In vitro pharmacologic restoration of CFTR-mediated chloride transport with sodium 4-phenylbutyrate in cystic fibrosis epithelial cells containing delta F508-CFTR. (Q37374706)

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English	In vitro pharmacologic restoration of CFTR-mediated chloride transport with sodium 4-phenylbutyrate in cystic fibrosis epithelial cells containing delta F508-CFTR.	scientific article

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scholarly article

1 reference

Europe PubMed Central

PMC publication ID

20 August 2017

In vitro pharmacologic restoration of CFTR-mediated chloride transport with sodium 4-phenylbutyrate in cystic fibrosis epithelial cells containing delta F508-CFTR. (English)

1 reference

Europe PubMed Central

PMC publication ID

20 August 2017

cystic fibrosis

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1 reference

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author name string

R C Rubenstein

1

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Europe PubMed Central

PMC publication ID

20 August 2017

M E Egan

2

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Europe PubMed Central

PMC publication ID

20 August 2017

P L Zeitlin

3

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Europe PubMed Central

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20 August 2017

language of work or name

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publication date

1 November 1997

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Europe PubMed Central

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20 August 2017

Journal of Clinical Investigation

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Europe PubMed Central

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20 August 2017

100

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Europe PubMed Central

PMC publication ID

20 August 2017

10

1 reference

Europe PubMed Central

PMC publication ID

20 August 2017

2457-2465

1 reference

Europe PubMed Central

PMC publication ID

20 August 2017

Reactivation of silenced, virally transduced genes by inhibitors of histone deacetylase

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=508446

6 September 2017

Alteration of the cystic fibrosis transmembrane conductance regulator folding pathway

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=508446

6 September 2017

Functional activation of the cystic fibrosis trafficking mutant delta F508-CFTR by overexpression

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=508446

6 September 2017

The common variant of cystic fibrosis transmembrane conductance regulator is recognized by hsp70 and degraded in a pre-Golgi nonlysosomal compartment

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=508446

6 September 2017

Degradation of CFTR by the ubiquitin-proteasome pathway

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=508446

6 September 2017

Participation of the endoplasmic reticulum chaperone calnexin (p88, IP90) in the biogenesis of the cystic fibrosis transmembrane conductance regulator

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=508446

6 September 2017

Phenylacetylglutamine May Replace Urea as a Vehicle for Waste Nitrogen Excretion

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=508446

6 September 2017

Expression of the cystic fibrosis transmembrane conductance regulator gene in the respiratory tract of normal individuals and individuals with cystic fibrosis

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=508446

6 September 2017

Chemical chaperones correct the mutant phenotype of the delta F508 cystic fibrosis transmembrane conductance regulator protein

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=508446

14 September 2017

Effects of the ?F508 mutation on the structure, function, and folding of the first nucleotide-binding domain of CFTR

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=508446

14 September 2017

Down-regulation of cystic fibrosis gene mRNA transcript levels and induction of the cystic fibrosis chloride secretory phenotype in epithelial cells by phorbol ester

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=508446

28 September 2017

Differential expression of ORCC and CFTR induced by low temperature in CF airway epithelial cells

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=508446

28 September 2017

Long-term treatment of girls with ornithine transcarbamylase deficiency

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=508446

26 June 2018

Mutant (delta F508) cystic fibrosis transmembrane conductance regulator Cl- channel is functional when retained in endoplasmic reticulum of mammalian cells.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=508446

26 June 2018

The cystic fibrosis mutation (delta F508) does not influence the chloride channel activity of CFTR.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=508446

26 June 2018

A Cystic Fibrosis Bronchial Epithelial Cell Line: Immortalization by Adeno-12-SV40 Infection

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=508446

26 June 2018

Cl- channel activity in Xenopus oocytes expressing the cystic fibrosis gene

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=508446

26 June 2018

Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature-sensitive

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=508446

26 June 2018

Defective regulation of outwardly rectifying Cl- channels by protein kinase A corrected by insertion of CFTR

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=508446

26 June 2018

cAMP-stimulated ion currents in Xenopus oocytes expressing CFTR cRNA

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=508446

26 June 2018

Both the wild type and a functional isoform of CFTR are expressed in kidney

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=508446

2 September 2018

Glycerol reverses the misfolding phenotype of the most common cystic fibrosis mutation.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=508446

2 September 2018

Cystic Fibrosis Gene and Protein Expression during Fetal Lung Development

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=508446

2 September 2018

Multiple proteolytic systems, including the proteasome, contribute to CFTR processing.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=508446

2 September 2018

Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=508446

2 September 2018

Altered chloride ion channel kinetics associated with the ΔF508 cystic fibrosis mutation

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=508446

3 December 2018

Phenylbutyrate induces apoptosis in human prostate cancer and is more potent than phenylacetate

1 reference

https://pubmed.ncbi.nlm.nih.gov/9366560

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

The cystic fibrosis transmembrane conductance regulator. Effects of the most common cystic fibrosis-causing mutation on the secondary structure and stability of a synthetic peptide

1 reference

https://pubmed.ncbi.nlm.nih.gov/9366560

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Increased fetal hemoglobin in patients receiving sodium 4-phenylbutyrate

1 reference

https://pubmed.ncbi.nlm.nih.gov/9366560

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

A Short-Term Trial of Butyrate to Stimulate Fetal-Globin-Gene Expression in the β-Globin Disorders

1 reference

https://pubmed.ncbi.nlm.nih.gov/9366560

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

CFTR nonsense mutations G542X and W1282X associated with severe reduction of CFTR mRNA in nasal epithelial cells

1 reference

https://pubmed.ncbi.nlm.nih.gov/9366560

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Oral sodium phenylbutyrate therapy in homozygous beta thalassemia: a clinical trial

1 reference

https://pubmed.ncbi.nlm.nih.gov/9366560

12 December 2020

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Aminoglycoside antibiotics restore CFTR function by overcoming premature stop mutations

1 reference

https://pubmed.ncbi.nlm.nih.gov/9366560

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Nonstop treatment of cystic fibrosis

1 reference

https://pubmed.ncbi.nlm.nih.gov/9366560

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Identifiers

10.1172/JCI119788

1 reference

Europe PubMed Central

PMC publication ID

20 August 2017

PMC publication ID

1 reference

Europe PubMed Central

PMC publication ID

20 August 2017

PubMed publication ID

1 reference

Europe PubMed Central

PMC publication ID

20 August 2017

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