Molecular Aspects of Disease Pathogenesis in the Transmissible Spongiform Encephalopathies

Novel Proteinaceous Infectious Particles Cause Scrapie

1982 • 4,972 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

Seeding “one-dimensional crystallization” of amyloid: A pathogenic mechanism in Alzheimer's disease and scrapie?

1993 • 2,146 citations

Mice devoid of PrP are resistant to scrapie

1993 • 2,058 citations

Molecular Biology of Prion Diseases

1991 • 1,978 citations

Transmissions to mice indicate that ‘new variant’ CJD is caused by the BSE agent

1997 • 1,951 citations

Molecular analysis of prion strain variation and the aetiology of 'new variant' CJD

1996 • 1,740 citations

Identification of a Protein That Purifies with the Scrapie Prion

1982 • 1,260 citations

NMR structure of the mouse prion protein domain PrP(121–231)

1996 • 1,251 citations

Eight prion strains have PrPSc molecules with different conformations

1998 • 1,236 citations

Sensitive detection of pathological prion protein by cyclic amplification of protein misfolding

2001 • 1,228 citations

Nature of the Scrapie Agent: Self-replication and Scrapie

1967 • 1,128 citations

Possible transmission of variant Creutzfeldt-Jakob disease by blood transfusion

2004 • 1,123 citations

NMR solution structure of the human prion protein

2000 • 1,075 citations

A novel progressive spongiform encephalopathy in cattle

1987 • 1,062 citations

Synthetic Mammalian Prions

2004 • 1,006 citations

Nucleated Conformational Conversion and the Replication of Conformational Information by a Prion Determinant

2000 • 947 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

Cell-free formation of protease-resistant prion protein

1994 • 872 citations

Homozygous prion protein genotype predisposes to sporadic Creutzfeldt–Jakob disease

1991 • 871 citations

Evidence for the Conformation of the Pathologic Isoform of the Prion Protein Enciphering and Propagating Prion Diversity

1996 • 857 citations

Preclinical vCJD after blood transfusion in a PRNP codon 129 heterozygous patient

2004 • 846 citations

Molecular basis of phenotypic variability in sporadc creudeldt‐jakob disease

1996 • 832 citations

Secondary structure analysis of the scrapie-associated protein PrP 27-30 in water by infrared spectroscopy

1991 • 812 citations

Prion protein is necessary for normal synaptic function

1994 • 802 citations

Normal host prion protein necessary for scrapie-induced neurotoxicity

1996 • 791 citations

Signal Transduction Through Prion Protein

2000 • 786 citations

In Vitro Generation of Infectious Scrapie Prions

2005 • 765 citations

CHRONIC WASTING DISEASE OF CAPTIVE MULE DEER: A SPONGIFORM ENCEPHALOPATHY

1980 • 760 citations

Depleting Neuronal PrP in Prion Infection Prevents Disease and Reverses Spongiosis

2003 • 728 citations

A Transmembrane Form of the Prion Protein in Neurodegenerative Disease

1998 • 724 citations

NMR characterization of the full‐length recombinant murine prion protein, mPrP(23–231)

1997 • 720 citations

Investigation of variant Creutzfeldt-Jakob disease and other human prion diseases with tonsil biopsy samples

1999 • 710 citations

Sulfated Glycans Stimulate Endocytosis of the Cellular Isoform of the Prion Protein, PrPC, in Cultured Cells

1995 • 702 citations

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

1989 • 696 citations

Does the Agent of Scrapie Replicate without Nucleic Acid ?

1967 • 671 citations

Distinct PrP properties suggest the molecular basis of strain variation in transmissible mink encephalopathy

1994 • 663 citations

Altered circadian activity rhythms and sleep in mice devoid of prion protein

1996 • 655 citations

The scrapie-associated form of PrP is made from a cell surface precursor that is both protease- and phospholipase-sensitive.

1991 • 627 citations

Anchorless Prion Protein Results in Infectious Amyloid Disease Without Clinical Scrapie

2005 • 624 citations

Self-Seeded Fibers Formed by Sup35, the Protein Determinant of [PSI+], a Heritable Prion-like Factor of S. cerevisiae

1997 • 604 citations

Transmission dynamics and epidemiology of BSE in British cattle

1996 • 601 citations

Cholesterol depletion and modification of COOH-terminal targeting sequence of the prion protein inhibit formation of the scrapie isoform [published erratum appears in J Cell Biol 1995 Jul;130(2):501]

1995 • 541 citations

Spontaneous Neurodegeneration in Transgenic Mice with Mutant Prion Protein

1990 • 531 citations

Non-genetic propagation of strain-specific properties of scrapie prion protein

1995 • 517 citations

Scrapie and cellular prion proteins differ in their kinetics of synthesis and topology in cultured cells.

1990 • 510 citations

Crystal structure of the human prion protein reveals a mechanism for oligomerization.

2001 • 508 citations

Evidence for synthesis of scrapie prion proteins in the endocytic pathway.

1992 • 497 citations

RNA molecules stimulate prion protein conversion

2003 • 494 citations

Preclinical variant CJD after blood transfusion in a PRNP codon 129 heterozygous patient

2004 • 492 citations

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

1996 • 483 citations

Abnormal fibrils from scrapie-infected brain

1981 • 473 citations

Biochemical and physical properties of the prion protein from two strains of the transmissible mink encephalopathy agent

1992 • 440 citations

PrP Genotype and Agent Effects in Scrapie: Change In Allelic Interaction With Different Isolates of Agent in Sheep, a Natural Host of Scrapie

1994 • 440 citations

N-terminal truncation of the scrapie-associated form of PrP by lysosomal protease(s): implications regarding the site of conversion of PrP to the protease-resistant state

1991 • 412 citations

Sulfated polyanion inhibition of scrapie-associated PrP accumulation in cultured cells

1993 • 391 citations

Prion Protein Protects Human Neurons against Bax-mediated Apoptosis

2001 • 374 citations

Scrapie-infected murine neuroblastoma cells produce protease-resistant prion proteins

1988 • 366 citations

Scrapie infectivity, fibrils and low molecular weight protein

1983 • 345 citations

Species specificity in the cell-free conversion of prion protein to protease-resistant forms: a model for the scrapie species barrier.

1995 • 343 citations

Cellular isoform of the scrapie agent protein participates in lymphocyte activation

1990 • 342 citations

Propagation of prions with artificial properties in transgenic mice expressing chimeric PrP genes

1993 • 331 citations

Prion protein biosynthesis in scrapie-infected and uninfected neuroblastoma cells

1989 • 330 citations

The major polypeptide of scrapie-associated fibrils (SAF) has the same size, charge distribution and N-terminal protein sequence as predicted for the normal brain protein (PrP).

1986 • 322 citations

Strain-dependent Differences in β-Sheet Conformations of Abnormal Prion Protein

1998 • 313 citations

Neurological Illness in Transgenic Mice Expressing a Prion Protein with an Insertional Mutation

1998 • 309 citations

Detection of prions in blood

2005 • 308 citations

Perturbation of the secondary structure of the scrapie prion protein under conditions that alter infectivity.

1993 • 307 citations

SPONGIFORM ENCEPHALOPATHY OF ROCKY MOUNTAIN ELK

1982 • 303 citations

Evidence of a molecular barrier limiting susceptibility of humans, cattle and sheep to chronic wasting disease

2000 • 300 citations

Species-barrier-independent prion replication in apparently resistant species

2000 • 293 citations

A hypothalamic neuronal cell line persistently infected with scrapie prions exhibits apoptosis

1997 • 279 citations

Prion Domain Initiation of Amyloid Formation in Vitro from Native Ure2p

1999 • 278 citations

Synthesis and trafficking of prion proteins in cultured cells.

1992 • 268 citations

Asparagine-linked glycosylation of the scrapie and cellular prion proteins

1989 • 268 citations

PrP gene dosage determines the timing but not the final intensity or distribution of lesions in scrapie pathology.

1994 • 267 citations

Molecular assessment of the potential transmissibilities of BSE and scrapie to humans

1997 • 260 citations

A single amino acid alteration (101L) introduced into murine PrP dramatically alters incubation time of transmissible spongiform encephalopathy

1999 • 243 citations

Chimeric prion protein expression in cultured cells and transgenic mice

1992 • 240 citations

Chaperone-supervised conversion of prion protein to its protease-resistant form

1997 • 232 citations

Binding of the protease-sensitive form of PrP (prion protein) to sulfated glycosaminoglycan and congo red [corrected]

1994 • 230 citations

Specific binding of normal prion protein to the scrapie form via a localized domain initiates its conversion to the protease-resistant state

1999 • 222 citations

Astrocyte-specific expression of hamster prion protein (PrP) renders PrP knockout mice susceptible to hamster scrapie

1997 • 212 citations

In Vitro Propagation of the Prion-Like State of Yeast Sup35 Protein

1997 • 210 citations

Ex vivo propagation of infectious sheep scrapie agent in heterologous epithelial cells expressing ovine prion protein

2001 • 209 citations

Thermal stability and conformational transitions of scrapie amyloid (prion) protein correlate with infectivity

1993 • 204 citations

Prion Protein of 106 Residues Creates an Artificial Transmission Barrier for Prion Replication in Transgenic Mice

1999 • 202 citations

Characterization of Scrapie Infection in Mouse Neuroblastoma Cells

1987 • 199 citations

Scrapie-associated fibrils in Creutzfeldt–Jakob disease

1983 • 199 citations

Ultrastructural localization of scrapie prion proteins in cytoplasmic vesicles of infected cultured cells.

1991 • 198 citations

Novel polymorphisms in the caprine PrP gene: a codon 142 mutation associated with scrapie incubation period

1996 • 182 citations

Specific Inhibition of in Vitro Formation of Protease-resistant Prion Protein by Synthetic Peptides

1998 • 179 citations

Molecular cloning and complete sequence of prion protein cDNA from mouse brain infected with the scrapie agent.

1986 • 176 citations

Scrapie susceptibility-linked polymorphisms modulate the in vitro conversion of sheep prion protein to protease-resistant forms

1997 • 174 citations

Long-Term Subclinical Carrier State Precedes Scrapie Replication and Adaptation in a Resistant Species: Analogies to Bovine Spongiform Encephalopathy and Variant Creutzfeldt-Jakob Disease in Humans

2001 • 171 citations

Unaltered susceptibility to BSE in transgenic mice expressing human prion protein

1997 • 170 citations

Interactions between heterologous forms of prion protein: Binding, inhibition of conversion, and species barriers

2000 • 168 citations

A Mutant Prion Protein Displays an Aberrant Membrane Association When Expressed in Cultured Cells

1995 • 160 citations

Comparison of Scrapie and Transmissible Mink Encephalopathy in Hamsters. II. Clinical Signs, Pathology, and Pathogenesis

1975 • 159 citations

Heterologous PrP molecules interfere with accumulation of protease-resistant PrP in scrapie-infected murine neuroblastoma cells

1994 • 156 citations

Transmissible and non-transmissible amyloidoses: autocatalytic post-translational conversion of host precursor proteins to β-pleated sheet configurations

1988 • 155 citations

Neuron-specific expression of a hamster prion protein minigene in transgenic mice induces susceptibility to hamster scrapie agent

1995 • 155 citations

Isolation and structural studies of the intact scrapie agent protein

1987 • 146 citations

Effect of the D178N Mutation and the Codon 129 Polymorphism on the Metabolism of the Prion Protein

1996 • 143 citations

A single hamster PrP amino acid blocks conversion to protease-resistant PrP in scrapie-infected mouse neuroblastoma cells

1995 • 142 citations

Suppression of scrapie infection in mice by heteropolyanion 23, dextran sulfate, and some other polyanions

1986 • 141 citations

Mouse T Cell Membrane Proteins Rt6−1 and Rt6−2 Are Arginine/Protein Mono(ADPribosyl)transferases and Share Secondary Structure Motifs with ADP-ribosylating Bacterial Toxins

1996 • 141 citations

Heparin‐like molecules bind differentially to prion‐proteins and change their intracellular metabolic fate

1993 • 140 citations

Mutant and Infectious Prion Proteins Display Common Biochemical Properties in Cultured Cells

1996 • 139 citations

Immunological comparison of scrapie-associated fibrils isolated from animals infected with four different scrapie strains

1986 • 137 citations

Unusual Topogenic Sequence Directs Prion Protein Biogenesis

1990 • 137 citations

Overexpression of Nonconvertible PrP ^c Δ114–121 in Scrapie-Infected Mouse Neuroblastoma Cells Leads to trans -Dominant Inhibition of Wild-Type PrP ^Sc Accumulation

1998 • 133 citations

Molecular pathology of scrapie‐associated fibril protein (PrP) in mouse brain affected by the ME7 strain of scrapie

1988 • 131 citations

Multiple Amino Acid Residues within the Rabbit Prion Protein Inhibit Formation of Its Abnormal Isoform

2003 • 128 citations

Sulfated glycosaminoglycans in amyloid plaques of prion diseases

1989 • 123 citations

Scrapie infectivity correlates with converting activity, protease resistance, and aggregation of scrapie-associated prion protein in guanidine denaturation studies

1997 • 122 citations

Changing a single amino acid in the N-terminus of murine PrP alters TSE incubation time across three species barriers

2001 • 119 citations

Susceptibility of Common Fibroblast Cell Lines to Transmissible Spongiform Encephalopathy Agents

2004 • 119 citations

Normal and scrapie-associated forms of prion protein differ in their sensitivities to phospholipase and proteases in intact neuroblastoma cells

1990 • 118 citations

Analyses of frequency of infection, specific infectivity, and prion protein biosynthesis in scrapie-infected neuroblastoma cell clones

1988 • 115 citations

Biochemical Differences among Scrapie-associated Fibrils Support the Biological Diversity of Scrapie Agents

1985 • 110 citations

In vitro Replication of Scrapie Agent in a Neuronal Model: Infection of PC12 Cells

1984 • 107 citations

Partial Unfolding and Refolding of Scrapie-Associated Prion Protein: Evidence for a Critical 16-kDa C-Terminal Domain

1996 • 107 citations

Stimulation of PrPC Retrograde Transport toward the Endoplasmic Reticulum Increases Accumulation of PrPSc in Prion-infected Cells

2002 • 104 citations

Cellular Prion Protein Sensitizes Neurons to Apoptotic Stimuli through Mdm2-regulated and p53-dependent Caspase 3-like Activation

2003 • 98 citations

Glycosylation influences cross-species formation of protease-resistant prion protein

2001 • 96 citations

Differential Metabolic Effects of Adenovirus-mediated Glucokinase and Hexokinase I Overexpression in Rat Primary Hepatocytes

1996 • 95 citations

Identification of Intermediate Steps in the Conversion of a Mutant Prion Protein to a Scrapie-like Form in Cultured Cells

1997 • 93 citations

Dominant-Negative Inhibition of Prion Formation Diminished by Deletion Mutagenesis of the Prion Protein

2000 • 90 citations

Abnormal Properties of Prion Protein with Insertional Mutations in Different Cell Types

1998 • 88 citations

N-terminal Truncation of Prion Protein Affects Both Formation and Conformation of Abnormal Protease-resistant Prion Protein Generatedin Vitro

2001 • 83 citations

Subclinical Scrapie Infection in a Resistant Species: Persistence, Replication, and Adaptation of Infectivity during Four Passages

2002 • 82 citations

Scrapie-Infected Spleens: Analysis of Infectivity, Scrapie-Associated Fibrils, and Protease-Resistant Proteins

1991 • 80 citations

Epidemiology and control of bovine spongiform encephalopathy (BSE)

1993 • 76 citations

Molecular Basis of Scrapie Strain Glycoform Variation

2002 • 76 citations

Strain Typing Studies of Scrapie and BSE

2003 • 75 citations

Efficient Conversion of Normal Prion Protein (PrP) by Abnormal Hamster PrP Is Determined by Homology at Amino Acid Residue 155

2001 • 74 citations

Correlative light and electron microscopy studies of PrP localisation in 87V scrapie

1994 • 74 citations

Inhibition of Interactions and Interconversions of Prion Protein Isoforms by Peptide Fragments from the C-terminal Folded Domain

2001 • 71 citations

A Modified Host Protein Model of Scrapie

2007 • 69 citations

Creutzfeldt-Jakob disease: diagnostic utility of 14–3–3 protein immunodetection in cerebrospinal fluid

2000 • 66 citations

Acute Formation of Protease-resistant Prion Protein Does Not Always Lead to Persistent Scrapie Infection in Vitro

2004 • 61 citations

Onset of Accumulation of PrPresin Murine ME7 Scrapie in Relation to Pathological and PrP Immunohistochemical Changes

2001 • 58 citations

Inhibition of scrapie-associated PrP accumulation

1994 • 54 citations

In Situ Formation of Protease-resistant Prion Protein in Transmissible Spongiform Encephalopathy-infected Brain Slices

1997 • 52 citations

Drug therapy in human and experimental transmissible spongiform encephalopathy

2002 • 51 citations

Murine scrapie-infected neurons in vivo release excess prion protein into the extracellular space

1994 • 50 citations

Prion protein and species barriers in the transmissible spongiform encephalopathies

1999 • 45 citations

Determination of the frequency and distribution of vascular and parenchymal amyloid with polyclonal and N‐terminalspecific PrP antibodies in scrapie‐affected sheep and mice

1998 • 38 citations

Deletion of β-Strand and α-Helix Secondary Structure in Normal Prion Protein Inhibits Formation of Its Protease-Resistant Isoform

2001 • 37 citations

Identification of possible animal origins of prion disease in human beings

2004 • 12 citations