Substitutions at residue 211 in the prion protein drive a switch between CJD and GSS syndrome, a new mechanism governing inherited neurodegenerative disorders. (Q48381755)

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scientific article published on 10 September 2012

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English	Substitutions at residue 211 in the prion protein drive a switch between CJD and GSS syndrome, a new mechanism governing inherited neurodegenerative disorders.	scientific article published on 10 September 2012

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scholarly article

1 reference

Europe PubMed Central

11 February 2018

http://europepmc.org/abstract/MED/22965875

Substitutions at residue 211 in the prion protein drive a switch between CJD and GSS syndrome, a new mechanism governing inherited neurodegenerative disorders. (English)

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Europe PubMed Central

11 February 2018

http://europepmc.org/abstract/MED/22965875

prion protein family

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neurodegeneration

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based on heuristic

inferred from title

Yassmine Chebaro

7

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Europe PubMed Central

11 February 2018

http://europepmc.org/abstract/MED/22965875

object named as

Katell Peoc'h

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Etienne Levavasseur

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object named as

Etienne Levavasseur

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Europe PubMed Central

11 February 2018

Jean-Jacques Hauw

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object named as

Jean-Jacques Hauw

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Europe PubMed Central

11 February 2018

http://europepmc.org/abstract/MED/22965875

author name string

Emilien Delmont

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Europe PubMed Central

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http://europepmc.org/abstract/MED/22965875

Alfonso De Simone

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http://europepmc.org/abstract/MED/22965875

Isabelle Laffont-Proust

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Europe PubMed Central

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http://europepmc.org/abstract/MED/22965875

Nicolas Privat

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Europe PubMed Central

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http://europepmc.org/abstract/MED/22965875

Céline Chapuis

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Europe PubMed Central

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http://europepmc.org/abstract/MED/22965875

Pierre Bedoucha

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http://europepmc.org/abstract/MED/22965875

Jean-Philippe Brandel

10

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http://europepmc.org/abstract/MED/22965875

Annie Laquerriere

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http://europepmc.org/abstract/MED/22965875

Jean-Louis Kemeny

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http://europepmc.org/abstract/MED/22965875

Michel Borg

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http://europepmc.org/abstract/MED/22965875

Human Rezaei

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http://europepmc.org/abstract/MED/22965875

Philippe Derreumaux

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http://europepmc.org/abstract/MED/22965875

Jean-Louis Laplanche

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http://europepmc.org/abstract/MED/22965875

Stéphane Haïk

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http://europepmc.org/abstract/MED/22965875

publication date

10 September 2012

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http://europepmc.org/abstract/MED/22965875

Human Molecular Genetics

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5417-5428

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https://api.crossref.org/works/10.1093%2FHMG%2FDDS377

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Novel proteinaceous infectious particles cause scrapie

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The state of the prion

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21 January 2018

The prion's elusive reason for being

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Mutation in codon 200 of scrapie amyloid protein gene in two clusters of Creutzfeldt-Jakob disease in Slovakia

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

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21 January 2018

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

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21 January 2018

Transmissible familial Creutzfeldt-Jakob disease associated with five, seven, and eight extra octapeptide coding repeats in the PRNP gene

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21 January 2018

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

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21 January 2018

Gerstmann-Sträussler-Scheinker disease and the Indiana kindred

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21 January 2018

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

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21 January 2018

Striking PrPsc heterogeneity in inherited prion diseases with the D178N mutation

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21 January 2018

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

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDS377

21 January 2018

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

1 reference

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21 January 2018

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

1 reference

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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/10.1093%2FHMG%2FDDS377

21 January 2018

Diversity in prion protein oligomerization pathways results from domain expansion as revealed by hydrogen/deuterium exchange and disulfide linkage

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDS377

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDS377

21 January 2018

Estimating prion concentration in fluids and tissues by quantitative PMCA.

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDS377

21 January 2018

In vitro generation of infectious scrapie prions.

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDS377

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDS377

21 January 2018

Effects of human PrPSc type and PRNP genotype in an in-vitro conversion assay

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDS377

21 January 2018

Crossing the species barrier by PrP(Sc) replication in vitro generates unique infectious prions.

1 reference

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21 January 2018

Molecular model of prion transmission to humans

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21 January 2018

Cell-free propagation of prion strains.

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https://api.crossref.org/works/10.1093%2FHMG%2FDDS377

21 January 2018

Homozygous prion protein genotype predisposes to sporadic Creutzfeldt–Jakob disease

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDS377

21 January 2018

Molecular genetics of prion diseases in France. French Research Group on Epidemiology of Human Spongiform Encephalopathies.

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDS377

21 January 2018

Genetic prion disease: the EUROCJD experience.

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDS377

21 January 2018

NMR solution structure of the human prion protein

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDS377

21 January 2018

Novel missense variants of prion protein in Creutzfeldt-Jakob disease or Gerstmann-Sträussler syndrome

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDS377

21 January 2018

Scrapie prion protein structural constraints obtained by limited proteolysis and mass spectrometry

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDS377

21 January 2018

Neurotoxic and gliotrophic activity of a synthetic peptide homologous to Gerstmann-Sträussler-Scheinker disease amyloid protein.

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDS377

21 January 2018

Glimepiride reduces the expression of PrPc, prevents PrPSc formation and protects against prion mediated neurotoxicity in cell lines

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDS377

21 January 2018

Prominent and persistent extraneural infection in human PrP transgenic mice infected with variant CJD.

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDS377

21 January 2018

Regulating factors of PrP glycosylation in Creutzfeldt-Jakob disease--implications for the dissemination and the diagnosis of human prion strains

1 reference

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21 January 2018

High yield purification and physico-chemical properties of full-length recombinant allelic variants of sheep prion protein linked to scrapie susceptibility

1 reference

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21 January 2018

GROMACS: A message-passing parallel molecular dynamics implementation

1 reference

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21 January 2018

Structural and hydration properties of the partially unfolded states of the prion protein

1 reference

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21 January 2018

Prion propagation in mice expressing human and chimeric PrP transgenes implicates the interaction of cellular PrP with another protein

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDS377

21 January 2018

Ultra-efficient replication of infectious prions by automated protein misfolding cyclic amplification.

1 reference

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21 January 2018

The oligomerization properties of prion protein are restricted to the H2H3 domain

1 reference

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21 January 2018

Identifiers

10.1093/HMG/DDS377

1 reference

Europe PubMed Central

11 February 2018

http://europepmc.org/abstract/MED/22965875

1 reference

Europe PubMed Central

11 February 2018

http://europepmc.org/abstract/MED/22965875

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