(Q36615230)

15 August 2017

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title

Targeted therapy for cystic fibrosis: cystic fibrosis transmembrane conductance regulator mutation-specific pharmacologic strategies (English)

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15 August 2017

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transmembrane protein

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author name string

Ronald C Rubenstein

series ordinal

1

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15 August 2017

1 January 2006

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Molecular Diagnosis and Therapy

15 August 2017

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15 August 2017

10

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15 August 2017

5

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15 August 2017

293-301

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https://scigraph.springernature.com/pub.10.1007/bf03256204

15 August 2017

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cites work

CFTR regulates outwardly rectifying chloride channels through an autocrine mechanism involving ATP

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

Cystic fibrosis transmembrane conductance regulator facilitates ATP release by stimulating a separate ATP release channel for autocrine control of cell volume regulation

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

Aberrant CFTR-dependent HCO3- transport in mutations associated with cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

Cystic fibrosis transmembrane conductance regulator-dependent up-regulation of Kir1.1 (ROMK) renal K+ channels by the epithelial sodium channel

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

CFTR is required for PKA-regulated ATP sensitivity of Kir1.1 potassium channels in mouse kidney

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

Cl- transport by cystic fibrosis transmembrane conductance regulator (CFTR) contributes to the inhibition of epithelial Na+ channels (ENaCs) in Xenopus oocytes co-expressing CFTR and ENaC.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

Regulation of epithelial sodium channels by the cystic fibrosis transmembrane conductance regulator.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

Epithelial sodium channels regulate cystic fibrosis transmembrane conductance regulator chloride channels in Xenopus oocytes.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

Genistein restores functional interactions between Delta F508-CFTR and ENaC in Xenopus oocytes

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

Activation of the epithelial Na+ channel (ENaC) requires CFTR Cl- channel function

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

Regulation of murine airway surface liquid volume by CFTR and Ca2+-activated Cl- conductances

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

Polarized signaling via purinoceptors in normal and cystic fibrosis airway epithelia

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

Evidence for periciliary liquid layer depletion, not abnormal ion composition, in the pathogenesis of cystic fibrosis airways disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

Effects of reduced mucus oxygen concentration in airway Pseudomonas infections of cystic fibrosis patients

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

The CF salt controversy: in vivo observations and therapeutic approaches.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

Cystic fibrosis transmembrane conductance regulator inhibits epithelial Na+ channels carrying Liddle's syndrome mutations.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

Inhibition of epithelial Na+ currents by intracellular domains of the cystic fibrosis transmembrane conductance regulator.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

Cystic fibrosis transmembrane conductance regulator differentially regulates human and mouse epithelial sodium channels in Xenopus oocytes

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

Non-specific activation of the epithelial sodium channel by the CFTR chloride channel

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

CFTR fails to inhibit the epithelial sodium channel ENaC expressed in Xenopus laevis oocytes.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

Cl- interference with the epithelial Na+ channel ENaC

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

Normal and cystic fibrosis airway surface liquid homeostasis. The effects of phasic shear stress and viral infections

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

Mechanisms of the inhibition of epithelial Na(+) channels by CFTR and purinergic stimulation.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

Local regulation of cystic fibrosis transmembrane regulator and epithelial sodium channel in airway epithelium.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

Increased airway epithelial Na+ absorption produces cystic fibrosis-like lung disease in mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

Abnormalities of nasal potential difference measurement in Liddle's syndrome

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

Novel pharmacologic therapies for cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

Are p.I148T, p.R74W and p.D1270N cystic fibrosis causing mutations?

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

Severe deficiency of cystic fibrosis transmembrane conductance regulator messenger RNA carrying nonsense mutations R553X and W1316X in respiratory epithelial cells of patients with cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

In vivo nasal potential difference: techniques and protocols for assessing efficacy of gene transfer in cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

Organic solutes rescue the functional defect in delta F508 cystic fibrosis transmembrane conductance regulator.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

Activation of endogenous deltaF508 cystic fibrosis transmembrane conductance regulator by phosphodiesterase inhibition

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

Stimulation by alkylxanthines of chloride efflux in CFPAC-1 cells does not involve A1 adenosine receptors

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

S-nitrosoglutathione increases cystic fibrosis transmembrane regulator maturation.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

S-Nitrosoglutathione induces functional DeltaF508-CFTR in airway epithelial cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

Calcium-pump inhibitors induce functional surface expression of Delta F508-CFTR protein in cystic fibrosis epithelial cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

Curcumin, a major constituent of turmeric, corrects cystic fibrosis defects

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

In vivo activation of the cystic fibrosis transmembrane conductance regulator mutant deltaF508 in murine nasal epithelium

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

Evidence of CFTR function in cystic fibrosis after systemic administration of 4-phenylbutyrate

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

Phenylacetylglutamine May Replace Urea as a Vehicle for Waste Nitrogen Excretion

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

Evidence against the rescue of defective DeltaF508-CFTR cellular processing by curcumin in cell culture and mouse models.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

SERCA pump inhibitors do not correct biosynthetic arrest of deltaF508 CFTR in cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

Maintaining low Ca2+ level in the endoplasmic reticulum restores abnormal endogenous F508del-CFTR trafficking in airway epithelial cells.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

Rescue of DeltaF508-CFTR (cystic fibrosis transmembrane conductance regulator) by curcumin: involvement of the keratin 18 network

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

Curcumin stimulates cystic fibrosis transmembrane conductance regulator Cl- channel activity.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

Nanomolar affinity small molecule correctors of defective Delta F508-CFTR chloride channel gating.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

Rescue of DeltaF508-CFTR trafficking and gating in human cystic fibrosis airway primary cultures by small molecules

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

Cell-based assays and instrumentation for screening ion-channel targets

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

Cystic fibrosis transmembrane conductance regulator mutations that disrupt nucleotide binding

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

Molecular mechanisms of CFTR chloride channel dysfunction in cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

The effect of isolated soy protein on plasma biomarkers in elderly men with elevated serum prostate specific antigen

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

CFTR activation in human bronchial epithelial cells by novel benzoflavone and benzimidazolone compounds

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

High-affinity activators of cystic fibrosis transmembrane conductance regulator (CFTR) chloride conductance identified by high-throughput screening

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

Novel CFTR chloride channel activators identified by screening of combinatorial libraries based on flavone and benzoquinolizinium lead compounds

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

Mutations in CFTR associated with mild-disease-form Cl- channels with altered pore properties.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

A mutation in the cystic fibrosis transmembrane conductance regulator generates a novel internalization sequence and enhances endocytic rates

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

The short apical membrane half-life of rescued {Delta}F508-cystic fibrosis transmembrane conductance regulator (CFTR) results from accelerated endocytosis of {Delta}F508-CFTR in polarized human airway epithelial cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

An intrinsic adenylate kinase activity regulates gating of the ABC transporter CFTR.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

Modulation of deltaF508 cystic fibrosis transmembrane regulator trafficking and function with 4-phenylbutyrate and flavonoids

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

Wild type but not deltaF508 CFTR inhibits Na+ conductance when coexpressed in Xenopus oocytes.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

Genistein improves regulatory interactions between G551D-cystic fibrosis transmembrane conductance regulator and the epithelial sodium channel in Xenopus oocytes

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

Use of protein repair therapy in the treatment of cystic fibrosis.

21 January 2018

2 references

https://api.crossref.org/works/10.1007%2FBF03256204

21 January 2018

12 December 2020

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

1 reference

https://api.crossref.org/works/10.1007%2FBF03256204

Cell-based assay for high-throughput quantitative screening of CFTR chloride transport agonists

21 January 2018

1 reference

12 December 2020

Defective function of the cystic fibrosis-causing missense mutation G551D is recovered by genistein

1 reference

12 December 2020

Sodium 4-phenylbutyrate downregulates Hsc70: implications for intracellular trafficking of DeltaF508-CFTR

1 reference

12 December 2020

Association of a nonsense mutation (W1282X), the most common mutation in the Ashkenazi Jewish cystic fibrosis patients in Israel, with presentation of severe disease

1 reference

12 December 2020

Genotype-phenotype correlation and frequency of the 3199del6 cystic fibrosis mutation among I148T carriers: results from a collaborative study

1 reference

12 December 2020

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

1 reference

12 December 2020

Sodium 4-phenylbutyrate downregulates HSC70 expression by facilitating mRNA degradation

1 reference

12 December 2020

UTP inhibits Na+ absorption in wild-type and DeltaF508 CFTR-expressing human bronchial epithelia

1 reference

12 December 2020

A pilot clinical trial of oral sodium 4-phenylbutyrate (Buphenyl) in deltaF508-homozygous cystic fibrosis patients: partial restoration of nasal epithelial CFTR function

1 reference

12 December 2020

Abnormal regulatory interactions of I148T-CFTR and the epithelial Na+ channel in Xenopus oocytes.

1 reference

12 December 2020

Activation of DeltaF508 CFTR in an epithelial monolayer.

1 reference

12 December 2020

Correction of delF508-CFTR activity with benzo(c)quinolizinium compounds through facilitation of its processing in cystic fibrosis airway cells

1 reference

12 December 2020

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

1 reference

12 December 2020

Difference in frequencies of the cystic fibrosis alleles, delta F508 and W1282X, between carriers and patients.

1 reference

12 December 2020

The cystic fibrosis transmembrane conductance regulator is a dual ATP and chloride channel

1 reference

12 December 2020

Identification of a splice site mutation (2789 +5 G > A) associated with small amounts of normal CFTR mRNA and mild cystic fibrosis

1 reference

12 December 2020

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

1 reference

12 December 2020

Genistein potentiates wild-type and delta F508-CFTR channel activity

1 reference

12 December 2020

Aminoglycoside antibiotics restore CFTR function by overcoming premature stop mutations

1 reference

12 December 2020

The delta F508 mutation decreases the stability of cystic fibrosis transmembrane conductance regulator in the plasma membrane. Determination of functional half-lives on transfected cells

1 reference

12 December 2020

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

1 reference

12 December 2020

A novel mutation in the cystic fibrosis gene in patients with pulmonary disease but normal sweat chloride concentrations

1 reference

12 December 2020

Partial restoration of defective chloride conductance in DeltaF508 CF mice by trimethylamine oxide

1 reference

12 December 2020

The I148T CFTR allele occurs on multiple haplotypes: a complex allele is associated with cystic fibrosis

1 reference

12 December 2020

Induction of HSP70 promotes DeltaF508 CFTR trafficking

1 reference

12 December 2020

Purinergic inhibition of the epithelial Na+ transport via hydrolysis of PIP2.

1 reference

12 December 2020

Glutathione permeability of CFTR.

1 reference

12 December 2020

Partial restoration of cAMP-stimulated CFTR chloride channel activity in DeltaF508 cells by deoxyspergualin

1 reference

12 December 2020

Butyrate increases apical membrane CFTR but reduces chloride secretion in MDCK cells

1 reference

12 December 2020

Increased functional cell surface expression of CFTR and DeltaF508-CFTR by the anthracycline doxorubicin

1 reference

12 December 2020

The cytosolic termini of the beta- and gamma-ENaC subunits are involved in the functional interactions between cystic fibrosis transmembrane conductance regulator and epithelial sodium channel.

1 reference

12 December 2020

Identifiers

DOI

10.1007/BF03256204

1 reference

15 August 2017

1 reference

PubMed ID

17022692