(Q36005158)

11 August 2017

3 references

23 March 2019

25 April 2019

21 December 2019

Molecular pathogenesis of sporadic prion diseases in man. (English)

1 reference

transmissible spongiform encephalopathy

11 August 2017

0 references

3 references

23 March 2019

25 April 2019

21 December 2019

1 reference

21 December 2019

Jiri G Safar

1

1 reference

11 August 2017

1 April 2012

4 references

11 August 2017

23 March 2019

25 April 2019

21 December 2019

4 references

11 August 2017

23 March 2019

25 April 2019

21 December 2019

6

1 reference

11 August 2017

2

1 reference

11 August 2017

108-115

1 reference

Creative Commons Attribution-NonCommercial 3.0 Unported

11 August 2017

3 references

23 March 2019

25 April 2019

on focus list of Wikimedia project

21 December 2019

ScienceSource

0 references

cites work

Protease-sensitive conformers in broad spectrum of distinct PrPSc structures in sporadic Creutzfeldt-Jakob disease are indicator of progression rate

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3366352

Conserved properties of human and bovine prion strains on transmission to guinea pigs

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3366352

A new method for the characterization of strain-specific conformational stability of protease-sensitive and protease-resistant PrPSc

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3366352

Distinct stability states of disease-associated human prion protein identified by conformation-dependent immunoassay

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3366352

Defining sporadic Creutzfeldt-Jakob disease strains and their transmission properties

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3366352

Protease-sensitive synthetic prions

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3366352

Human prion diseases in the United States.

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3366352

Darwinian evolution of prions in cell culture

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3366352

Exogenous alpha-synuclein fibrils seed the formation of Lewy body-like intracellular inclusions in cultured cells

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3366352

Co-existence of scrapie prion protein types 1 and 2 in sporadic Creutzfeldt-Jakob disease: its effect on the phenotype and prion-type characteristics

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3366352

Extracellular mutant SOD1 induces microglial-mediated motoneuron injury

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3366352

Inclusion formation and neuronal cell death through neuron-to-neuron transmission of alpha-synuclein

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3366352

Distinct structures of scrapie prion protein (PrPSc)-seeded versus spontaneous recombinant prion protein fibrils revealed by hydrogen/deuterium exchange.

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3366352

Prion proteins in subpopulations of white blood cells from patients with sporadic Creutzfeldt-Jakob disease

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3366352

Propagation of tau misfolding from the outside to the inside of a cell.

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3366352

Prion diseases and their biochemical mechanisms

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3366352

Getting a grip on prions: oligomers, amyloids, and pathological membrane interactions

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3366352

Characterization of truncated forms of abnormal prion protein in Creutzfeldt-Jakob disease

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3366352

Detection and characterization of proteinase K-sensitive disease-related prion protein with thermolysin.

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3366352

A novel human disease with abnormal prion protein sensitive to protease

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3366352

The prion's elusive reason for being

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3366352

Beyond PrP9res) type 1/type 2 dichotomy in Creutzfeldt-Jakob disease

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3366352

A general model of prion strains and their pathogenicity

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3366352

In vitro amplification and detection of variant Creutzfeldt-Jakob disease PrPSc.

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3366352

The cellular prion protein (PrP(C)): its physiological function and role in disease

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3366352

The prion strain phenomenon: molecular basis and unprecedented features

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3366352

Exogenous induction of cerebral beta-amyloidogenesis is governed by agent and host

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3366352

Analysis of prion strains by PrPSc profiling in sporadic Creutzfeldt-Jakob disease

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3366352

Search for a prion-specific nucleic acid

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3366352

Immunodetection of disease-associated mutant PrP, which accelerates disease in GSS transgenic mice

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3366352

Diagnosis of human prion disease

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3366352

A case-control study of sporadic Creutzfeldt-Jakob disease in the United Kingdom: analysis of clustering.

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3366352

Mutant PrPSc conformers induced by a synthetic peptide and several prion strains

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3366352

Sporadic and familial CJD: classification and characterisation.

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3366352

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

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3366352

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

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3366352

Protease-sensitive scrapie prion protein in aggregates of heterogeneous sizes

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3366352

Shattuck lecture--neurodegenerative diseases and prions

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3366352

Strain-specified relative conformational stability of the scrapie prion protein

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3366352

NMR solution structure of the human prion protein

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3366352

Eight prion strains have PrP(Sc) molecules with different conformations

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3366352

Prion protein expression in different species: analysis with a panel of new mAbs

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3366352

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

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3366352

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

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3366352

Fatal familial insomnia and familial Creutzfeldt-Jakob disease: different prion proteins determined by a DNA polymorphism

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3366352

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

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3366352

Novel proteinaceous infectious particles cause scrapie

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3366352

Experimental transmission of a Kuru-like syndrome to chimpanzees

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3366352

Creutzfeldt-Jakob Disease (Spongiform Encephalopathy): Transmission to the Chimpanzee

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3366352

Creutzfeldt-Jakob disease. The neuropathology of a transmission experiment

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3366352

Human prion protein cDNA: molecular cloning, chromosomal mapping, and biological implications

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3366352

A cellular gene encodes scrapie PrP 27-30 protein

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3366352

Asparagine-linked glycosylation of the scrapie and cellular prion proteins

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3366352

Mouse polyclonal and monoclonal antibody to scrapie-associated fibril proteins

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3366352

Creutzfeldt-Jakob disease in France: II. Clinical characteristics of 124 consecutive verified cases during the decade 1968-1977

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3366352

Proteinase K-sensitive disease-associated ovine prion protein revealed by conformation-dependent immunoassay

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3366352

Insomnia in prion diseases: sporadic and familial.

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3366352

Characterization of a prion protein (PrP) gene from rabbit; a species with apparent resistance to infection by prions

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3366352

Evidence for the conformation of the pathologic isoform of the prion protein enciphering and propagating prion diversity

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3366352

Molecular basis of phenotypic variability in sporadic Creutzfeldt-Jakob disease

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3366352

Slow-Virus Infections of the Nervous System

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3366352

Experimental spongiform encephalopathy (Creutzfeldt-Jakob disease) in chimpanzees. Electron microscopic studies

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3366352

Experimental subacute spongiform virus encephalopathies in primates and other laboratory animals

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3366352

Creutzfeldt-Jakob disease: clinical analysis of a consecutive series of 230 neuropathologically verified cases

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3366352

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

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3366352

Identification of glycoinositol phospholipid linked and truncated forms of the scrapie prion protein

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3366352

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

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3366352

Differences in the Membrane Interaction of Scrapie Amyloid Precursor Proteins in Normal and Scrapie- or Creutzfeldt-Jakob Disease-Infected Brains

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3366352

Subacute spongiform encephalopathy--a subacute form of encephalopathy attributable to vascular dysfunction (spongiform cerebral atrophy).

11 July 2018

1 reference

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

21 January 2018

1 reference

First experimental transmission of fatal familial insomnia

21 January 2018

1 reference

Prion clearance in bigenic mice

21 January 2018

1 reference

Insoluble aggregates and protease-resistant conformers of prion protein in uninfected human brains.

21 January 2018

1 reference

21 January 2018

Typing prion isoforms.

1 reference

Identification of novel proteinase K-resistant C-terminal fragments of PrP in Creutzfeldt-Jakob disease

21 January 2018

1 reference

Coexistence of multiple PrPSc types in individuals with Creutzfeldt-Jakob disease.

21 January 2018

1 reference

Sporadic Creutzfeldt-Jakob disease: co-occurrence of different types of PrP(Sc) in the same brain

21 January 2018

1 reference

Prion protein heterogeneity in sporadic but not variant Creutzfeldt-Jakob disease: UK cases 1991-2002.

21 January 2018

1 reference

Australian sporadic CJD analysis supports endogenous determinants of molecular-clinical profiles.

21 January 2018

1 reference

Human platelets as a substrate source for the in vitro amplification of the abnormal prion protein (PrP) associated with variant Creutzfeldt-Jakob disease.

21 January 2018

1 reference

Isolation and characterization of a proteinase K-sensitive PrPSc fraction.

21 January 2018

1 reference

Targeting cellular prion protein reverses early cognitive deficits and neurophysiological dysfunction in prion-infected mice

21 January 2018

1 reference

The physical basis of how prion conformations determine strain phenotypes

21 January 2018

1 reference

Immunohistochemistry for the prion protein: comparison of different monoclonal antibodies in human prion disease subtypes.

21 January 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3366352

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

23 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3366352

Strain-specific prion-protein conformation determined by metal ions

23 September 2018

1 reference

The same prion strain causes vCJD and BSE

21 January 2018

1 reference

Conformational transitions, dissociation, and unfolding of scrapie amyloid (prion) protein

21 January 2018

1 reference

12 December 2020

Scrapie amyloid (prion) protein has the conformational characteristics of an aggregated molten globule folding intermediate

1 reference

12 December 2020

Kuru; clinical, pathological and epidemiological study of an acute progressive degenerative disease of the central nervous system among natives of the Eastern Highlands of New Guinea

1 reference

12 December 2020

1 reference

12 December 2020

1 reference

12 December 2020

Oxidative stress and the prion protein in transmissible spongiform encephalopathies

1 reference

12 December 2020

Improved conformation-dependent immunoassay: suitability for human prion detection with enhanced sensitivity

1 reference

12 December 2020

Identifiers

DOI

10.4161/PRI.18666

1 reference

11 August 2017

1 reference

PMC publication ID

3366352

11 August 2017

PubMed publication ID

22421210

1 reference

PMC publication ID

3366352