A domain mimic increases DeltaF508 CFTR trafficking and restores cAMP-stimulated anion secretion in cystic fibrosis epithelia (Q44803815)

6 December 2017

A domain mimic increases DeltaF508 CFTR trafficking and restores cAMP-stimulated anion secretion in cystic fibrosis epithelia (English)

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6 December 2017

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Lane L Clarke

1

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6 December 2017

Lara R Gawenis

2

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6 December 2017

Tzyh-Chang Hwang

3

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6 December 2017

Nancy M Walker

4

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6 December 2017

Darren B Gruis

5

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6 December 2017

Elmer M Price

6

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6 December 2017

17 March 2004

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American Journal of Physiology - Cell Physiology

6 December 2017

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6 December 2017

287

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6 December 2017

C192-9

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6 December 2017

1

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Regulation of plasma membrane recycling by CFTR.

6 December 2017

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https://api.crossref.org/works/10.1152%2FAJPCELL.00337.2003

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00337.2003

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00337.2003

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/10.1152%2FAJPCELL.00337.2003

Cystic fibrosis transmembrane conductance regulator (CFTR) nucleotide-binding domain 1 (NBD-1) and CFTR truncated within NBD-1 target to the epithelial plasma membrane and increase anion permeability

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00337.2003

CFTR is required for cAMP inhibition of intestinal Na+ absorption in a cystic fibrosis mouse model

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00337.2003

Effects of bradykinin on Na+ and Cl- transport in human nasal epithelium

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00337.2003

Cystic fibrosis: molecular biology and therapeutic implications

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00337.2003

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00337.2003

Quantitative analysis of the packaging capacity of recombinant adeno-associated virus

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00337.2003

Long-term survival of the exon 10 insertional cystic fibrosis mutant mouse is a consequence of low level residual wild-type Cftr gene expression.

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00337.2003

A delta F508 mutation in mouse cystic fibrosis transmembrane conductance regulator results in a temperature-sensitive processing defect in vivo

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00337.2003

High conservation of sequences involved in cystic fibrosis mutations in five mammalian species

21 January 2018

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https://api.crossref.org/works/10.1152%2FAJPCELL.00337.2003

Expression and characterization of the cystic fibrosis transmembrane conductance regulator.

21 January 2018

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https://api.crossref.org/works/10.1152%2FAJPCELL.00337.2003

The nucleotide binding folds of the cystic fibrosis transmembrane conductance regulator are extracellularly accessible

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00337.2003

Delta F508-CFTR channels: kinetics, activation by forskolin, and potentiation by xanthines

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00337.2003

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/10.1152%2FAJPCELL.00337.2003

Mammalian osmolytes and S-nitrosoglutathione promote Delta F508 cystic fibrosis transmembrane conductance regulator (CFTR) protein maturation and function

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00337.2003

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00337.2003

Molecular pharmacology of the CFTR Cl- channel

21 January 2018

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https://api.crossref.org/works/10.1152%2FAJPCELL.00337.2003

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00337.2003

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00337.2003

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00337.2003

Comparison of the gating behaviour of human and murine cystic fibrosis transmembrane conductance regulator Cl- channels expressed in mammalian cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00337.2003

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/10.1152%2FAJPCELL.00337.2003

Antagonism of catecholamine receptor signaling by expression of cytoplasmic domains of the receptors

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00337.2003

Function of the R domain in the cystic fibrosis transmembrane conductance regulator chloride channel

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00337.2003

6-mer peptide selectively anneals to a pathogenic serpin conformation and blocks polymerization. Implications for the prevention of Z alpha(1)-antitrypsin-related cirrhosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00337.2003

Syntaxin 1A inhibits CFTR chloride channels by means of domain-specific protein-protein interactions

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00337.2003

Role of CFTR in airway disease.

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00337.2003

Biosynthetic and growth abnormalities are associated with high-level expression of CFTR in heterologous cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00337.2003

Rescue of dysfunctional deltaF508-CFTR chloride channel activity by IBMX.

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00337.2003

In vivo protein transduction: delivery of a biologically active protein into the mouse

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00337.2003

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00337.2003

CFTR as a cAMP-dependent regulator of sodium channels

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00337.2003

The spectrum of cystic fibrosis mutations

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00337.2003

Deletion of phenylalanine 508 causes attenuated phosphorylation-dependent activation of CFTR chloride channels

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00337.2003

Degradation of CFTR by the ubiquitin-proteasome pathway

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00337.2003

A mouse model for the delta F508 allele of cystic fibrosis.

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00337.2003

A single conductance pore for chloride ions formed by two cystic fibrosis transmembrane conductance regulator molecules

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00337.2003

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00337.2003

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00337.2003

10.1152/AJPCELL.00337.2003

21 January 2018

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6 December 2017

1 reference