Familial prion protein mutants inhibit Hrd1-mediated retrotranslocation of misfolded proteins by depleting misfolded protein sensor BiP. (Q36581422)

15 August 2017

Familial prion protein mutants inhibit Hrd1-mediated retrotranslocation of misfolded proteins by depleting misfolded protein sensor BiP. (English)

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15 August 2017

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Sarah L Peters

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15 August 2017

Marc-André Déry

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2

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15 August 2017

Andrea C LeBlanc

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3

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15 August 2017

5 January 2016

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15 August 2017

Human Molecular Genetics

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15 August 2017

25

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15 August 2017

5

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15 August 2017

976-988

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describes a project that uses

15 August 2017

ImageJ

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11 June 2022

https://www.ebi.ac.uk/europepmc/webservices/rest/PMC4754050/fullTextXML

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9 September 2017

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Oral treatment targeting the unfolded protein response prevents neurodegeneration and clinical disease in prion-infected mice

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9 September 2017

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Changes in BiP availability reveal hypersensitivity to acute endoplasmic reticulum stress in cells expressing mutant huntingtin

9 September 2017

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9 September 2017

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Expression of mutant or cytosolic PrP in transgenic mice and cells is not associated with endoplasmic reticulum stress or proteasome dysfunction

9 September 2017

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Familial CJD associated PrP mutants within transmembrane region induced Ctm-PrP retention in ER and triggered apoptosis by ER stress in SH-SY5Y cells

9 September 2017

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Yokukansan Inhibits Neuronal Death during ER Stress by Regulating the Unfolded Protein Response

9 September 2017

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Loss of anti-Bax function in Gerstmann-Sträussler-Scheinker syndrome-associated prion protein mutants

9 September 2017

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Selective processing and metabolism of disease-causing mutant prion proteins

9 September 2017

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N-glycosylation enhances presentation of a MHC class I-restricted epitope from tyrosinase

9 September 2017

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Role of GRP78/BiP degradation and ER stress in deoxynivalenol-induced interleukin-6 upregulation in the macrophage.

9 September 2017

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A randomized cross-over study of a traditional Japanese medicine (kampo), yokukansan, in the treatment of the behavioural and psychological symptoms of dementia

9 September 2017

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Cytosolic prion protein is the predominant anti-Bax prion protein form: exclusion of transmembrane and secreted prion protein forms in the anti-Bax function

9 September 2017

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OS-9 and GRP94 deliver mutant alpha1-antitrypsin to the Hrd1-SEL1L ubiquitin ligase complex for ERAD

9 September 2017

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Unfolded protein response transcription factor XBP-1 does not influence prion replication or pathogenesis.

9 September 2017

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ER stress differentially regulates the stabilities of ERAD ubiquitin ligases and their substrates

9 September 2017

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Ubiquitin ligase Hrd1 enhances the degradation and suppresses the toxicity of polyglutamine-expanded huntingtin

9 September 2017

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A ubiquitin ligase HRD1 promotes the degradation of Pael receptor, a substrate of Parkin

9 September 2017

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From acute ER stress to physiological roles of the Unfolded Protein Response

9 September 2017

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Mutations in SIL1 cause Marinesco-Sjögren syndrome, a cerebellar ataxia with cataract and myopathy

9 September 2017

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Retrotranslocation of the chaperone calreticulin from the endoplasmic reticulum lumen to the cytosol

9 September 2017

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Protein accumulation and neurodegeneration in the woozy mutant mouse is caused by disruption of SIL1, a cochaperone of BiP

9 September 2017

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Protein disulfide bond formation in the cytoplasm during oxidative stress

9 September 2017

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Human HRD1 is an E3 ubiquitin ligase involved in degradation of proteins from the endoplasmic reticulum

9 September 2017

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Ubiquitin, proteasomes, and the aging brain

9 September 2017

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Synaptic pathology and cell death in the cerebellum in Creutzfeldt-Jakob disease

9 September 2017

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A novel mammalian endoplasmic reticulum ubiquitin ligase homologous to the yeast Hrd1

9 September 2017

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Human HRD1 protects against ER stress-induced apoptosis through ER-associated degradation

9 September 2017

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Overexpressed protein disulfide isomerase in brains of patients with sporadic Creutzfeldt-Jakob disease

9 September 2017

1 reference

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IRE1-mediated unconventional mRNA splicing and S2P-mediated ATF6 cleavage merge to regulate XBP1 in signaling the unfolded protein response

9 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4754050

Wild-type PrP and a mutant associated with prion disease are subject to retrograde transport and proteasome degradation

9 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4754050

The tumor autocrine motility factor receptor, gp78, is a ubiquitin protein ligase implicated in degradation from the endoplasmic reticulum

9 September 2017

1 reference

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Proteasomes and ubiquitin are involved in the turnover of the wild-type prion protein

9 September 2017

1 reference

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Presenilin-1 mutations downregulate the signalling pathway of the unfolded-protein response

9 September 2017

1 reference

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Identification of the cis-acting endoplasmic reticulum stress response element responsible for transcriptional induction of mammalian glucose-regulated proteins. Involvement of basic leucine zipper transcription factors

9 September 2017

1 reference

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Meningocerebrovascular amyloidosis associated with a novel transthyretin mis-sense mutation at codon 18 (TTRD 18G)

9 September 2017

1 reference

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Cleavage of Structural Proteins during the Assembly of the Head of Bacteriophage T4

9 September 2017

1 reference

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Rapid redistribution of Golgi proteins into the ER in cells treated with brefeldin A: evidence for membrane cycling from Golgi to ER

9 September 2017

1 reference

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Mouse polyclonal and monoclonal antibody to scrapie-associated fibril proteins

9 September 2017

1 reference

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Loss of HRD1-mediated protein degradation causes amyloid precursor protein accumulation and amyloid-beta generation

9 September 2017

1 reference

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A different pathway in the endoplasmic reticulum stress-induced expression of human HRD1 and SEL1 genes

30 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4754050

Defective retrotranslocation causes loss of anti-Bax function in human familial prion protein mutants.

30 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4754050

Cellular prion protein inhibits proapoptotic Bax conformational change in human neurons and in breast carcinoma MCF-7 cells

30 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4754050

Caspase-12 and endoplasmic reticulum stress mediate neurotoxicity of pathological prion protein.

30 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4754050

Prion protein protects human neurons against Bax-mediated apoptosis

30 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4754050

A shared endoplasmic reticulum-associated degradation pathway involving the EDEM1 protein for glycosylated and nonglycosylated proteins

30 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4754050

The Endoplasmic Reticulum-associated Degradation of Transthyretin Variants Is Negatively Regulated by BiP in Mammalian Cells

28 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4754050

Misfolded BiP is degraded by a proteasome-independent endoplasmic-reticulum-associated degradation pathway

28 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4754050

Neuronal apoptosis in Creutzfeldt-Jakob disease

28 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4754050

Neuronal apoptosis in fatal familial insomnia

28 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4754050

Cytosolic prion protein is not toxic and protects against Bax-mediated cell death in human primary neurons

28 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4754050

Neurotoxicity and neurodegeneration when PrP accumulates in the cytosol

20 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4754050

The Chaperone Protein BiP Binds to a Mutant Prion Protein and Mediates Its Degradation by the Proteasome

20 September 2018

1 reference

12 December 2020

Proteasomal Degradation and N-terminal Protease Resistance of the Codon 145 Mutant Prion Protein

1 reference

12 December 2020

Endoplasmic reticulum stress features are prominent in Alzheimer disease but not in prion diseases in vivo

1 reference

12 December 2020

Cellular tolerance of prion protein PrP in yeast involves proteolysis and the unfolded protein response

1 reference

12 December 2020

Transcriptional induction of mammalian ER quality control proteins is mediated by single or combined action of ATF6alpha and XBP1

1 reference

12 December 2020

Identifiers

DOI

10.1093/HMG/DDV630

1 reference

15 August 2017

1 reference

15 August 2017

PubMed publication ID

26740554

1 reference