Processing and function of CFTR-DeltaF508 are species-dependent (Q35991100)

11 August 2017

Processing and function of CFTR-DeltaF508 are species-dependent (English)

1 reference

11 August 2017

Christoph O Randak

4

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Lynda S Ostedgaard

object named as

Lynda S Ostedgaard

1

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author name string

Christopher S Rogers

2

1 reference

11 August 2017

Qian Dong

3

1 reference

11 August 2017

Daniel W Vermeer

5

1 reference

11 August 2017

Tatiana Rokhlina

6

1 reference

11 August 2017

Philip H Karp

7

1 reference

11 August 2017

Michael J Welsh

8

1 reference

11 August 2017

language of work or name

English

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publication date

14 September 2007

1 reference

Proceedings of the National Academy of Sciences of the United States of America

11 August 2017

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11 August 2017

104

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11 August 2017

39

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11 August 2017

15370-15375

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Revertant mutants G550E and 4RK rescue cystic fibrosis mutants in the first nucleotide-binding domain of CFTR by different mechanisms

11 August 2017

1 reference

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Bioelectric properties of chloride channels in human, pig, ferret, and mouse airway epithelia

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1976592

Cystic fibrosis mouse models

2 October 2017

1 reference

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CFTR chloride channel drug discovery--inhibitors as antidiarrheals and activators for therapy of cystic fibrosis

2 October 2017

1 reference

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

2 October 2017

1 reference

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The short apical membrane half-life of rescued {Delta}F508-cystic fibrosis transmembrane conductance regulator (CFTR) results from accelerated endocytosis of {Delta}F508-CFTR in polarized human airway epithelial cells

2 October 2017

1 reference

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

2 October 2017

1 reference

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Activating cystic fibrosis transmembrane conductance regulator channels with pore blocker analogs

2 October 2017

1 reference

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Processing of CFTR: traversing the cellular maze--how much CFTR needs to go through to avoid cystic fibrosis?

2 October 2017

1 reference

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The DeltaF508 cystic fibrosis mutation impairs domain-domain interactions and arrests post-translational folding of CFTR.

2 October 2017

1 reference

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COPII-dependent export of cystic fibrosis transmembrane conductance regulator from the ER uses a di-acidic exit code

2 October 2017

1 reference

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Cystic fibrosis: recent structural insights

2 October 2017

1 reference

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

2 October 2017

1 reference

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Endocytic trafficking routes of wild type and DeltaF508 cystic fibrosis transmembrane conductance regulator

2 October 2017

1 reference

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Structure of nucleotide-binding domain 1 of the cystic fibrosis transmembrane conductance regulator

2 October 2017

1 reference

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CFTR with a partially deleted R domain corrects the cystic fibrosis chloride transport defect in human airway epithelia in vitro and in mouse nasal mucosa in vivo

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1976592

Regulated trafficking of the CFTR chloride channel

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1976592

Cystic fibrosis F508del patients have apically localized CFTR in a reduced number of airway cells.

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1976592

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

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1976592

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

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1976592

DeltaF508 CFTR protein expression in tissues from patients with cystic fibrosis

2 October 2017

1 reference

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Pathophysiology of gene-targeted mouse models for cystic fibrosis

2 October 2017

1 reference

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

2 October 2017

1 reference

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Chemical chaperones correct the mutant phenotype of the delta F508 cystic fibrosis transmembrane conductance regulator protein

2 October 2017

1 reference

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

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1976592

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

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1976592

Cystic fibrosis: genotypic and phenotypic variations

2 October 2017

1 reference

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

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1976592

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

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1976592

A mouse model for the cystic fibrosis delta F508 mutation.

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1976592

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

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1976592

Characterization of glycoproteins and their associated oligosaccharides through the use of endoglycosidases

2 October 2017

1 reference

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

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1976592

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

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1976592

Cloning the mouse homolog of the human cystic fibrosis transmembrane conductance regulator gene.

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1976592

An animal model for cystic fibrosis made by gene targeting

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1976592

Abnormal localization of cystic fibrosis transmembrane conductance regulator in primary cultures of cystic fibrosis airway epithelia

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1976592

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

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1976592

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

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1976592

Mutation of R555 in CFTR-delta F508 enhances function and partially corrects defective processing.

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1976592

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

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1976592

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

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1976592

SERCA pump inhibitors do not correct biosynthetic arrest of deltaF508 CFTR in cystic fibrosis

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1976592

Characterization of wild-type and deltaF508 cystic fibrosis transmembrane regulator in human respiratory epithelia

23 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1976592

Curcumin stimulates cystic fibrosis transmembrane conductance regulator Cl- channel activity.

23 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1976592

Impact of the deltaF508 mutation in first nucleotide-binding domain of human cystic fibrosis transmembrane conductance regulator on domain folding and structure

23 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1976592

An intrinsic adenylate kinase activity regulates gating of the ABC transporter CFTR.

23 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1976592

An in vitro model of differentiated human airway epithelia. Methods for establishing primary cultures.

23 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1976592

Correction of delF508-CFTR activity with benzo(c)quinolizinium compounds through facilitation of its processing in cystic fibrosis airway cells

23 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1976592

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

23 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1976592

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

23 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1976592

Intracellular turnover of cystic fibrosis transmembrane conductance regulator. Inefficient processing and rapid degradation of wild-type and mutant proteins

23 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1976592

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

23 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1976592

Biochemical methods to assess CFTR expression and membrane localization

23 September 2018

1 reference

Crossref

https://api.crossref.org/works/10.1073%2FPNAS.0706974104

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

21 January 2018

1 reference

Crossref

https://api.crossref.org/works/10.1073%2FPNAS.0706974104

Pharmacologic approaches to correcting the basic defect in cystic fibrosis

21 January 2018

1 reference

12 December 2020

Processing of CFTR bearing the P574H mutation differs from wild-type and deltaF508-CFTR

1 reference

12 December 2020

Mislocalization of ΔF508 CFTR in cystic fibrosis sweat gland

1 reference

12 December 2020

Correction of lethal intestinal defect in a mouse model of cystic fibrosis by human CFTR

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

Removal of multiple arginine-framed trafficking signals overcomes misprocessing of delta F508 CFTR present in most patients with cystic fibrosis

1 reference

12 December 2020

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

1 reference

12 December 2020

Identifiers

DOI

10.1073/PNAS.0706974104

1 reference

11 August 2017

0 references

1 reference

11 August 2017

1 reference