Early onset prion disease from octarepeat expansion correlates with copper binding properties (Q28910222)

7 March 2017

title

Early onset prion disease from octarepeat expansion correlates with copper binding properties (English)

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7 March 2017

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GOA release 2020-03-11

copper

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author name string

Daniel J. Stevens

1

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7 March 2017

Eric D. Walter

2

1 reference

7 March 2017

Abel Rodríguez

3

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7 March 2017

David Draper

4

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7 March 2017

Paul Davies

5

1 reference

7 March 2017

David R. Brown

6

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7 March 2017

Glenn L. Millhauser

7

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7 March 2017

publication date

1 April 2009

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7 March 2017

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7 March 2017

volume

5

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7 March 2017

issue

4

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7 March 2017

page(s)

e1000390

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Creative Commons Attribution 4.0 International

7 March 2017

copyright license

start time

17 April 2009

1 reference

April 2022 Public Data File from Crossref

copyright status

copyrighted

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cites work

Probing the role of PrP repeats in conformational conversion and amyloid assembly of chimeric yeast prions

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2663819

Protein NMR structure determination with automated NOE assignment using the new software CANDID and the torsion angle dynamics algorithm DYANA

16 March 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2663819

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

16 March 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2663819

Shattuck lecture--neurodegenerative diseases and prions

16 March 2017

2 references

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2663819

16 March 2017

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2663819

Copper stimulates endocytosis of the prion protein

6 April 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2663819

16 March 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2663819

Nerve endings from rat brain tissue release copper upon depolarization. A possible role in regulating neuronal excitability

16 March 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2663819

The prion protein is a combined zinc and copper binding protein: Zn2+ alters the distribution of Cu2+ coordination modes

16 March 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2663819

Inherited prion disease with 5-OPRI: phenotype modification by repeat length and codon 129.

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2663819

Familial prion disease in a Hungarian family with a novel 144-base pair insertion in the prion protein gene

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2663819

Lethal recessive myelin toxicity of prion protein lacking its central domain

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2663819

Copper and the prion protein: methods, structures, function, and disease

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2663819

Octapeptide repeat insertions increase the rate of protease-resistant prion protein formation

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2663819

The expanded octarepeat domain selectively binds prions and disrupts homomeric prion protein interactions

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2663819

The octarepeat domain of the prion protein binds Cu(II) with three distinct coordination modes at pH 7.4.

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2663819

Clinical course in young patients with sporadic Creutzfeldt-Jakob disease

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2663819

Octapeptide repeat insertions in the prion protein gene and early onset dementia

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2663819

Copper binding in the prion protein

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2663819

Cellular prion protein function in copper homeostasis and redox signalling at the synapse

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2663819

Molecular distinction between pathogenic and infectious properties of the prion protein

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2663819

Rapidly progressive dementia syndrome associated with a novel four extra repeat mutation in the prion protein gene

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2663819

Mutant prion proteins are partially retained in the endoplasmic reticulum

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2663819

Identification of the Cu2+ binding sites in the N-terminal domain of the prion protein by EPR and CD spectroscopy

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2663819

Creutzfeldt-Jakob disease with a novel four extra-repeat insertional mutation in the PrP gene

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2663819

Accumulation of protease-resistant prion protein (PrP) and apoptosis of cerebellar granule cells in transgenic mice expressing a PrP insertional mutation

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2663819

Familial Creutzfeldt-Jakob disease with a novel 120-bp insertion in the prion protein gene

3 June 2018

2 references

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2663819

3 June 2018

https://api.crossref.org/works/10.1371%2FJOURNAL.PPAT.1000390

Solution structure of Syrian hamster prion protein rPrP(90-231).

21 January 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2663819

Abnormal properties of prion protein with insertional mutations in different cell types

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2663819

Prion diseases and the BSE crisis

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2663819

Familial prion disease with a novel 144-bp insertion in the prion protein gene in a Basque family.

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2663819

Familial Creutzfeldt-Jakob disease with a five-repeat octapeptide insert mutation

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2663819

Prion disease associated with a novel nine octapeptide repeat insertion in the PRNP gene.

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2663819

Two mutant prion proteins expressed in cultured cells acquire biochemical properties reminiscent of the scrapie isoform.

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2663819

A Mutant Prion Protein Displays an Aberrant Membrane Association When Expressed in Cultured Cells

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2663819

A peculiar type of presenile dementia

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2663819

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

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2663819

An insert mutation in the chromosome 20 amyloid precursor gene in a Gerstmann-Sträussler-Scheinker family

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2663819

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

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2663819

A dementing illness associated with a novel insertion in the prion protein gene

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2663819

Insertion in prion protein gene in familial Creutzfeldt-Jakob disease

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2663819

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

28 July 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PPAT.1000390

Transgenic mice expressing bovine PrP with a four extra repeat octapeptide insert mutation show a spontaneous, non-transmissible, neurodegenerative disease and an expedited course of BSE infection.

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PPAT.1000390

A protease-resistant protein is a structural component of the scrapie prion

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PPAT.1000390

The affinity of copper binding to the prion protein octarepeat domain: evidence for negative cooperativity

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PPAT.1000390

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PPAT.1000390

Structure, stability, and ligand exchange of copper(II) complexes with oxidized glutathione

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PPAT.1000390

Methods for studying synaptosomal copper release

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PPAT.1000390

Neonatal lethality in transgenic mice expressing prion protein with a deletion of residues 105-125.

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PPAT.1000390

The low-density lipoprotein receptor-related protein 1 (LRP1) mediates the endocytosis of the cellular prion protein

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PPAT.1000390

Copper(II) inhibits in vitro conversion of prion protein into amyloid fibrils.

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PPAT.1000390

Hydrogen peroxide cleavage of the prion protein generates a fragment able to initiate polymerisation of full length prion protein.

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PPAT.1000390

High affinity binding between copper and full-length prion protein identified by two different techniques

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PPAT.1000390

Two novel insertions in the prion protein gene in patients with lateonset dementia

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PPAT.1000390

Creutzfeldt-Jakob disease with a novel extra-repeat insertional mutation in the PRNP gene.

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PPAT.1000390

A prion disease with a novel 96-base pair insertional mutation in the prion protein gene

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PPAT.1000390

A case with a 120 base pair insertional mutation in the prion protein gene: the first case in Japan

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PPAT.1000390

Prion disease with 144 base pair insertion in a Japanese family line

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PPAT.1000390

Phenotypic variability in the brains of a family with a prion disease characterized by a 144-base pair insertion in the prion protein gene

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PPAT.1000390

Prion disease with a 144 base pair insertion: unusual cerebellar prion protein immunoreactivity

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PPAT.1000390

Novel prion protein insert mutation associated with prolonged neurodegenerative illness

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PPAT.1000390

Dementia associated with a 216 base pair insertion in the prion protein gene. Clinical and neuropathological features

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PPAT.1000390

Prominent psychiatric features and early onset in an inherited prion disease with a new insertional mutation in the prion protein gene

21 January 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2663819

Creutzfeldt-Jakob disease with a novel insertion and codon 219 Lys/Lys polymorphism in PRNP

26 November 2018

1 reference

12 December 2020

Prion protein binds copper within the physiological concentration range

1 reference

12 December 2020

Expansion of the octarepeat domain alters the misfolding pathway but not the folding pathway of the prion protein

1 reference

12 December 2020

Familial Creutzfeldt-Jakob disease in a patient carrying both a presenilin 1 missense substitution and a prion protein gene insertion

1 reference

12 December 2020

Creutzfeldt-Jakob disease in a Chinese patient with a novel seven extra-repeat insertion in PRNP

1 reference

12 December 2020

A novel three extra-repeat insertion in the prion protein gene (PRNP) in a patient with Creutzfeldt-Jakob disease

1 reference

12 December 2020

A new mutation in the prion protein gene: a patient with dementia and white matter changes

1 reference

12 December 2020

Hypokinesia and presenile dementia in a Dutch family with a novel insertion in the prion protein gene

1 reference

12 December 2020

10.1371/JOURNAL.PPAT.1000390

Identifiers

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