(Q35004466)

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Inherited prion disease A117V is not simply a proteinopathy but produces prions transmissible to transgenic mice expressing homologous prion protein.

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https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:24086135%20AND%20SRC:MED&resulttype=core&format=json

26 February 2020

Inherited prion disease A117V is not simply a proteinopathy but produces prions transmissible to transgenic mice expressing homologous prion protein (English)

1 reference

Europe PubMed Central

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https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:24086135%20AND%20SRC:MED&resulttype=core&format=json

26 February 2020

prion protein family

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inherited prion disease

1 reference

based on heuristic

inferred from title

Emmanuel A. Asante

1

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Europe PubMed Central

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26 February 2020

Jonathan Wadsworth

11

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26 February 2020

12

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John Collinge

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26 February 2020

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Jacqueline M Linehan

2

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26 February 2020

Michelle Smidak

3

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26 February 2020

Andrew Tomlinson

4

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26 February 2020

Andrew Grimshaw

5

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26 February 2020

Asif Jeelani

6

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26 February 2020

Tatiana Jakubcova

7

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26 February 2020

Shyma Hamdan

8

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26 February 2020

Caroline Powell

9

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26 February 2020

Sebastian Brandner

10

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26 February 2020

publication date

26 September 2013

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26 February 2020

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Europe PubMed Central

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https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:24086135%20AND%20SRC:MED&resulttype=core&format=json

26 February 2020

9

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26 February 2020

9

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26 February 2020

e1003643

1 reference

Europe PubMed Central

PMC publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:24086135%20AND%20SRC:MED&resulttype=core&format=json

26 February 2020

copyright license

Creative Commons Attribution 4.0 International

26 September 2013

1 reference

April 2022 Public Data File from Crossref

copyright status

0 references

Levels of the Mahogunin Ring Finger 1 E3 ubiquitin ligase do not influence prion disease

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3784465

8 July 2018

Prion propagation and toxicity in vivo occur in two distinct mechanistic phases

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3784465

8 July 2018

Functional depletion of mahogunin by cytosolically exposed prion protein contributes to neurodegeneration

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3784465

8 July 2018

Folding kinetics of the human prion protein probed by temperature jump.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3784465

8 July 2018

Absence of spontaneous disease and comparative prion susceptibility of transgenic mice expressing mutant human prion proteins

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3784465

8 July 2018

Kuru prions and sporadic Creutzfeldt-Jakob disease prions have equivalent transmission properties in transgenic and wild-type mice

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3784465

8 July 2018

A general model of prion strains and their pathogenicity

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3784465

8 July 2018

Update on human prion disease.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3784465

8 July 2018

Accumulation of prion protein in the brain that is not associated with transmissible disease

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3784465

8 July 2018

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3784465

8 July 2018

Approaches to therapy of prion diseases

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3784465

8 July 2018

PrP knock-out and PrP transgenic mice in prion research

2 references

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3784465

8 July 2018

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3784465

26 October 2018

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3784465

8 July 2018

Mutations of the prion protein gene phenotypic spectrum

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3784465

8 July 2018

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://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3784465

8 July 2018

Prion proteins with different conformations accumulate in Gerstmann-Sträussler-Scheinker disease caused by A117V and F198S mutations

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3784465

8 July 2018

Prion diseases of humans and animals: their causes and molecular basis

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3784465

8 July 2018

Transmissible and genetic prion diseases share a common pathway of neurodegeneration

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3784465

8 July 2018

Inherited prion disease with an alanine to valine mutation at codon 117 in the prion protein gene

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3784465

8 July 2018

Variant Creutzfeldt-Jakob disease.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3784465

8 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3784465

8 July 2018

A comparative study of abnormal prion protein isoforms between Gerstmann-Sträussler-Scheinker syndrome and Creutzfeldt-Jakob disease.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3784465

8 July 2018

Different patterns of truncated prion protein fragments correlate with distinct phenotypes in P102L Gerstmann-Sträussler-Scheinker disease

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3784465

8 July 2018

A transmembrane form of the prion protein in neurodegenerative disease

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3784465

8 July 2018

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3784465

8 July 2018

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3784465

8 July 2018

Novel proteinaceous infectious particles cause scrapie

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3784465

8 July 2018

Alzheimer's disease. A family study.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3784465

8 July 2018

Self-replication and scrapie

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3784465

8 July 2018

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3784465

8 July 2018

Familial dementia with PrP-positive amyloid plaques: a variant of Gerstmann-Sträussler syndrome

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3784465

8 July 2018

Mouse polyclonal and monoclonal antibody to scrapie-associated fibril proteins

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3784465

8 July 2018

Homozygous prion protein genotype predisposes to sporadic Creutzfeldt–Jakob disease

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3784465

8 July 2018

Genetic predisposition to iatrogenic Creutzfeldt-Jakob disease

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3784465

8 July 2018

A prion protein variant in a family with the telencephalic form of Gerstmann-Straussler-Scheinker syndrome

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3784465

8 July 2018

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3784465

8 July 2018

Distinct glycoform ratios of protease resistant prion protein associated with PRNP point mutations.

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PPAT.1003643

21 January 2018

Fatal insomnia in a case of familial Creutzfeldt-Jakob disease with the codon 200(Lys) mutation

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PPAT.1003643

21 January 2018

Phenotypic variability of Gerstmann-Sträussler-Scheinker disease is associated with prion protein heterogeneity.

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PPAT.1003643

21 January 2018

A 7-kDa prion protein (PrP) fragment, an integral component of the PrP region required for infectivity, is the major amyloid protein in Gerstmann-Sträussler-Scheinker disease A117V.

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PPAT.1003643

21 January 2018

Phenotypic heterogeneity in inherited prion disease (P102L) is associated with differential propagation of protease-resistant wild-type and mutant prion protein.

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PPAT.1003643

21 January 2018

Unaltered susceptibility to BSE in transgenic mice expressing human prion protein.

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PPAT.1003643

21 January 2018

Human prion protein with valine 129 prevents expression of variant CJD phenotype

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PPAT.1003643

21 January 2018

Experimental transmission of Creutzfeldt-Jakob disease and related diseases to rodents

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PPAT.1003643

21 January 2018

Spontaneous neurodegeneration in transgenic mice with mutant prion protein

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PPAT.1003643

21 January 2018

Neurological illness in transgenic mice expressing a prion protein with an insertional mutation.

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PPAT.1003643

21 January 2018

Molecular classification of sporadic Creutzfeldt-Jakob disease.

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PPAT.1003643

21 January 2018

Species-barrier-independent prion replication in apparently resistant species

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PPAT.1003643

21 January 2018

The same prion strain causes vCJD and BSE

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PPAT.1003643

21 January 2018

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/10.1371%2FJOURNAL.PPAT.1003643

21 January 2018

Polymorphism at codon 129 or codon 219 of PRNP and clinical heterogeneity in a previously unreported family with Gerstmann-Sträussler-Scheinker disease (PrP-P102L mutation)

1 reference

https://pubmed.ncbi.nlm.nih.gov/24086135

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Extremely rapid folding of the C-terminal domain of the prion protein without kinetic intermediates

1 reference

https://pubmed.ncbi.nlm.nih.gov/24086135

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Identifiers

10.1371/JOURNAL.PPAT.1003643

1 reference

Europe PubMed Central

PMC publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:24086135%20AND%20SRC:MED&resulttype=core&format=json

26 February 2020

PMC publication ID

1 reference

Europe PubMed Central

PMC publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:24086135%20AND%20SRC:MED&resulttype=core&format=json

26 February 2020

PubMed publication ID

1 reference

Europe PubMed Central

PMC publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:24086135%20AND%20SRC:MED&resulttype=core&format=json

26 February 2020

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