Pharmacological approaches to correcting the ion transport defect in cystic fibrosis (Q35627639)

9 August 2017

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title

Pharmacological approaches to correcting the ion transport defect in cystic fibrosis (English)

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9 August 2017

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Godfried M Roomans

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9 August 2017

publication date

1 January 2003

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Treatments in Respiratory Medicine

9 August 2017

published in

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9 August 2017

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9 August 2017

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9 August 2017

page(s)

413-431

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Incidence, population, and survival of cystic fibrosis in the UK, 1968-95. UK Cystic Fibrosis Survey Management Committee

9 August 2017

cites work

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https://api.crossref.org/works/10.1007%2FBF03256668

Identification of the cystic fibrosis gene: chromosome walking and jumping

21 January 2018

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Molecular insights into the physiology of the 'thin film' of airway surface liquid

21 January 2018

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https://api.crossref.org/works/10.1007%2FBF03256668

Loss of CFTR chloride channels alters salt absorption by cystic fibrosis airway epithelia in vitro.

21 January 2018

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https://api.crossref.org/works/10.1007%2FBF03256668

Diffuse panbronchiolitis--pathophysiology and treatment mechanisms

21 January 2018

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https://api.crossref.org/works/10.1007%2FBF03256668

Small airways diseases: detection and insights with computed tomography

21 January 2018

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https://api.crossref.org/works/10.1007%2FBF03256668

Oral administration of specific yolk antibodies (IgY) may prevent Pseudomonas aeruginosa infections in patients with cystic fibrosis: A phase I feasibility study

21 January 2018

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https://api.crossref.org/works/10.1007%2FBF03256668

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

21 January 2018

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https://api.crossref.org/works/10.1007%2FBF03256668

Retroviral approaches to gene therapy of cystic fibrosis

21 January 2018

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https://api.crossref.org/works/10.1007%2FBF03256668

Pharmacological treatment of the biochemical defect in cystic fibrosis airways

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

Pharmacological treatment of the ion transport defect in cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

Thiazolidinone CFTR inhibitor identified by high-throughput screening blocks cholera toxin-induced intestinal fluid secretion.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

Interleukin 2 modulates ion secretion and cell proliferation in cultured human small intestinal enterocytes

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

Upregulation of cystic fibrosis transmembrane conductance regulator expression by oestrogen and Bak Foong Pill in mouse uteri

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

Effect of phenol red and steroid hormones on cystic fibrosis transmembrane conductance regulator in mouse endometrial epithelial cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

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%2FBF03256668

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

Degradation of CFTR by the ubiquitin-proteasome pathway

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

Cystic fibrosis F508del patients have apically localized CFTR in a reduced number of airway cells.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

Maturation and function of cystic fibrosis transmembrane conductance regulator variants bearing mutations in putative nucleotide-binding domains 1 and 2

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

Chemical chaperones correct the mutant phenotype of the ΔF508 cystic fibrosis transmembrane conductance regulator protein

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

Influence of molecular and chemical chaperones on protein folding

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

Surface expression of the cystic fibrosis transmembrane conductance regulator mutant DeltaF508 is markedly upregulated by combination treatment with sodium butyrate and low temperature

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

Bicarbonate and chloride secretion in Calu-3 human airway epithelial cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

Anion selectivity of apical membrane conductance of Calu 3 human airway epithelium

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

Control of dynamic CFTR selectivity by glutamate and ATP in epithelial cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

Compartmentalized autocrine signaling to cystic fibrosis transmembrane conductance regulator at the apical membrane of airway epithelial cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

The cystic fibrosis transmembrane conductance regulator (CFTR) inhibits ENaC through an increase in the intracellular Cl- concentration

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

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%2FBF03256668

Interaction between calcium-activated chloride channels and the cystic fibrosis transmembrane conductance regulator

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

Aquaporin 3 cloned from Xenopus laevis is regulated by the cystic fibrosis transmembrane conductance regulator

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

Defective acidification of intracellular organelles in cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

Synthesis and characterization of improved chloride-sensitive fluorescent indicators for biological applications

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

Activation of deltaF508 CFTR in a cystic fibrosis respiratory epithelial cell line by 4-phenylbutyrate, genistein and CPX.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

Assessment of CFTR chloride channel openers in intact normal and cystic fibrosis murine epithelia

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

Food restriction prevents age-related polyuria by vasopressin-dependent recruitment of aquaporin-2.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

Green fluorescent protein-based halide indicators with improved chloride and iodide affinities

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

Mechanism and cellular applications of a green fluorescent protein-based halide sensor

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

Assessment of chloride secretion in human nasal epithelial cells by X-ray microanalysis

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

Cystic fibrosis transmembrane conductance regulator (CFTR) activity in nasal epithelial cells from cystic fibrosis patients with severe genotypes

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

Use of nasal potential difference and sweat chloride as outcome measures in multicenter clinical trials in subjects with cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

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%2FBF03256668

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%2FBF03256668

Direct block of the cystic fibrosis transmembrane conductance regulator Cl(-) channel by butyrate and phenylbutyrate

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

Inhibitory effects of phenylbutyrate on the proliferation, morphology, migration and invasiveness of malignant glioma cells.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

Pharmacologic restoration of delta F508 CFTR-mediated chloride current.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

Endogenous nitrogen oxides and bronchodilator S-nitrosothiols in human airways

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

S-nitrosothiols signal the ventilatory response to hypoxia

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

Nitric oxide and thiol groups

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

Bronchodilator S-nitrosothiol deficiency in asthmatic respiratory failure

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

S-nitrosoglutathione increases cystic fibrosis transmembrane regulator maturation.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

Acute effects of aerosolized S-nitrosoglutathione in cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

Structural basis for specificity and potency of xanthine derivatives as activators of the CFTR chloride channel.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

Molecular basis of defective anion transport in L cells expressing recombinant forms of CFTR

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

A1 adenosine-receptor antagonists activate chloride efflux from cystic fibrosis cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

A1 receptor antagonist 8-cyclopentyl-1,3-dipropylxanthine selectively activates chloride efflux from human epithelial and mouse fibroblast cell lines expressing the cystic fibrosis transmembrane regulator delta F508 mutation

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

No evidence for direct activation of the cystic fibrosis transmembrane conductance regulator by 8-cyclopentyl-1,3-dipropylxanthine

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

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%2FBF03256668

Actions of adenosine A1 and A2 receptor antagonists on CFTR antibody-inhibited beta-adrenergic mucin secretion response

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

A phase I randomized, multicenter trial of CPX in adult subjects with mild cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

Use and specificity of genistein as inhibitor of protein-tyrosine kinases

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

Effects of isoflavone compounds on the activation of phospholipase C

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

Stimulation of secretion by the T84 colonic epithelial cell line with dietary flavonols

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

Modulation of Cl- secretion in rat distal colon by genistein, a protein tyrosine kinase inhibitor

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

Direct action of genistein on CFTR.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

Actions of genistein on cystic fibrosis transmembrane conductance regulator channel gating. Evidence for two binding sites with opposite effects

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

Inhibition of ATPase, GTPase and adenylate kinase activities of the second nucleotide-binding fold of the cystic fibrosis transmembrane conductance regulator by genistein.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

Effect of genistein on native epithelial tissue from normal individuals and CF patients and on ion channels expressed in Xenopus oocytes

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

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%2FBF03256668

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%2FBF03256668

Genistein modifies the activation kinetics and magnitude of phosphorylated wild-type and G551D-CFTR chloride currents.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

Vasoactive intestinal peptide, forskolin, and genistein increase apical CFTR trafficking in the rectal gland of the spiny dogfish, Squalus acanthias. Acute regulation of CFTR trafficking in an intact epithelium

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

Two mechanisms of genistein inhibition of cystic fibrosis transmembrane conductance regulator Cl- channels expressed in murine cell line

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

Activation of CFTR by genistein in human airway epithelial cell lines

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

Phosphatase inhibitors activate normal and defective CFTR chloride channels

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

cAMP- and Ca2+-independent activation of cystic fibrosis transmembrane conductance regulator channels by phenylimidazothiazole drugs

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

Regulation of CFTR by protein phosphatase 2B and protein kinase C.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

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%2FBF03256668

EBIO, an agent causing maintained epithelial chloride secretion by co-ordinate actions at both apical and basolateral membranes

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

Development of substituted Benzo[c]quinolizinium compounds as novel activators of the cystic fibrosis chloride channel.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

Benzo(c)quinolizinium drugs inhibit degradation of Delta F508-CFTR cytoplasmic domain.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

Phenanthrolines--a new class of CFTR chloride channel openers

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

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%2FBF03256668

Chloride secretory response of cystic fibrosis human airway epithelia. Preservation of calcium but not protein kinase C- and A-dependent mechanisms

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

Characterization of immortal cystic fibrosis tracheobronchial gland epithelial cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

Regulation of transepithelial ion transport and intracellular calcium by extracellular ATP in human normal and cystic fibrosis airway epithelium

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

Epithelial P2X purinergic receptor channel expression and function

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

Inhaled P2Y2 receptor agonists as a treatment for patients with Cystic Fibrosis lung disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

Effect of amiloride and saline on nasal mucociliary clearance and potential difference in cystic fibrosis and normal subjects

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

Pharmacologic modulation of salt and water in the airway epithelium in cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

Physical and functional properties of airway secretions in cystic fibrosis--therapeutic approaches

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

Nebulised amiloride in respiratory exacerbations of cystic fibrosis: a randomised controlled trial

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

The effect of topical benzamil and amiloride on nasal potential difference in cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

Interest of colchicine for the treatment of cystic fibrosis patients. Preliminary report

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

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%2FBF03256668

Effects of topically delivered benzamil and amiloride on nasal potential difference in cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

Altering airway surface liquid volume: inhalation therapy with amiloride and hyperosmotic agents

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

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%2FBF03256668

Correction of CFTR malfunction and stimulation of Ca-activated Cl channels restore HCO3- secretion in cystic fibrosis bile ductular cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

Rethinking cystic fibrosis pathology: the critical role of abnormal reduced glutathione (GSH) transport caused by CFTR mutation

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

Pulmonary oxidative stress response in young children with cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

21 January 2018

Colchicine: 1998 update.

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

Inflammation and CFTR: might neutrophils be the key in cystic fibrosis?

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

ABC-ATPases, adaptable energy generators fuelling transmembrane movement of a variety of molecules in organisms from bacteria to humans

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

Analysis of ion transport perturbations caused by hu MDR 1 protein overexpression

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256668

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%2FBF03256668

21 January 2018

Identifiers

DOI

10.1007/BF03256668

1 reference

9 August 2017

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