DeltaF508 CFTR protein expression in tissues from patients with cystic fibrosis (Q33854446)

29 July 2017

title

DeltaF508 CFTR protein expression in tissues from patients with cystic fibrosis (English)

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29 July 2017

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N Kälin

1

1 reference

29 July 2017

A Claass

2

1 reference

29 July 2017

M Sommer

3

1 reference

29 July 2017

E Puchelle

4

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29 July 2017

B Tümmler

5

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29 July 2017

language of work or name

English

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publication date

1 May 1999

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Journal of Clinical Investigation

29 July 2017

published in

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29 July 2017

volume

103

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29 July 2017

issue

10

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29 July 2017

page(s)

1379-1389

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Targeting of CFTR protein is linked to the polarization of human pancreatic duct cells in culture

29 July 2017

cites work

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Membrane trafficking of the cystic fibrosis gene product, cystic fibrosis transmembrane conductance regulator, tagged with green fluorescent protein in madin-darby canine kidney cells

5 July 2018

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Activation of DeltaF508 CFTR in an epithelial monolayer.

5 July 2018

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Cotranslational ubiquitination of cystic fibrosis transmembrane conductance regulator in vitro

5 July 2018

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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

5 July 2018

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Mice deficient in lysosomal acid phosphatase develop lysosomal storage in the kidney and central nervous system

5 July 2018

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

5 July 2018

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In vivo activation of the cystic fibrosis transmembrane conductance regulator mutant deltaF508 in murine nasal epithelium

5 July 2018

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Host cell-specific folding and assembly of the neuronal nicotinic acetylcholine receptor alpha7 subunit

5 July 2018

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CFTR-dependent membrane insertion is linked to stimulation of the CFTR chloride conductance

5 July 2018

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

5 July 2018

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A demonstration using mouse models that successful gene therapy for cystic fibrosis requires only partial gene correction.

5 July 2018

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

5 July 2018

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Defective ATP-dependent mucin secretion by cystic fibrosis pancreatic epithelial cells

5 July 2018

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Genotypic analysis of respiratory mucous sulfation defects in cystic fibrosis.

5 July 2018

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Cyclic AMP and chloride-dependent regulation of the apical constitutive secretory pathway in colonic epithelial cells

5 July 2018

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Determinants of mild clinical symptoms in cystic fibrosis patients. Residual chloride secretion measured in rectal biopsies in relation to the genotype

5 July 2018

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ATP-stimulated electrolyte and mucin secretion in the human intestinal goblet cell line HT29-Cl.16E.

5 July 2018

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

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=408454

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

5 July 2018

1 reference

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Degradation of CFTR by the ubiquitin-proteasome pathway

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=408454

CFTR and differentiation markers expression in non-CF and delta F 508 homozygous CF nasal epithelium

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=408454

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

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=408454

Decreased expression of the CFTR protein in remodeled human nasal epithelium from non-cystic fibrosis patients

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=408454

Conformational maturation of CFTR but not its mutant counterpart (delta F508) occurs in the endoplasmic reticulum and requires ATP

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=408454

Constitutive Mucin Secretion Linked to CFTR Expression

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=408454

Subcellular localization of CFTR to endosomes in a ductal epithelium

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=408454

Heterotrimeric G proteins, vesicle trafficking, and CFTR Cl- channels

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=408454

Rapid endocytosis of the cystic fibrosis transmembrane conductance regulator chloride channel

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=408454

Participation of the endoplasmic reticulum chaperone calnexin (p88, IP90) in the biogenesis of the cystic fibrosis transmembrane conductance regulator

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=408454

Biochemical and biophysical identification of cystic fibrosis transmembrane conductance regulator chloride channels as components of endocytic clathrin-coated vesicles

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=408454

Localization of cystic fibrosis transmembrane conductance regulator mRNA in the human gastrointestinal tract by in situ hybridization

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=408454

Localization of the cystic fibrosis transmembrane conductance regulator in human bile duct epithelial cells

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=408454

An osmotic mechanism for exocytosis from dissociated chromaffin cells

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=408454

Altered intestinal chloride transport in cystic fibrosis

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=408454

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

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=408454

Rapid nonradioactive detection of the major cystic fibrosis mutation.

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=408454

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

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=408454

Localization of the cystic fibrosis transmembrane conductance regulator in pancreas

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=408454

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

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=408454

Immunological localization of cystic fibrosis candidate gene products

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=408454

Cellular differentiation regulates expression of Cl- transport and cystic fibrosis transmembrane conductance regulator mRNA in human intestinal cells.

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=408454

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

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=408454

Regulation of plasma membrane recycling by CFTR.

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=408454

Localization of cystic fibrosis transmembrane conductance regulator in chloride secretory epithelia

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=408454

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

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=408454

A novel model for the first nucleotide binding domain of the cystic fibrosis transmembrane conductance regulator.

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=408454

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

29 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=408454

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

29 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=408454

Generation and characterization of a delta F508 cystic fibrosis mouse model.

29 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=408454

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

29 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=408454

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

29 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=408454

Effect of anion transport inhibition on mucus secretion by airway submucosal glands

29 October 2018

1 reference

12 December 2020

Characterization of polyclonal and monoclonal antibodies to cystic fibrosis transmembrane conductance regulator

1 reference

12 December 2020

The delta F508 mutation decreases the stability of cystic fibrosis transmembrane conductance regulator in the plasma membrane. Determination of functional half-lives on transfected cells

1 reference

12 December 2020

Methods for the isolation of intact epithelium from the mouse intestine

1 reference

12 December 2020

Isolation of transporting plasma membrane vesicles from bovine tracheal epithelium

1 reference

12 December 2020

Higher proportion of intact exon 9 CFTR mRNA in nasal epithelium compared with vas deferens.

1 reference

12 December 2020

Tissue-specific in vivo transcription start sites of the human and murine cystic fibrosis genes

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

Limited proteolysis as a probe for arrested conformational maturation of delta F508 CFTR

1 reference

12 December 2020

A mutation in the cystic fibrosis transmembrane conductance regulator gene associated with elevated sweat chloride concentrations in the absence of cystic fibrosis

1 reference

12 December 2020

Vector-specific complementation profiles of two independent primary defects in cystic fibrosis airways

1 reference

12 December 2020

Expression of cystic fibrosis transmembrane conductance regulator corrects defective chloride channel regulation in cystic fibrosis airway epithelial cells

1 reference

12 December 2020

CFTR: Development of high- affinity antibodies and localization in sweat gland

1 reference

12 December 2020

Differential localization of the cystic fibrosis transmembrane conductance regulator in normal and cystic fibrosis airway epithelium

1 reference

12 December 2020

An antibody against a cftr-derived synthetic peptide, incorporated into living submandibular cells, inhibits beta-adrenergic stimulation of mucin secretion

1 reference

12 December 2020

Mislocalization of ΔF508 CFTR in cystic fibrosis sweat gland

1 reference

12 December 2020

Genetic basis of variable exon 9 skipping in cystic fibrosis transmembrane conductance regulator mRNA

1 reference

12 December 2020

Submucosal glands are the predominant site of CFTR expression in the human bronchus

1 reference

12 December 2020

Coupled secretion of chloride and mucus in skin of Xenopus laevis: possible role for CFTR

1 reference

12 December 2020

A unique subset of rat and human intestinal villus cells express the cystic fibrosis transmembrane conductance regulator

1 reference

12 December 2020

Cellular localization of cystic fibrosis transmembrane regulator protein in piglet and mouse intestine

1 reference

12 December 2020

Identifiers

DOI

10.1172/JCI5731

1 reference

29 July 2017

1 reference

29 July 2017

1 reference