(Q33799590)

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Synthetic aminoglycosides efficiently suppress cystic fibrosis transmembrane conductance regulator nonsense mutations and are enhanced by ivacaftor

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scholarly article

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Europe PubMed Central

PubMed publication ID

29 July 2017

Synthetic aminoglycosides efficiently suppress cystic fibrosis transmembrane conductance regulator nonsense mutations and are enhanced by ivacaftor (English)

1 reference

Europe PubMed Central

PubMed publication ID

29 July 2017

cystic fibrosis

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transmembrane protein

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author name string

Xiaojiao Xue

1

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Europe PubMed Central

PubMed publication ID

29 July 2017

Venkateshwar Mutyam

2

1 reference

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29 July 2017

Liping Tang

3

1 reference

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29 July 2017

Silpak Biswas

4

1 reference

Europe PubMed Central

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29 July 2017

Ming Du

5

1 reference

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29 July 2017

Laura A Jackson

6

1 reference

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29 July 2017

Yanying Dai

7

1 reference

Europe PubMed Central

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29 July 2017

Valery Belakhov

8

1 reference

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29 July 2017

Moran Shalev

9

1 reference

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29 July 2017

Fuquan Chen

10

1 reference

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29 July 2017

Jochen Schacht

11

1 reference

Europe PubMed Central

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29 July 2017

Robert J Bridges

12

1 reference

Europe PubMed Central

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29 July 2017

Timor Baasov

13

1 reference

Europe PubMed Central

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29 July 2017

Jeong Hong

14

1 reference

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29 July 2017

David M Bedwell

15

1 reference

Europe PubMed Central

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29 July 2017

Steven M Rowe

16

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29 July 2017

language of work or name

1 reference

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publication date

1 April 2014

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Europe PubMed Central

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29 July 2017

American Journal of Respiratory Cell and Molecular Biology

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Europe PubMed Central

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29 July 2017

50

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Europe PubMed Central

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29 July 2017

4

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Europe PubMed Central

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29 July 2017

805-816

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Europe PubMed Central

PubMed publication ID

29 July 2017

Attenuation of nonsense-mediated mRNA decay enhances in vivo nonsense suppression

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4068923

4 July 2018

Development of generic immunoassay for the detection of a series of aminoglycosides with 6'-OH group for the treatment of genetic diseases in biological samples.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4068923

4 July 2018

Increased selectivity toward cytoplasmic versus mitochondrial ribosome confers improved efficiency of synthetic aminoglycosides in fixing damaged genes: a strategy for treatment of genetic diseases caused by nonsense mutations

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4068923

4 July 2018

NMD: a multifaceted response to premature translational termination

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4068923

4 July 2018

A pharmacologic approach to acquired cystic fibrosis transmembrane conductance regulator dysfunction in smoking related lung disease

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4068923

4 July 2018

Suppression of premature termination codons as a therapeutic approach.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4068923

4 July 2018

Ivacaftor potentiation of multiple CFTR channels with gating mutations

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4068923

4 July 2018

Requirements for Efficient Correction of ΔF508 CFTR Revealed by Analyses of Evolved Sequences

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4068923

4 July 2018

Pharmacological considerations for the proper clinical use of aminoglycosides

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4068923

4 July 2018

The designer aminoglycoside NB84 significantly reduces glycosaminoglycan accumulation associated with MPS I-H in the Idua-W392X mouse

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4068923

4 July 2018

A CFTR potentiator in patients with cystic fibrosis and the G551D mutation

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4068923

4 July 2018

Correction of the F508del-CFTR protein processing defect in vitro by the investigational drug VX-809.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4068923

4 July 2018

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4068923

4 July 2018

Regulatory domain phosphorylation to distinguish the mechanistic basis underlying acute CFTR modulators

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4068923

4 July 2018

Readthrough of nonsense mutations in Rett syndrome: evaluation of novel aminoglycosides and generation of a new mouse model

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4068923

4 July 2018

The effect of gentamicin-induced readthrough on a novel premature termination codon of CD18 leukocyte adhesion deficiency patients

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4068923

4 July 2018

Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4068923

4 July 2018

Production of beta-globin and adult hemoglobin following G418 treatment of erythroid precursor cells from homozygous beta(0)39 thalassemia patients

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4068923

4 July 2018

CFTR delivery to 25% of surface epithelial cells restores normal rates of mucus transport to human cystic fibrosis airway epithelium

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4068923

4 July 2018

Development of novel aminoglycoside (NB54) with reduced toxicity and enhanced suppression of disease-causing premature stop mutations

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4068923

4 July 2018

Mechanism of PTC124 activity in cell-based luciferase assays of nonsense codon suppression

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4068923

4 July 2018

Aminoglycoside-induced histone deacetylation and hair cell death in the mouse cochlea

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4068923

4 July 2018

PTC124 is an orally bioavailable compound that promotes suppression of the human CFTR-G542X nonsense allele in a CF mouse model

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4068923

4 July 2018

In vitro and ex vivo suppression by aminoglycosides of PCDH15 nonsense mutations underlying type 1 Usher syndrome

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4068923

4 July 2018

Restoration of W1282X CFTR activity by enhanced expression

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4068923

4 July 2018

PTC124 targets genetic disorders caused by nonsense mutations

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4068923

4 July 2018

In vitro prediction of stop-codon suppression by intravenous gentamicin in patients with cystic fibrosis: a pilot study

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4068923

4 July 2018

Cystic fibrosis

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4068923

4 July 2018

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4068923

4 July 2018

Aminoglycoside suppression of a premature stop mutation in a Cftr-/- mouse carrying a human CFTR-G542X transgene.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4068923

4 July 2018

Aminoglycoside-mediated suppression of nonsense mutations in late infantile neuronal ceroid lipofuscinosis

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4068923

4 July 2018

Evidence that systemic gentamicin suppresses premature stop mutations in patients with cystic fibrosis

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4068923

4 July 2018

Gentamicin-mediated suppression of Hurler syndrome stop mutations restores a low level of alpha-L-iduronidase activity and reduces lysosomal glycosaminoglycan accumulation

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4068923

4 July 2018

Aminoglycoside antibiotics

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4068923

4 July 2018

Aminoglycoside antibiotics restore dystrophin function to skeletal muscles of mdx mice

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4068923

4 July 2018

A dual-luciferase reporter system for studying recoding signals

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4068923

4 July 2018

Suppression of a CFTR premature stop mutation in a bronchial epithelial cell line

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4068923

4 July 2018

The relation between genotype and phenotype in cystic fibrosis--analysis of the most common mutation (delta F508).

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4068923

4 July 2018

Efficiency of gene transfer for restoration of normal airway epithelial function in cystic fibrosis

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4068923

4 July 2018

Poly-L-aspartic acid enhances and prolongs gentamicin-mediated suppression of the CFTR-G542X mutation in a cystic fibrosis mouse model

1 reference

https://api.crossref.org/works/10.1165%2FRCMB.2013-0282OC

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1165%2FRCMB.2013-0282OC

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1165%2FRCMB.2013-0282OC

21 January 2018

Release factor RF-3 GTPase activity acts in disassembly of the ribosome termination complex

1 reference

https://api.crossref.org/works/10.1165%2FRCMB.2013-0282OC

21 January 2018

Repairing faulty genes by aminoglycosides: development of new derivatives of geneticin (G418) with enhanced suppression of diseases-causing nonsense mutations

1 reference

https://api.crossref.org/works/10.1165%2FRCMB.2013-0282OC

21 January 2018

alpha-L-iduronidase premature stop codons and potential read-through in mucopolysaccharidosis type I patients.

1 reference

https://api.crossref.org/works/10.1165%2FRCMB.2013-0282OC

21 January 2018

Chronic ataluren (PTC124) treatment of nonsense mutation cystic fibrosis

1 reference

https://api.crossref.org/works/10.1165%2FRCMB.2013-0282OC

21 January 2018

Gentamicin-induced readthrough of stop codons in Duchenne muscular dystrophy

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4068923

30 October 2018

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4068923

30 October 2018

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4068923

30 October 2018

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/24251786

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Sequence specificity of aminoglycoside-induced stop condon readthrough: potential implications for treatment of Duchenne muscular dystrophy

1 reference

https://pubmed.ncbi.nlm.nih.gov/24251786

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Correction of lethal intestinal defect in a mouse model of cystic fibrosis by human CFTR

1 reference

https://pubmed.ncbi.nlm.nih.gov/24251786

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Aminoglycoside antibiotics restore CFTR function by overcoming premature stop mutations

1 reference

https://pubmed.ncbi.nlm.nih.gov/24251786

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Clinical doses of amikacin provide more effective suppression of the human CFTR-G542X stop mutation than gentamicin in a transgenic CF mouse model

1 reference

https://pubmed.ncbi.nlm.nih.gov/24251786

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Identifiers

10.1165/RCMB.2013-0282OC

1 reference

Europe PubMed Central

PubMed publication ID

29 July 2017

PMC publication ID

1 reference

Europe PubMed Central

PubMed publication ID

29 July 2017

PubMed publication ID

1 reference

Europe PubMed Central

PubMed publication ID

29 July 2017

ResearchGate publication ID

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