(Q37694524)

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:20166764%20AND%20SRC:MED&resulttype=core&format=json

18 January 2020

review article

1 reference

title

Cystic fibrosis transmembrane conductance regulator modulators for personalized drug treatment of cystic fibrosis: progress to date (English)

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:20166764%20AND%20SRC:MED&resulttype=core&format=json

18 January 2020

main subject

cystic fibrosis

0 references

transmembrane protein

0 references

author

Frédéric Becq

series ordinal

1

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:20166764%20AND%20SRC:MED&resulttype=core&format=json

18 January 2020

author name string

Frédéric Becq

series ordinal

1

1 reference

22 August 2017

1 February 2010

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:20166764%20AND%20SRC:MED&resulttype=core&format=json

18 January 2020

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:20166764%20AND%20SRC:MED&resulttype=core&format=json

18 January 2020

volume

70

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:20166764%20AND%20SRC:MED&resulttype=core&format=json

18 January 2020

page(s)

241-259

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:20166764%20AND%20SRC:MED&resulttype=core&format=json

18 January 2020

issue

3

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:20166764%20AND%20SRC:MED&resulttype=core&format=json

https://scigraph.springernature.com/pub.10.2165/11316160-000000000-00000

18 January 2020

exact match

0 references

cites work

Prevalence of deltaF508, G551D, G542X, and R553X mutations among cystic fibrosis patients in the North of Brazil

1 reference

https://api.crossref.org/works/10.2165%2F11316160-000000000-00000

Structural analog of sildenafil identified as a novel corrector of the F508del-CFTR trafficking defect

21 January 2018

1 reference

https://api.crossref.org/works/10.2165%2F11316160-000000000-00000

Review of miglustat for clinical management in Gaucher disease type 1.

21 January 2018

1 reference

https://api.crossref.org/works/10.2165%2F11316160-000000000-00000

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

21 January 2018

1 reference

https://api.crossref.org/works/10.2165%2F11316160-000000000-00000

Phosphorylation-regulated CI− channel in CHO cells stably expressing the cystic fibrosis gene

21 January 2018

1 reference

12 December 2020

Targeting glycosylation as a therapeutic approach

1 reference

12 December 2020

Anti-inflammatory effect of miglustat in bronchial epithelial cells.

1 reference

12 December 2020

Rescue of functional delF508-CFTR channels in cystic fibrosis epithelial cells by the alpha-glucosidase inhibitor miglustat

1 reference

12 December 2020

Identification of the cystic fibrosis gene: chromosome walking and jumping

1 reference

12 December 2020

Chloride Conductance Expressed by ΔF508 and Other Mutant CFTRs In Xenopus Oocytes

1 reference

12 December 2020

Apical and basolateral ATP stimulates tracheal epithelial chloride secretion via multiple purinergic receptors

1 reference

12 December 2020

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

1 reference

12 December 2020

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

1 reference

12 December 2020

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

1 reference

12 December 2020

The role of intracellular calcium signals in inflammatory responses of polarised cystic fibrosis human airway epithelia

1 reference

12 December 2020

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

1 reference

12 December 2020

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

1 reference

12 December 2020

1 reference

12 December 2020

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

1 reference

12 December 2020

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

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

Molecular mechanisms of CFTR chloride channel dysfunction in cystic fibrosis

1 reference

12 December 2020

Cystic fibrosis: a disease of vulnerability to airway surface dehydration.

1 reference

12 December 2020

A phase I trial of intranasal Moli1901 for cystic fibrosis

1 reference

12 December 2020

Control of CFTR channel gating by phosphorylation and nucleotide hydrolysis.

1 reference

12 December 2020

Antagonists of myo-inositol 3,4,5,6-tetrakisphosphate allow repeated epithelial chloride secretion

1 reference

12 December 2020

Dynasore inhibits removal of wild-type and DeltaF508 cystic fibrosis transmembrane conductance regulator (CFTR) from the plasma membrane.

1 reference

12 December 2020

C-terminal truncations destabilize the cystic fibrosis transmembrane conductance regulator without impairing its biogenesis. A novel class of mutation

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

INO-4995 therapeutic efficacy is enhanced with repeat dosing in cystic fibrosis knockout mice and human epithelia

1 reference

12 December 2020

A cystic fibrosis respiratory epithelial cell chronically treated by miglustat acquires a non-cystic fibrosis-like phenotype

1 reference

12 December 2020

Regulation of the epithelial Na+ channel by peptidases

1 reference

12 December 2020

Growing old with cystic fibrosis - the characteristics of long-term survivors of cystic fibrosis

1 reference

12 December 2020

Aerosolized amiloride: dose effect on nasal bioelectric properties, pharmacokinetics, and effect on sputum expectoration in patients with cystic fibrosis

1 reference

12 December 2020

CFTR inhibition mimics the cystic fibrosis inflammatory profile

1 reference

12 December 2020

CFTR mutations in the Algerian population

1 reference

12 December 2020

A novel CFTR mutation found in a Chinese patient with cystic fibrosis

1 reference

12 December 2020

Phenylglycine and sulfonamide correctors of defective delta F508 and G551D cystic fibrosis transmembrane conductance regulator chloride-channel gating

1 reference

12 December 2020

Readthrough strategies for stop codons in Duchenne muscular dystrophy

1 reference

12 December 2020

Chloride channels as drug targets

1 reference

12 December 2020

Activation of G551D CFTR channel with MPB-91: regulation by ATPase activity and phosphorylation

1 reference

12 December 2020

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

1 reference

12 December 2020

Guanabenz. A review of its pharmacodynamic properties and therapeutic efficacy in hypertension

1 reference

12 December 2020

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

1 reference

12 December 2020

Cystic fibrosis in India

1 reference

12 December 2020

Na+ transport in normal and CF human bronchial epithelial cells is inhibited by BAY 39-9437.

1 reference

12 December 2020

Phosphatase inhibitors activate normal and defective CFTR chloride channels

1 reference

12 December 2020

MPB-07 Reduces the Inflammatory Response toPseudomonas aeruginosain Cystic Fibrosis Bronchial Cells

1 reference

12 December 2020

A century of (epithelial) transport physiology: from vitalism to molecular cloning

1 reference

12 December 2020

Clinical trials in cystic fibrosis

1 reference

12 December 2020

A cluster of cystic fibrosis mutations in the first nucleotide-binding fold of the cystic fibrosis conductance regulator protein

1 reference

12 December 2020

Selective inhibition of endoplasmic reticulum-associated degradation rescues DeltaF508-cystic fibrosis transmembrane regulator and suppresses interleukin-8 levels: therapeutic implications.

1 reference

12 December 2020

French multicenter randomized double-blind placebo-controlled trial on nebulized amiloride in cystic fibrosis patients. The Amiloride-AFLM Collaborative Study Group

1 reference

12 December 2020

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

1 reference

12 December 2020

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

1 reference

12 December 2020

Duramycin increases intracellular calcium in airway epithelium

1 reference

12 December 2020

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

1 reference

12 December 2020

Cystic fibrosis: a worldwide analysis of CFTR mutations--correlation with incidence data and application to screening

1 reference

12 December 2020

Synthesis, SAR, crystal structure, and biological evaluation of benzoquinoliziniums as activators of wild-type and mutant cystic fibrosis transmembrane conductance regulator channels.

1 reference

12 December 2020

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

1 reference

12 December 2020

Guanabenz, an alpha2-selective adrenergic agonist, activates Ca2+-dependent chloride currents in cystic fibrosis human airway epithelial cells

1 reference

12 December 2020

Inhalation of Moli1901 in patients with cystic fibrosis.

1 reference

12 December 2020

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

1 reference

12 December 2020

Sildenafil (Viagra) corrects DeltaF508-CFTR location in nasal epithelial cells from patients with cystic fibrosis.

1 reference

12 December 2020

Cystic fibrosis carrier frequency and estimated prevalence of the disease in Morocco.

1 reference

12 December 2020

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

1 reference

12 December 2020

On the discovery and development of CFTR chloride channel activators

1 reference

12 December 2020

Small-molecule correctors of defective DeltaF508-CFTR cellular processing identified by high-throughput screening.

1 reference

12 December 2020

Airway delivery of low-dose miglustat normalizes nasal potential difference in F508del cystic fibrosis mice

1 reference

12 December 2020

The Delta F508 mutation shortens the biochemical half-life of plasma membrane CFTR in polarized epithelial cells

1 reference

12 December 2020

Denufosol: a review of studies with inhaled P2Y(2) agonists that led to Phase 3.

1 reference

12 December 2020

Inositol polyphosphate derivative inhibits Na+ transport and improves fluid dynamics in cystic fibrosis airway epithelia

1 reference

12 December 2020

Digitoxin mimics gene therapy with CFTR and suppresses hypersecretion of IL-8 from cystic fibrosis lung epithelial cells

1 reference

12 December 2020

The estimated incidence of cystic fibrosis in Japan

1 reference

12 December 2020

Spatial and temporal distribution of cystic fibrosis and of its mutations in Brittany, France: a retrospective study from 1960.

1 reference

12 December 2020

1 reference

12 December 2020

Identification of the cystic fibrosis gene: genetic analysis

1 reference

12 December 2020

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

1 reference

12 December 2020

Degradation of CFTR by the ubiquitin-proteasome pathway

1 reference

12 December 2020

Distribution of CFTR mutations in cystic fibrosis patients of Tunisian origin: identification of two novel mutations.

1 reference

12 December 2020

Improvements in lung function of a pediatric cystic fibrosis population in a developing country.

1 reference

12 December 2020

Genotype-phenotype relationship for five CFTR mutations frequently identified in western France

1 reference

12 December 2020

Induction of HSP70 promotes DeltaF508 CFTR trafficking

1 reference

12 December 2020

3-(2-Benzyloxyphenyl)isoxazoles and isoxazolines: synthesis and evaluation as CFTR activators

1 reference

12 December 2020

Chemical conjugation of DeltaF508-CFTR corrector deoxyspergualin to transporter human serum albumin enhances its ability to rescue Cl- channel functions.

1 reference

12 December 2020

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

1 reference

12 December 2020

Preclinical evidence that sildenafil and vardenafil activate chloride transport in cystic fibrosis

1 reference

12 December 2020

Proteasome-dependent pharmacological rescue of cystic fibrosis transmembrane conductance regulator revealed by mutation of glycine 622.

1 reference

12 December 2020

Airway epithelial cell inflammatory signalling in cystic fibrosis.

1 reference

12 December 2020

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

1 reference

12 December 2020

No added benefit from nebulized amiloride in patients with cystic fibrosis

1 reference

12 December 2020

Discovery of pyrrolo[2,3-b]pyrazines derivatives as submicromolar affinity activators of wild type, G551D, and F508del cystic fibrosis transmembrane conductance regulator chloride channels

1 reference

12 December 2020

Drug discovery in academia

1 reference

12 December 2020

Pharmacological modulation of cGMP levels by phosphodiesterase 5 inhibitors as a therapeutic strategy for treatment of respiratory pathology in cystic fibrosis

1 reference

12 December 2020

Pharmacological Properties of N-(3,5-Diamino-6-chloropyrazine-2-carbonyl)-N'-4-[4-(2,3-dihydroxypropoxy)phenyl]butyl-guanidine Methanesulfonate (552-02), a Novel Epithelial Sodium Channel Blocker with Potential Clinical Efficacy for Cystic Fibrosis L

1 reference

12 December 2020

Corr4A and VRT325 do not reduce the inflammatory response to P. aeruginosa in human cystic fibrosis airway epithelial cells

1 reference

12 December 2020

Parallel improvement of sodium and chloride transport defects by miglustat (n-butyldeoxynojyrimicin) in cystic fibrosis epithelial cells.

1 reference

12 December 2020

Calcium homeostasis is abnormal in cystic fibrosis airway epithelial cells but is normalized after rescue of F508del-CFTR.

1 reference

12 December 2020

Effectiveness of PTC124 treatment of cystic fibrosis caused by nonsense mutations: a prospective phase II trial

1 reference

12 December 2020

Isobutylmethylxanthine fails to stimulate chloride secretion in cystic fibrosis airway epithelia

1 reference

12 December 2020

Lung transplantation in cystic fibrosis

1 reference

12 December 2020

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

1 reference

12 December 2020

Consensus on the use and interpretation of cystic fibrosis mutation analysis in clinical practice

1 reference

12 December 2020