Cystic fibrosis transmembrane regulator protein mutations: 'class' opportunity for novel drug innovation (Q36732484)

16 August 2017

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title

Cystic fibrosis transmembrane regulator protein mutations: 'class' opportunity for novel drug innovation (English)

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16 August 2017

main subject

transmembrane protein

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cystic fibrosis

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author name string

Kelvin D MacDonald

series ordinal

1

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16 August 2017

Karen R McKenzie

series ordinal

2

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16 August 2017

Pamela L Zeitlin

series ordinal

3

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16 August 2017

publication date

1 January 2007

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16 August 2017

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16 August 2017

volume

9

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16 August 2017

issue

1

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16 August 2017

page(s)

1-10

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https://scigraph.springernature.com/pub.10.2165/00148581-200709010-00001

16 August 2017

exact match

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cites work

Five Percent of Normal Cystic Fibrosis Transmembrane Conductance Regulator mRNA Ameliorates the Severity of Pulmonary Disease in Cystic Fibrosis

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Crossref

https://api.crossref.org/works/10.2165%2F00148581-200709010-00001

Distribution of ClC-2 chloride channel in rat and human epithelial tissues

21 January 2018

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Crossref

https://api.crossref.org/works/10.2165%2F00148581-200709010-00001

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

Restoration of the cystic fibrosis transmembrane conductance regulator function by splicing modulation

1 reference

12 December 2020

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

1 reference

12 December 2020

Pharmacologic therapy for stop mutations: how much CFTR activity is enough?

1 reference

12 December 2020

Protocols for in vivo measurement of the ion transport defects in cystic fibrosis nasal epithelium

1 reference

12 December 2020

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

1 reference

12 December 2020

Mild cystic fibrosis and normal or borderline sweat test in patients with the 3849 + 10 kb C-->T mutation

1 reference

12 December 2020

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

1 reference

12 December 2020

Protein kinase A regulates ATP hydrolysis and dimerization by a CFTR (cystic fibrosis transmembrane conductance regulator) 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

Selective activation of cystic fibrosis transmembrane conductance regulator Cl- and HCO3- conductances.

1 reference

12 December 2020

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

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12 December 2020

Control of epithelial Na+ conductance by the cystic fibrosis transmembrane conductance regulator

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12 December 2020

Activation of CFTR by genistein in human airway epithelial cell lines

1 reference

12 December 2020

Normal function of the cystic fibrosis conductance regulator protein can be associated with homozygous (Delta)F508 mutation

1 reference

12 December 2020

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

1 reference

12 December 2020

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

1 reference

12 December 2020

Analysis of CFTR transcripts in nasal epithelial cells and lymphoblasts of a cystic fibrosis patient with 621 + 1G-->T and 711 + 1G-->T mutations

1 reference

12 December 2020

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

1 reference

12 December 2020

Characterization of mutations located in exon 18 of the CFTR gene.

1 reference

12 December 2020

Curcuma longa (turmeric). Monograph

1 reference

12 December 2020

Increased bioelectric potential difference across respiratory epithelia in cystic fibrosis

1 reference

12 December 2020

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

1 reference

12 December 2020

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

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12 December 2020

Phosphatase inhibitors activate normal and defective CFTR chloride channels

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12 December 2020

Airway gene therapy and cystic fibrosis

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12 December 2020

CFTR is a pattern recognition molecule that extracts Pseudomonas aeruginosa LPS from the outer membrane into epithelial cells and activates NF-kappa B translocation

1 reference

12 December 2020

Aminoglycoside antibiotics restore CFTR function by overcoming premature stop mutations

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12 December 2020

The CFTR chloride channel of mammalian heart

1 reference

12 December 2020

Correction of the cystic fibrosis defect in vitro by retrovirus-mediated gene transfer

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12 December 2020

Pancreatitis in young children with cystic fibrosis

1 reference

12 December 2020

Curcumin does not stimulate cAMP-mediated chloride transport in cystic fibrosis 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

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

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

1 reference

12 December 2020

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

1 reference

12 December 2020

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

1 reference

12 December 2020

CFTR-mediated chloride permeability is regulated by type III phosphodiesterases in airway epithelial cells

1 reference

12 December 2020

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

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12 December 2020

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

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12 December 2020

The molecular basis of disease variability among cystic fibrosis patients carrying the 3849+10 kb C-->T mutation

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12 December 2020

Treatment of spinal muscular atrophy by sodium butyrate

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12 December 2020

ATPase activity of the cystic fibrosis transmembrane conductance regulator

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12 December 2020

Flavonoids stimulate Cl conductance of human airway epithelium in vitro and in vivo.

1 reference

12 December 2020

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

1 reference

12 December 2020

European Epidemiologic Registry of Cystic Fibrosis (ERCF): comparison of major disease manifestations between patients with different classes of mutations

1 reference

12 December 2020

Type I, II, III, IV, and V cystic fibrosis transmembrane conductance regulator defects and opportunities for therapy.

1 reference

12 December 2020

Identification of the cystic fibrosis gene: genetic analysis

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12 December 2020

CFTR: more than just a chloride channel

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12 December 2020

Induction of HSP70 promotes DeltaF508 CFTR trafficking

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12 December 2020

ATP hydrolysis by a CFTR domain: pharmacology and effects of G551D mutation

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12 December 2020

Drug discovery and epithelial physiology

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12 December 2020

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

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12 December 2020

Gene therapy progress and prospects: cystic fibrosis

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12 December 2020

Control of the CFTR channel's gates

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12 December 2020

Ubiquitin and the control of protein fate in the secretory and endocytic pathways

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12 December 2020

Correction of ATM gene function by aminoglycoside-induced read-through of premature termination codons

1 reference

12 December 2020

Normal gating of CFTR requires ATP binding to both nucleotide-binding domains and hydrolysis at the second nucleotide-binding domain

1 reference

12 December 2020

Isobutylmethylxanthine fails to stimulate chloride secretion in cystic fibrosis airway epithelia

1 reference

12 December 2020

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

1 reference

12 December 2020

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

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12 December 2020

10.2165/00148581-200709010-00001

Identifiers

DOI

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Dimensions Publication ID

16 August 2017

1023772478

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