Pharmacological treatment of the ion transport defect in cystic fibrosis (Q34104575)

31 July 2017

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title

Pharmacological treatment of the ion transport defect in cystic fibrosis (English)

1 reference

31 July 2017

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1 reference

Roomans GM

1

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PubMed ID

11116277

31 July 2017

language of work or name

English

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publication date

1 January 2001

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Expert Opinion on Investigational Drugs

31 July 2017

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31 July 2017

10

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31 July 2017

1

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31 July 2017

1-19

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Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA

31 July 2017

1 reference

https://api.crossref.org/works/10.1517%2F13543784.10.1.1

Cystic fibrosis: current survival and population estimates to the year 2000.

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F13543784.10.1.1

Cystic fibrosis: molecular biology and therapeutic implications

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F13543784.10.1.1

Pyrophosphate stimulates wild-type and mutant cystic fibrosis transmembrane conductance regulator Cl- channels

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F13543784.10.1.1

Cystic fibrosis as a disease of misprocessing of the cystic fibrosis transmembrane conductance regulator glycoprotein

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F13543784.10.1.1

Defects in processing and trafficking of the cystic fibrosis transmembrane conductance regulator

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F13543784.10.1.1

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/10.1517%2F13543784.10.1.1

Perturbation of Hsp90 interaction with nascent CFTR prevents its maturation and accelerates its degradation by the proteasome

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F13543784.10.1.1

The Hdj-2/Hsc70 chaperone pair facilitates early steps in CFTR biogenesis

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F13543784.10.1.1

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/10.1517%2F13543784.10.1.1

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F13543784.10.1.1

Degradation of CFTR by the ubiquitin-proteasome pathway

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F13543784.10.1.1

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F13543784.10.1.1

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/10.1517%2F13543784.10.1.1

Molecular mechanisms of CFTR chloride channel dysfunction in cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F13543784.10.1.1

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F13543784.10.1.1

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/10.1517%2F13543784.10.1.1

Osmotic water permeabilities of cultured, well-differentiated normal and cystic fibrosis airway epithelia

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F13543784.10.1.1

Cystic fibrosis airway epithelia fail to kill bacteria because of abnormal airway surface fluid.

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F13543784.10.1.1

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F13543784.10.1.1

Influence of molecular and chemical chaperones on protein folding

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F13543784.10.1.1

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F13543784.10.1.1

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/10.1517%2F13543784.10.1.1

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/10.1517%2F13543784.10.1.1

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/10.1517%2F13543784.10.1.1

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/10.1517%2F13543784.10.1.1

Pharmacologic restoration of delta F508 CFTR-mediated chloride current.

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F13543784.10.1.1

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/10.1517%2F13543784.10.1.1

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/10.1517%2F13543784.10.1.1

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/10.1517%2F13543784.10.1.1

Direct activation of cystic fibrosis transmembrane conductance regulator channels by 8-cyclopentyl-1,3-dipropylxanthine (CPX) and 1,3-diallyl-8-cyclohexylxanthine (DAX).

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F13543784.10.1.1

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/10.1517%2F13543784.10.1.1

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F13543784.10.1.1

Effects of isoflavone compounds on the activation of phospholipase C

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F13543784.10.1.1

Genistein, an inhibitor of protein tyrosine kinase, is also a competitive antagonist for P1-purinergic (adenosine) receptor in FRTL-5 thyroid cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F13543784.10.1.1

Interactions of flavonoids and other phytochemicals with adenosine receptors

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F13543784.10.1.1

Soy intake and cancer risk: a review of the in vitro and in vivo data

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F13543784.10.1.1

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F13543784.10.1.1

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/10.1517%2F13543784.10.1.1

Activation of cystic fibrosis transmembrane conductance regulator in rat epididymal epithelium by genistein

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F13543784.10.1.1

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/10.1517%2F13543784.10.1.1

Phosphatase inhibitors activate normal and defective CFTR chloride channels

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F13543784.10.1.1

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/10.1517%2F13543784.10.1.1

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F13543784.10.1.1

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F13543784.10.1.1

Selective activation of Ca(2+)-dependent K+ channels by novel benzimidazolone.

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F13543784.10.1.1

Psoriasis: current perspectives with an emphasis on treatment

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F13543784.10.1.1

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/10.1517%2F13543784.10.1.1

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F13543784.10.1.1

The actin filament disrupter cytochalasin D activates the recombinant cystic fibrosis transmembrane conductance regulator Cl- channel in mouse 3T3 fibroblasts

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F13543784.10.1.1

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F13543784.10.1.1

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/10.1517%2F13543784.10.1.1

Characterization of immortal cystic fibrosis tracheobronchial gland epithelial cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F13543784.10.1.1

Epithelial P2X purinergic receptor channel expression and function

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F13543784.10.1.1

Regulation of intracellular calcium by extracellular nucleotides in pig tracheal submucosal gland cells.

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F13543784.10.1.1

Effect of luminal nucleotides on Cl- secretion and Na+ absorption in distal bronchi.

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F13543784.10.1.1

Adenosine triphosphate: established and potential clinical applications

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F13543784.10.1.1

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/10.1517%2F13543784.10.1.1

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F13543784.10.1.1

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F13543784.10.1.1

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F13543784.10.1.1

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F13543784.10.1.1

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F13543784.10.1.1

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F13543784.10.1.1

Identification of transport abnormalities in duodenal mucosa and duodenal enterocytes from patients with cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F13543784.10.1.1

Post-translational disruption of the delta F508 cystic fibrosis transmembrane conductance regulator (CFTR)-molecular chaperone complex with geldanamycin stabilizes delta F508 CFTR in the rabbit reticulocyte lysate.

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F13543784.10.1.1

Retrograde protein translocation: ERADication of secretory proteins in health and disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F13543784.10.1.1

21 January 2018

Identifiers

DOI

10.1517/13543784.10.1.1

1 reference

31 July 2017

1 reference

PubMed ID

11116277