The unfolded protein response affects readthrough of premature termination codons (Q33623379)

28 July 2017

The unfolded protein response affects readthrough of premature termination codons (English)

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28 July 2017

Yifat S Oren

1

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28 July 2017

Michelle L McClure

2

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28 July 2017

Steven M Rowe

3

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28 July 2017

Eric J Sorscher

4

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28 July 2017

Assaf C Bester

5

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28 July 2017

Miriam Manor

6

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28 July 2017

Eitan Kerem

7

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28 July 2017

Joseph Rivlin

8

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28 July 2017

Fouad Zahdeh

9

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28 July 2017

Matthias Mann

10

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28 July 2017

Tamar Geiger

11

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28 July 2017

Batsheva Kerem

12

1 reference

28 July 2017

1 May 2014

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28 July 2017

EMBO Molecular Medicine

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28 July 2017

6

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28 July 2017

5

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28 July 2017

685-701

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describes a project that uses

28 July 2017

ImageJ

1 reference

11 June 2022

https://www.ebi.ac.uk/europepmc/webservices/rest/PMC4023889/fullTextXML

inferred from PubMed Central ID database lookup

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21 June 2018

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21 June 2018

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21 June 2018

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21 June 2018

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21 June 2018

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Comparative proteomic analysis of eleven common cell lines reveals ubiquitous but varying expression of most proteins

21 June 2018

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21 June 2018

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21 June 2018

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Overexpression of the c-myc oncogene inhibits nonsense-mediated RNA decay in B lymphocytes

21 June 2018

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Readthrough of premature termination codons in the adenomatous polyposis coli gene restores its biological activity in human cancer cells

21 June 2018

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Complementary cell-based high-throughput screens identify novel modulators of the unfolded protein response

21 June 2018

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Suppression of CFTR premature termination codons and rescue of CFTR protein and function by the synthetic aminoglycoside NB54

21 June 2018

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Inhibition of nonsense-mediated RNA decay by the tumor microenvironment promotes tumorigenesis

21 June 2018

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Quantitative, high-resolution proteomics for data-driven systems biology

21 June 2018

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Role of endoplasmic reticulum stress in cystic fibrosis-related airway inflammatory responses

21 June 2018

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Nonsense-mediated RNA decay regulation by cellular stress: implications for tumorigenesis.

21 June 2018

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The ubiquitin landscape at DNA double-strand breaks

21 June 2018

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Principles of ubiquitin and SUMO modifications in DNA repair.

21 June 2018

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NMD: multitasking between mRNA surveillance and modulation of gene expression

21 June 2018

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Introducing sense into nonsense in treatments of human genetic diseases

21 June 2018

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Transcriptional induction of the human asparagine synthetase gene during the unfolded protein response does not require the ATF6 and IRE1/XBP1 arms of the pathway

21 June 2018

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Mutations in LPIN1 cause recurrent acute myoglobinuria in childhood.

21 June 2018

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From endoplasmic-reticulum stress to the inflammatory response

21 June 2018

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Hypoxic inhibition of nonsense-mediated RNA decay regulates gene expression and the integrated stress response

21 June 2018

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Eukaryotic ribosomal RNA determinants of aminoglycoside resistance and their role in translational fidelity.

21 June 2018

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How transmembrane proteins sense endoplasmic reticulum stress

21 June 2018

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Mutations in UPF3B, a member of the nonsense-mediated mRNA decay complex, cause syndromic and nonsyndromic mental retardation

21 June 2018

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The efficiency of nonsense-mediated mRNA decay is an inherent character and varies among different cells

21 June 2018

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Signal integration in the endoplasmic reticulum unfolded protein response

21 June 2018

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Restoration of W1282X CFTR activity by enhanced expression

21 June 2018

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The protective and destructive roles played by molecular chaperones during ERAD (endoplasmic-reticulum-associated degradation).

21 June 2018

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PTC124 targets genetic disorders caused by nonsense mutations

21 June 2018

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Safety, tolerability, and pharmacokinetics of PTC124, a nonaminoglycoside nonsense mutation suppressor, following single- and multiple-dose administration to healthy male and female adult volunteers

21 June 2018

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An alternative branch of the nonsense-mediated decay pathway

21 June 2018

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The nonsense-mediated decay RNA surveillance pathway

21 June 2018

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https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4023889

No detectable improvements in cystic fibrosis transmembrane conductance regulator by nasal aminoglycosides in patients with cystic fibrosis with stop mutations

21 June 2018

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https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4023889

Nonsense-mediated mRNA decay affects nonsense transcript levels and governs response of cystic fibrosis patients to gentamicin

21 June 2018

1 reference

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Domain interdependence in the biosynthetic assembly of CFTR.

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4023889

Applying nonsense-mediated mRNA decay research to the clinic: progress and challenges

21 June 2018

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https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4023889

XLF interacts with the XRCC4-DNA ligase IV complex to promote DNA nonhomologous end-joining

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4023889

Cernunnos, a novel nonhomologous end-joining factor, is mutated in human immunodeficiency with microcephaly

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4023889

Nonsense surveillance regulates expression of diverse classes of mammalian transcripts and mutes genomic noise

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4023889

The unfolded protein response--a stress signaling pathway of the endoplasmic reticulum

21 June 2018

1 reference

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Nonsense-mediated decay approaches the clinic

21 June 2018

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Gentamicin-Induced Correction of CFTR Function in Patients with Cystic Fibrosis andCFTRStop Mutations

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4023889

Separable roles for rent1/hUpf1 in altered splicing and decay of nonsense transcripts

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4023889

When the message goes awry: disease-producing mutations that influence mRNA content and performance.

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4023889

SC35 autoregulates its expression by promoting splicing events that destabilize its mRNAs

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4023889

Gene ontology: tool for the unification of biology

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4023889

Nonsense-mediated mRNA decay in health and disease

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4023889

Aminoglycoside antibiotics restore dystrophin function to skeletal muscles of mdx mice

21 June 2018

1 reference

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Basis for prokaryotic specificity of action of aminoglycoside antibiotics

21 June 2018

1 reference

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Stress signaling from the lumen of the endoplasmic reticulum: coordination of gene transcriptional and translational controls

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4023889

RNA surveillance. Unforeseen consequences for gene expression, inherited genetic disorders and cancer

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4023889

Structure of the A site of Escherichia coli 16S ribosomal RNA complexed with an aminoglycoside antibiotic

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4023889

RNA sequence determinants for aminoglycoside binding to an A-site rRNA model oligonucleotide

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4023889

Suppression of a nonsense mutation in mammalian cells in vivo by the aminoglycoside antibiotics G-418 and paromomycin

21 June 2018

1 reference

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Aminoglycoside suppression at UAG, UAA and UGA codons in Escherichia coli and human tissue culture cells

21 June 2018

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Extensive posttranscriptional deletion of the coding sequences for part of nucleotide-binding fold 1 in respiratory epithelial mRNA transcripts of the cystic fibrosis transmembrane conductance regulator gene is not associated with the clinical manif

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4023889

Interference of nonsense mutations with eukaryotic messenger RNA stability

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4023889

SnapShot: Nonsense-mediated mRNA decay.

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4023889

Ataluren (PTC124) induces cystic fibrosis transmembrane conductance regulator protein expression and activity in children with nonsense mutation cystic fibrosis

30 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4023889

Restoration of APC gene function in colorectal cancer cells by aminoglycoside- and macrolide-induced read-through of premature termination codons

30 October 2018

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The unfolded protein response in health and disease.

30 October 2018

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Airway epithelial inflammation-induced endoplasmic reticulum Ca2+ store expansion is mediated by X-box binding protein-1.

30 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4023889

Effectiveness of PTC124 treatment of cystic fibrosis caused by nonsense mutations: a prospective phase II trial

30 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4023889

DHCR7 nonsense mutations and characterisation of mRNA nonsense mediated decay in Smith-Lemli-Opitz syndrome

30 October 2018

1 reference

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Gentamicin administration in Duchenne patients with premature stop codon. Preliminary results

30 October 2018

1 reference

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Gentamicin treatment of Duchenne and Becker muscular dystrophy due to nonsense mutations

30 October 2018

1 reference

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Functional and quantitative proteomics using SILAC

30 October 2018

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Gentamicin fails to increase dystrophin expression in dystrophin-deficient muscle

30 October 2018

1 reference

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Database on the structure of small ribosomal subunit RNA

30 October 2018

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12 December 2020

Correlation of sweat chloride concentration with classes of the cystic fibrosis transmembrane conductance regulator gene mutations

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12 December 2020

A novel therapeutic approach for genetic diseases by introduction of suppressor tRNA

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12 December 2020

The unfolded protein response

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12 December 2020

The mechanism of cystic fibrosis transmembrane conductance regulator transcriptional repression during the unfolded protein response

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12 December 2020

Activation of the unfolded protein response and alternative splicing of ATF6α in HLA-B27 positive lymphocytes

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12 December 2020

A pilot study of the effect of gentamicin on nasal potential difference measurements in cystic fibrosis patients carrying stop mutations

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12 December 2020

R-domain interactions with distal regions of CFTR lead to phosphorylation and activation

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12 December 2020

Identifiers

DOI

10.1002/EMMM.201303347

1 reference

28 July 2017

1 reference

28 July 2017

1 reference