(Q39680946)

13 September 2017

title

The hydrophobic core region governs mutant prion protein aggregation and intracellular retention (English)

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13 September 2017

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Emiliano Biasini

1

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13 September 2017

Laura Tapella

2

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13 September 2017

Elena Restelli

3

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13 September 2017

Manuela Pozzoli

4

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13 September 2017

Tania Massignan

5

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13 September 2017

Roberto Chiesa

6

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13 September 2017

language of work or name

English

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publication date

1 September 2010

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13 September 2017

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13 September 2017

volume

430

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13 September 2017

issue

3

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13 September 2017

page(s)

477-486

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Fishing for prion protein function

13 September 2017

cites work

1 reference

https://api.crossref.org/works/10.1042%2FBJ20100615

Novel proteinaceous infectious particles cause scrapie

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Cell-free formation of protease-resistant prion protein.

21 January 2018

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In vitro generation of infectious scrapie prions.

21 January 2018

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21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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Mutant prion protein expression causes motor and memory deficits and abnormal sleep patterns in a transgenic mouse model.

21 January 2018

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Spontaneous generation of prion infectivity in fatal familial insomnia knockin mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBJ20100615

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBJ20100615

Cytosolic prion protein (PrP) is not toxic in N2a cells and primary neurons expressing pathogenic PrP mutations

21 January 2018

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Prion diseases: what is the neurotoxic molecule?

21 January 2018

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Mutant prion protein expression is associated with an alteration of the Rab GDP dissociation inhibitor alpha (GDI)/Rab11 pathway

21 January 2018

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Stressing out the ER: a role of the unfolded protein response in prion-related disorders

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBJ20100615

A conformational transition at the N terminus of the prion protein features in formation of the scrapie isoform

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBJ20100615

Predicted alpha-helical regions of the prion protein when synthesized as peptides form amyloid

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBJ20100615

Synthetic peptides homologous to prion protein residues 106-147 form amyloid-like fibrils in vitro

21 January 2018

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Structural properties of Gerstmann-Straussler-Scheinker disease amyloid protein.

21 January 2018

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The deletion of amino acids 114-121 in the TM1 domain of mouse prion protein stabilizes its conformation but does not affect the overall structure.

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBJ20100615

The AGAAAAGA palindrome in PrP is required to generate a productive PrPSc-PrPC complex that leads to prion propagation

21 January 2018

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https://api.crossref.org/works/10.1042%2FBJ20100615

Rapid and discriminatory diagnosis of scrapie and BSE in retro-pharyngeal lymph nodes of sheep

21 January 2018

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

21 January 2018

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Cultured cell sublines highly susceptible to prion infection

21 January 2018

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https://api.crossref.org/works/10.1042%2FBJ20100615

Mutant and infectious prion proteins display common biochemical properties in cultured cells.

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBJ20100615

Non-infectious aggregates of the prion protein react with several PrPSc-directed antibodies.

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBJ20100615

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBJ20100615

Mutant prion proteins are partially retained in the endoplasmic reticulum

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBJ20100615

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBJ20100615

Prion proteins carrying pathogenic mutations are resistant to phospholipase cleavage of their glycolipid anchors

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBJ20100615

NMR solution structure of the human prion protein

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBJ20100615

NMR structure of the bovine prion protein isolated from healthy calf brains

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBJ20100615

Lethal recessive myelin toxicity of prion protein lacking its central domain

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBJ20100615

Identification of an intracellular site of prion conversion

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBJ20100615

Molecular distinction between pathogenic and infectious properties of the prion protein

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBJ20100615

Molecular determinants of the physicochemical properties of a critical prion protein region comprising residues 106-126.

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBJ20100615

The hydrophobic core sequence modulates the neurotoxic and secondary structure properties of the prion peptide 106-126.

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBJ20100615

Cellular phenotyping of secretory and nuclear prion proteins associated with inherited prion diseases

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBJ20100615

Mutant prion protein-mediated aggregation of normal prion protein in the endoplasmic reticulum: implications for prion propagation and neurotoxicity

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBJ20100615

Mutant prion protein D202N associated with familial prion disease is retained in the endoplasmic reticulum and forms 'curly' intracellular aggregates

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBJ20100615

Selective processing and metabolism of disease-causing mutant prion proteins

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBJ20100615

A novel, drug-based, cellular assay for the activity of neurotoxic mutants of the prion protein.

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBJ20100615

Preventing misfolding of the prion protein by trimethylamine N-oxide

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBJ20100615

Doxycycline and protein folding agents rescue the abnormal phenotype of familial CJD H187R in a cell model

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBJ20100615

Variety of antiprion compounds discovered through an in silico screen based on cellular-form prion protein structure: Correlation between antiprion activity and binding affinity.

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBJ20100615

The Metabolism and Imaging in Live Cells of the Bovine Prion Protein in Its Native Form or Carrying Single Amino Acid Substitutions

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBJ20100615

21 January 2018

Identifiers

DOI

10.1042/BJ20100615

1 reference

13 September 2017

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