Transmissible spongiform encephalopathies. (Q34289917)

1 August 2017

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title

Transmissible spongiform encephalopathies. (English)

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1 August 2017

3

Colin L Masters

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1 August 2017

Steven J Collins

1

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PubMed ID

14723996

1 August 2017

Victoria A Lawson

series ordinal

2

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PubMed ID

14723996

1 August 2017

language of work or name

English

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publication date

1 January 2004

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1 August 2017

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1 August 2017

363

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1 August 2017

9402

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1 August 2017

51-61

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Shattuck lecture--neurodegenerative diseases and prions

1 August 2017

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https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Inherited prion disease with 144 base pair gene insertion. 2. Clinical and pathological features.

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

A new variant of Creutzfeldt-Jakob disease in the UK.

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

The same prion strain causes vCJD and BSE

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Identification of a protein that purifies with the scrapie prion.

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Naturally secreted oligomers of amyloid beta protein potently inhibit hippocampal long-term potentiation in vivo

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

A cellular gene encodes scrapie PrP 27-30 protein

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

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

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https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

NMR structure of the bovine prion protein

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https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

The cellular prion protein binds copper in vivo

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https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Prion protein is necessary for normal synaptic function.

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https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Molecular features of the copper binding sites in the octarepeat domain of the prion protein

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Copper and zinc binding modulates the aggregation and neurotoxic properties of the prion peptide PrP106-126.

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Location and properties of metal-binding sites on the human prion protein.

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Copper coordination in the full-length, recombinant prion protein

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Copper binding to the octarepeats of the prion protein. Affinity, specificity, folding, and cooperativity: insights from circular dichroism

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Ablation of the metal ion-induced endocytosis of the prion protein by disease-associated mutation of the octarepeat region

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Reversibility of scrapie inactivation is enhanced by copper.

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Trafficking of prion proteins through a caveolae-mediated endosomal pathway

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https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

A glycolipid-anchored prion protein is endocytosed via clathrin-coated pits.

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Mutant prion proteins are partially retained in the endoplasmic reticulum

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Conversion of raft associated prion protein to the protease-resistant state requires insertion of PrP-res (PrP(Sc)) into contiguous membranes

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Proteasomes and ubiquitin are involved in the turnover of the wild-type prion protein

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Mutant PrP is delayed in its exit from the endoplasmic reticulum, but neither wild-type nor mutant PrP undergoes retrotranslocation prior to proteasomal degradation

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Neurotoxicity and neurodegeneration when PrP accumulates in the cytosol

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Conversion of PrP to a self-perpetuating PrPSc-like conformation in the cytosol

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Cell-free formation of protease-resistant prion protein.

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Reversible conversion of monomeric human prion protein between native and fibrilogenic conformations.

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

A prion protein epitope selective for the pathologically misfolded conformation

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https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Immobilized prion protein undergoes spontaneous rearrangement to a conformation having features in common with the infectious form

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Evidence for protein X binding to a discontinuous epitope on the cellular prion protein during scrapie prion propagation

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Reversibility of scrapie-associated prion protein aggregation

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Separation and properties of cellular and scrapie prion proteins

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Efficient conversion of normal prion protein (PrP) by abnormal hamster PrP is determined by homology at amino acid residue 155.

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Strain-specific prion-protein conformation determined by metal ions

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https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Mice devoid of PrP are resistant to scrapie

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Protease-resistant prion protein produced in vitro lacks detectable infectivity.

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

A transmembrane form of the prion protein in neurodegenerative disease

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Fatal familial insomnia: a second kindred with mutation of prion protein gene at codon 178

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Spontaneous neurodegeneration in transgenic mice with mutant prion protein

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Transmission of the BSE agent to mice in the absence of detectable abnormal prion protein

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https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Creutzfeldt-Jakob disease in Australia 1970-1999.

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https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Human spongiform encephalopathy: the National Institutes of Health series of 300 cases of experimentally transmitted disease.

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Sporadic Creutzfeldt-Jakob disease in the United Kingdom: analysis of epidemiological surveillance data for 1970-96

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Prion protein conformation in a patient with sporadic fatal insomnia

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Analysis of EEG and CSF 14-3-3 proteins as aids to the diagnosis of Creutzfeldt-Jakob disease

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

14-3-3 protein, neuron-specific enolase, and S-100 protein in cerebrospinal fluid of patients with Creutzfeldt-Jakob disease

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

The 14-3-3 brain protein in cerebrospinal fluid as a marker for transmissible spongiform encephalopathies.

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

14-3-3 Protein isoforms and atypical patterns of the 14-3-3 assay in the diagnosis of Creutzfeldt-Jakob disease

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Tau protein and 14-3-3 protein in the differential diagnosis of Creutzfeldt-Jakob disease

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

MRI of Creutzfeldt-Jakob disease: imaging features and recommended MRI protocol

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Genetic basis of Creutzfeldt-Jakob disease in the United Kingdom: a systematic analysis of predisposing mutations and allelic variation in the PRNP gene.

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Novel prion protein insert mutation associated with prolonged neurodegenerative illness.

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Spontaneous mutations in the prion protein gene causing transmissible spongiform encephalopathy

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Creutzfeldt-Jacob disease associated with the PRNP codon 200Lys mutation: an analysis of 45 families

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Creutzfeldt-Jakob disease virus isolations from the Gerstmann-Sträussler syndrome with an analysis of the various forms of amyloid plaque deposition in the virus-induced spongiform encephalopathies

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Linkage of a prion protein missense variant to Gerstmann-Sträussler syndrome

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

A synthetic peptide initiates Gerstmann-Sträussler-Scheinker (GSS) disease in transgenic mice

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Fatal familial insomnia, a prion disease with a mutation at codon 178 of the prion protein gene

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

First experimental transmission of fatal familial insomnia.

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Codon 178 mutation in ethnically diverse Creutzfeldt-Jakob disease families

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Fatal familial insomnia and familial Creutzfeldt-Jakob disease: disease phenotype determined by a DNA polymorphism

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

The D178N (cis-129M) "fatal familial insomnia" mutation associated with diverse clinicopathologic phenotypes in an Australian kindred.

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Phenotypic variability in fatal familial insomnia (D178N-129M) genotype.

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

[18F]FDG PET in fatal familial insomnia: the functional effects of thalamic lesions.

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Clinical and genetic studies of fatal familial insomnia.

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Iatrogenic Creutzfeldt-Jakob disease at the millennium.

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

First hundred cases of variant Creutzfeldt-Jakob disease: retrospective case note review of early psychiatric and neurological features

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Use of 14-3-3 and other brain-specific proteins in CSF in the diagnosis of variant Creutzfeldt-Jakob disease

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Extraneural pathologic prion protein in sporadic Creutzfeldt-Jakob disease.

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Tissue distribution of protease resistant prion protein in variant Creutzfeldt-Jakob disease using a highly sensitive immunoblotting assay

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Transmission of prion diseases by blood transfusion

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Species-barrier-independent prion replication in apparently resistant species

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Degenerative disease of the central nervous system in New Guinea; the endemic occurrence of kuru in the native population

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Unconventional Viruses and the Origin and Disappearance of Kuru

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Kuru: clinical study of a new syndrome resembling paralysis agitans in natives of the Eastern Highlands of Australian New Guinea.

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Experimental transmission of a Kuru-like syndrome to chimpanzees

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Phenotype-genotype studies in kuru: implications for new variant Creutzfeldt-Jakob disease

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Increased susceptibility to Kuru of carriers of the PRNP 129 methionine/methionine genotype

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Balancing selection at the prion protein gene consistent with prehistoric kurulike epidemics

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

1 reference

7 January 2021

Preclinical lesions and their progression in the experimental spongiform encephalopathies (kuru and Creutzfeldt-Jakob disease) in primates

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Chronic subclinical prion disease induced by low-dose inoculum

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

BSE prions propagate as either variant CJD-like or sporadic CJD-like prion strains in transgenic mice expressing human prion protein

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Scrapie pathogenesis in subclinically infected B-cell-deficient mice

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Subclinical scrapie infection in a resistant species: persistence, replication, and adaptation of infectivity during four passages

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Creutzfeldt-Jakob disease: Patterns of worldwide occurrence and the significance of familial and sporadic clustering

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Incidence of Creutzfeldt-Jakob disease in Switzerland

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Sporadic Creutzfeldt-Jakob disease and surgery: a case-control study using community controls

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Creutzfeldt-Jakob disease cluster in an Australian rural city

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Bovine spongiform encephalopathy case found in Japan

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Implications of BSE infection screening data for the scale of the British BSE epidemic and current European infection levels.

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Measuring prions causing bovine spongiform encephalopathy or chronic wasting disease by immunoassays and transgenic mice

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Prions in skeletal muscle

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Estimation of epidemic size and incubation time based on age characteristics of vCJD in the United Kingdom

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Predictability of the UK variant Creutzfeldt-Jakob disease epidemic

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Inactivation of transmissible degenerative encephalopathy agents: A review

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Creutzfeldt-Jakob disease: recommendations for disinfection and sterilization

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Drug therapy in human and experimental transmissible spongiform encephalopathy

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Specific inhibition of pathological prion protein accumulation by small interfering RNAs

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Lysosomotropic agents and cysteine protease inhibitors inhibit scrapie-associated prion protein accumulation

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Acridine and phenothiazine derivatives as pharmacotherapeutics for prion disease

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Quinacrine does not prolong survival in a murine Creutzfeldt-Jakob disease model

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Evaluation of quinacrine treatment for prion diseases

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

Prevention of scrapie pathogenesis by transgenic expression of anti-prion protein antibodies.

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2803%2915171-9

7 January 2021

10.1016/S0140-6736(03)15171-9

Identifiers

DOI

1 reference

1 August 2017

1 reference

PubMed ID

14723996