Prion-Induced Neuronal Damage — The Mechanisms of Neuronal Destruction in the Subacute Spongiform Encephalopathies

Apoptosis: A Basic Biological Phenomenon with Wideranging Implications in Tissue Kinetics

1972 • 15,349 citations

Identification of programmed cell death in situ via specific labeling of nuclear DNA fragmentation.

1992 • 9,540 citations

Prions

1998 • 5,102 citations

Novel Proteinaceous Infectious Particles Cause Scrapie

1982 • 4,972 citations

Glucocorticoid-induced thymocyte apoptosis is associated with endogenous endonuclease activation

1980 • 4,591 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

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

1996 • 1,740 citations

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

1992 • 1,632 citations

Chromatin cleavage in apoptosis: Association with condensed chromatin morphology and dependence on macromolecular synthesis

1984 • 1,586 citations

A cellular gene encodes scrapie PrP 27-30 protein

1985 • 1,494 citations

Classification of sporadic Creutzfeldt-Jakob disease based on molecular and phenotypic analysis of 300 subjects

1999 • 1,354 citations

The cellular prion protein binds copper in vivo

1997 • 1,263 citations

Identification of a Protein That Purifies with the Scrapie Prion

1982 • 1,260 citations

Eight prion strains have PrPSc molecules with different conformations

1998 • 1,236 citations

Copper biochemistry and molecular biology

1996 • 1,171 citations

Scrapie prion protein contains a phosphatidylinositol glycolipid

1987 • 1,104 citations

Scrapie prions aggregate to form amyloid-like birefringent rods

1983 • 1,048 citations

Neurotoxicity of a prion protein fragment

1993 • 937 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

Molecular basis of phenotypic variability in sporadc creudeldt‐jakob disease

1996 • 832 citations

Scrapie and cellular PrP isoforms are encoded by the same chromosomal gene

1986 • 829 citations

Prion protein is necessary for normal synaptic function

1994 • 802 citations

Normal host prion protein necessary for scrapie-induced neurotoxicity

1996 • 791 citations

A Transmembrane Form of the Prion Protein in Neurodegenerative Disease

1998 • 724 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

Separation and properties of cellular and scrapie prion proteins.

1986 • 619 citations

Structural studies of the scrapie prion protein using mass spectrometry and amino acid sequencing

1993 • 578 citations

Copper binding to the prion protein: Structural implications of four identical cooperative binding sites

1999 • 541 citations

Normal prion protein has an activity like that of superoxide dismutase

1999 • 520 citations

Prion Protein Selectively Binds Copper(II) Ions

1998 • 515 citations

Role of microglia and host prion protein in neurotoxicity of a prion protein fragment

1996 • 506 citations

Necrosis and apoptosis: distinct modes of cell death with fundamentally different significance.

1982 • 500 citations

Purification and structural studies of a major scrapie prion protein

1984 • 497 citations

Identification of scrapie prion protein-specific mRNA in scrapie-infected and uninfected brain

1985 • 490 citations

Nearly ubiquitous tissue distribution of the scrapie agent precursor protein

1992 • 468 citations

Prion Protein-Deficient Cells Show Altered Response to Oxidative Stress Due to Decreased SOD-1 Activity

1997 • 447 citations

Scrapie prion proteins are synthesized in neurons.

1986 • 422 citations

The exceptionally small size of the scrapie agent

1966 • 403 citations

Copper Binding to the N-Terminal Tandem Repeat Regions of Mammalian and Avian Prion Protein

1995 • 353 citations

Identification of Microglial Signal Transduction Pathways Mediating a Neurotoxic Response to Amyloidogenic Fragments of β-Amyloid and Prion Proteins

1999 • 353 citations

Evidence of Presynaptic Location and Function of the Prion Protein

1999 • 350 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

Scrapie in mice

1973 • 340 citations

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

1998 • 313 citations

SUBACUTE SPONGIFORM ENCEPHALOPATHY (CREUTZFELDT-JAKOB DISEASE)

1978 • 311 citations

Transmissible and genetic prion diseases share a common pathway of neurodegeneration

1999 • 302 citations

Chronic inhibition of superoxide dismutase produces apoptotic death of spinal neurons.

1994 • 291 citations

Diagnostic Criteria for Sporadic Creutzfeldt-Jakob Disease

1996 • 290 citations

No propagation of prions in mice devoid of PrP

1994 • 282 citations

Detection of DNA fragmentation in apoptosis: application of in situ nick translation to cell culture systems and tissue sections.

1993 • 276 citations

The prion protein gene: a role in mouse embryogenesis?

1992 • 274 citations

Changes in the localization of brain prion proteins during scrapie infection

1987 • 268 citations

Differential release of cellular and scrapie prion proteins from cellular membranes by phosphatidylinositol-specific phospholipase C

1990 • 263 citations

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

1994 • 245 citations

Scrapie strain variation and mutation

1993 • 240 citations

Role of Microglia in Neuronal Cell Death in Prion Disease

1998 • 237 citations

Apoptosis and necrosis in toxicology: A continuum or distinct modes of cell death?

1997 • 213 citations

Processing of a cellular prion protein: identification of N- and C-terminal cleavage sites

1993 • 211 citations

Molecular Characteristics of a Protease-Resistant, Amyloidogenic and Neurotoxic Peptide Homologous to Residues 106-126 of the Prion Protein

1993 • 208 citations

Changes in Tissue Growth, Concentrations of Copper, Iron, Cytochrome Oxidase and Superoxide Dismutase Subsequent to Dietary or Genetic Copper Deficiency in Mice

1983 • 207 citations

Mouse cortical cells lacking cellular PrP survive in culture with a neurotoxic PrP fragment

1994 • 198 citations

PrP Deposition, Microglial Activation, and Neuronal Apoptosis in Murine Scrapie

1997 • 195 citations

Channel Formation by a Neurotoxic Prion Protein Fragment

1997 • 191 citations

Purification and properties of the cellular prion protein from Syrian hamster brain

1992 • 187 citations

Conformational polymorphism of the amyloidogenic and neurotoxic peptide homologous to residues 106-126 of the prion protein.

1994 • 186 citations

Genetic and Infectious Prion Diseases

1993 • 175 citations

Sequential appearance and accumulation of pathognomonic markers in the central nervous system of hamsters orally infected with scrapie

1996 • 173 citations

Proteinase‐resistant prion protein accumulation in Syrian hamster brain correlates with regional pathology and scrapie infectivity

1991 • 167 citations

Neuronal Apoptosis in Creutzfeldt-Jakob Disease

1999 • 161 citations

Neuronal Cell Death in Scrapie‐Infected Mice Is Due to Apoptosis

1995 • 157 citations

CEREBRAL AMYLOIDOSIS IN SCRAPIE IN THE MOUSE: EFFECT OF AGENT STRAIN AND MOUSE GENOTYPE

1976 • 152 citations

Molecular characteristics of the major scrapie prion protein

1984 • 152 citations

Cytoprotective effect of NMDA receptor antagonists on prion protein (PrionSc)-induced toxicity in rat cortical cell cultures

1993 • 151 citations

Chemical Disinfection of Creutzfeldt–Jakob Disease Virus

1982 • 144 citations

Characterization of the microglial response in murine scrapie

1994 • 144 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

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

Non-hydrophobic extracytoplasmic determinant of stop transfer in the prion protein

1990 • 132 citations

Immunocytochemical appearance of cytokines, prostaglandin E2 and lipocortin-1 in the CNS during the incubation period of murine scrapie correlates with progressive PrP accumulations

1997 • 131 citations

Further analysis of nucleic acids in purified scrapie prion preparations by improved return refocusing gel electrophoresis

1992 • 131 citations

Effect of Dietary or Genetic Copper Deficiency on Brain Catecholamines, Trace Metals and Enzymes in Mice and Rats

1982 • 122 citations

Microglial cells respond to amyloidogenic PrP peptide by the production of inflammatory cytokines

1999 • 117 citations

Cytokines, prostaglandins and lipocortin-1 are present in the brains of scrapie-infected mice

1994 • 101 citations

Detection of apoptosis in murine scrapie

1995 • 90 citations

Severe, Early and Selective Loss of a Subpopulation of GABAergic Inhibitory Neurons in Experimental Transmissible Spongiform Encephalopathies

1998 • 90 citations

Neuronal Apoptosis in Fatal Familial Insomnia

1998 • 84 citations

Neural Lesions in the Offspring of Female Rats Fed α Copper-deficient Diet

1969 • 83 citations

Effect of Flupirtine on Bcl-2 and Glutathione Level in Neuronal Cells Treatedin Vitrowith the Prion Protein Fragment (PrP106-126)

1997 • 78 citations

EFFECT OF COPPER DEFICIENCY ON THE CONCENTRATIONS OF CATECHOLAMINES AND RELATED ENZYME ACTIVITIES IN THE RAT BRAIN

1977 • 74 citations

Histopathological Similarities between Scrapie and Cuprizone Toxicity in Mice

1971 • 69 citations

In vivo toxicity of prion protein in murine scrapie: ultrastructural and immunogold studies

1997 • 68 citations

Permethylation and tandem mass spectrometry of oligosaccharides having free hexosamine: Analysis of the glycoinositol phospholipid anchor glycan from the scrapie prion protein

1990 • 67 citations

Diversity in the neuropathology of scrapie-like diseases in animals

1993 • 64 citations

Spongiform encephalopathy induced in rats and guinea pigs by cuprizone

1969 • 63 citations

A Neurotoxic and Gliotrophic Fragment of the Prion Protein Increases Plasma Membrane Microviscosity

1997 • 60 citations

Degenerative hippocampal pathology in mice infected with scrapie

1984 • 59 citations

Nuclear DNA fragmentation in Creutzfeldt-Jakob disease: does a mere positive in situ nuclear end-labeling indicate apoptosis?

1999 • 51 citations

Scrapie infection alters the membrane and synaptic properties of mouse hippocampal CA1 pyramidal neurones.

1997 • 51 citations

PrP in pathology and pathogenesis in scrapie-infected mice

1994 • 51 citations

Increase of intracellular free Ca2+ in microglia activated by prion protein fragment

1997 • 48 citations

Lipid Composition in Scrapie‐Infected Mouse Brain: Prion Infection Increases the Levels of Dolichyl Phosphate and Ubiquinone

1996 • 47 citations

Expression of Proteins Linked to Exocytosis and Neurotransmission in Patients with Creutzfeldt–Jakob Disease

1999 • 47 citations

Cell death in prion disease

1997 • 41 citations

Scrapie Infection Diminishes Spines and Increases Varicosities of Dendrites in Hamsters

1987 • 37 citations

Prion's Progress: Patterns and Rates of Molecular Evolution in Relation to Spongiform Disease

1998 • 31 citations

A 2-year longitudinal study of swimming navigation in mice devoid of the prion protein: no evidence for neurological anomalies or spatial learning impairments

1998 • 29 citations

Prions and nucleic acids: search for "residual" nucleic acids and screening for mutations in the PrP-gene.

1993 • 27 citations

Analysis of nucleic acids in purified scrapie prion preparations

1993 • 27 citations

Postmortem delay and temperature conditions affect the in situ end-labeling (ISEL) assay in brain tissue of mice.

1997 • 18 citations