Suppression of CFTR premature termination codons and rescue of CFTR protein and function by the synthetic aminoglycoside NB54 (Q35482151)

8 August 2017

title

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

1 reference

8 August 2017

Steven M Rowe

1

1 reference

8 August 2017

Peter Sloane

2

1 reference

8 August 2017

Li Ping Tang

3

1 reference

8 August 2017

Kyle Backer

4

1 reference

8 August 2017

Marina Mazur

5

1 reference

8 August 2017

Jessica Buckley-Lanier

6

1 reference

8 August 2017

Igor Nudelman

7

1 reference

8 August 2017

Valery Belakhov

8

1 reference

8 August 2017

Zsuzsa Bebok

9

1 reference

8 August 2017

Erik Schwiebert

10

1 reference

8 August 2017

Timor Baasov

11

1 reference

8 August 2017

David M Bedwell

12

1 reference

8 August 2017

publication date

22 July 2011

1 reference

Journal of Molecular Medicine

8 August 2017

published in

1 reference

8 August 2017

volume

89

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8 August 2017

issue

11

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8 August 2017

page(s)

1149-1161

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DeltaF508 CFTR processing correction and activity in polarized airway and non-airway cell monolayers

8 August 2017

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

12 July 2018

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12 July 2018

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12 July 2018

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12 July 2018

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12 July 2018

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A(2) adenosine receptors regulate CFTR through PKA and PLA(2).

12 July 2018

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

12 July 2018

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

12 July 2018

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Aminoglycoside antibiotics mediate context-dependent suppression of termination codons in a mammalian translation system

12 July 2018

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12 July 2018

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12 July 2018

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Cystic fibrosis transmembrane conductance regulator (CFTR) nucleotide-binding domain 1 (NBD-1) and CFTR truncated within NBD-1 target to the epithelial plasma membrane and increase anion permeability

12 July 2018

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

12 July 2018

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12 July 2018

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The efficiency of translation termination is determined by a synergistic interplay between upstream and downstream sequences in Saccharomyces cerevisiae.

12 July 2018

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12 July 2018

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12 July 2018

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12 July 2018

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Normal function of the cystic fibrosis conductance regulator protein can be associated with homozygous (Delta)F508 mutation

26 September 2018

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

26 September 2018

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12 December 2020

Correlation between genotype and phenotype in patients with cystic fibrosis

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12 December 2020

Aminoglycoside antibiotics restore CFTR function by overcoming premature stop mutations

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12 December 2020

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

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12 December 2020

Redesign of aminoglycosides for treatment of human genetic diseases caused by premature stop mutations

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12 December 2020

10.1007/S00109-011-0787-6

Identifiers

DOI

1 reference

8 August 2017

1 reference

8 August 2017

1 reference