Repetitive Immunization Enhances the Susceptibility of Mice to Peripherally Administered Prions

2009 • 22 citations

Unswitched immunoglobulin M response prolongs mouse survival in prion disease

2009 • 20 citations

Prion propagation in mice lacking central nervous system NF-κB signalling

2008 • 20 citations

Immunization with Recombinant Prion Protein Leads to Partial Protection in a Murine Model of TSEs through a Novel Mechanism

2013 • 20 citations

Dendritic Cell-Mediated-Immunization with Xenogenic PrP and Adenoviral Vectors Breaks Tolerance and Prolongs Mice Survival against Experimental Scrapie

2009 • 19 citations

Germinal center B cells are dispensable in prion transport and neuroinvasion

2007 • 18 citations

Antigenic mimicry-mediated anti-prion effects induced by bacterial enzyme succinylarginine dihydrolase in mice

2011 • 16 citations

Prion Replication in the Hematopoietic Compartment Is Not Required for Neuroinvasion in Scrapie Mouse Model

2010 • 14 citations

Immunisation strategies against prion diseases: Prime-boost immunisation with a PrP DNA vaccine containing foreign helper T-cell epitopes does not prevent mouse scrapie

2007 • 14 citations

Lymphotoxin, but Not TNF, Is Required for Prion Invasion of Lymph Nodes

2012 • 13 citations

Contribution of Antibody and T Cell-Specific Responses to the Progression of 139A-Scrapie in C57BL/6 Mice Immunized with Prion Protein Peptides

2008 • 12 citations