(Q35012417)

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:12458151%20AND%20SRC:MED&resulttype=core&format=json

15 December 2019

review article

1 reference

title

Therapeutic approaches to repair defects in deltaF508 CFTR folding and cellular targeting (English)

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:12458151%20AND%20SRC:MED&resulttype=core&format=json

15 December 2019

2

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:12458151%20AND%20SRC:MED&resulttype=core&format=json

15 December 2019

author name string

Kristina Powell

series ordinal

1

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:12458151%20AND%20SRC:MED&resulttype=core&format=json

15 December 2019

language of work or name

English

0 references

publication date

1 December 2002

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:12458151%20AND%20SRC:MED&resulttype=core&format=json

Advanced Drug Delivery Reviews

15 December 2019

published in

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:12458151%20AND%20SRC:MED&resulttype=core&format=json

15 December 2019

volume

54

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:12458151%20AND%20SRC:MED&resulttype=core&format=json

15 December 2019

issue

11

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:12458151%20AND%20SRC:MED&resulttype=core&format=json

15 December 2019

page(s)

1395-1408

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:12458151%20AND%20SRC:MED&resulttype=core&format=json

Clinical implications of cystic fibrosis transmembrane conductance regulator mutations

15 December 2019

cites work

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

Demonstration That CFTR Is a Chloride Channel by Alteration of Its Anion Selectivity

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

Differential expression of ORCC and CFTR induced by low temperature in CF airway epithelial cells

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

CFTR and outward rectifying chloride channels are distinct proteins with a regulatory relationship

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

CFTR as a cAMP-dependent regulator of sodium channels

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

The diagnosis of cystic fibrosis: a consensus statement. Cystic Fibrosis Foundation Consensus Panel

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

Degradation of CFTR by the ubiquitin-proteasome pathway

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

DeltaF508 CFTR protein expression in tissues from patients with cystic fibrosis

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

Cystic fibrosis locus defined by a genetically linked polymorphic DNA marker.

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

A polymorphic DNA marker linked to cystic fibrosis is located on chromosome 7.

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

Identification of the cystic fibrosis gene: chromosome walking and jumping

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

Cellular heterogeneity of CFTR expression and function in the lung: implications for gene therapy of cystic fibrosis.

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

New models of the tracheal airway define the glandular contribution to airway surface fluid and electrolyte composition.

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

CFTR regulates outwardly rectifying chloride channels through an autocrine mechanism involving ATP

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

Defective regulation of outwardly rectifying Cl- channels by protein kinase A corrected by insertion of CFTR

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

Regulation of epithelial sodium channels by the cystic fibrosis transmembrane conductance regulator.

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

Traffic pattern of cystic fibrosis transmembrane regulator through the early exocytic pathway

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

Delta F508 CFTR localizes in the endoplasmic reticulum-Golgi intermediate compartment in cystic fibrosis cells.

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

Cotranslational ubiquitination of cystic fibrosis transmembrane conductance regulator in vitro

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

Molecular mechanisms of CFTR chloride channel dysfunction in cystic fibrosis

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

Empty MHC class I molecules come out in the cold

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

Purification and functional reconstitution of the cystic fibrosis transmembrane conductance regulator (CFTR)

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

Mechanism of protein stabilization by glycerol: preferential hydration in glycerol-water mixtures

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

Microtubule Assembly in the Absence of Added Nucleotides

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

Molecular chaperones in cellular protein folding

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

The molecular chaperone Hsc70 assists the in vitro folding of the N-terminal nucleotide-binding domain of the cystic fibrosis transmembrane conductance regulator.

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

Post-translational disruption of the delta F508 cystic fibrosis transmembrane conductance regulator (CFTR)-molecular chaperone complex with geldanamycin stabilizes delta F508 CFTR in the rabbit reticulocyte lysate.

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

Sodium 4-phenylbutyrate downregulates HSC70 expression by facilitating mRNA degradation

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

Induction of HSP70 promotes DeltaF508 CFTR trafficking

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

The effects of sodium butyrate on transcription are mediated through activation of a protein phosphatase

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

Concentration-dependent effects of sodium butyrate in Chinese hamster cells: cell-cycle progression, inner-histone acetylation, histone H1 dephosphorylation, and induction of an H1-like protein.

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

Scaffold-attached regions (SAR elements) mediate transcriptional effects due to butyrate

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

Increased fetal hemoglobin in patients receiving sodium 4-phenylbutyrate

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

Differentiation of cultured human melanoma cells induced by the aromatic fatty acids phenylacetate and phenylbutyrate

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

Butyrate and phenylacetate as differentiating agents: practical problems and opportunities.

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

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

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

Evidence of CFTR function in cystic fibrosis after systemic administration of 4-phenylbutyrate

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

Modulation of heat-shock protein 70 (HSP70) gene expression by sodium butyrate in U-937 promonocytic cells: relationships with differentiation and apoptosis

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

Conformational and temperature-sensitive stability defects of the delta F508 cystic fibrosis transmembrane conductance regulator in post-endoplasmic reticulum compartments.

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

Isobutylmethylxanthine fails to stimulate chloride secretion in cystic fibrosis airway epithelia

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

A1 receptor antagonist 8-cyclopentyl-1,3-dipropylxanthine selectively activates chloride efflux from human epithelial and mouse fibroblast cell lines expressing the cystic fibrosis transmembrane regulator delta F508 mutation

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

Direct activation of cystic fibrosis transmembrane conductance regulator channels by 8-cyclopentyl-1,3-dipropylxanthine (CPX) and 1,3-diallyl-8-cyclohexylxanthine (DAX).

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

8-cyclopentyl-1,3-dipropylxanthine and other xanthines differentially bind to the wild-type and delta F508 first nucleotide binding fold (NBF-1) domains of the cystic fibrosis transmembrane conductance regulator

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

A phase I randomized, multicenter trial of CPX in adult subjects with mild cystic fibrosis

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

Alternate stimulation of apical CFTR by genistein in epithelia

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

Defective function of the cystic fibrosis-causing missense mutation G551D is recovered by genistein

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

CFTR chloride channel activation by genistein: the role of serine/threonine protein phosphatases

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

Genistein directly induces cardiac CFTR chloride current by a tyrosine kinase-independent and protein kinase A-independent pathway in guinea pig ventricular myocytes

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

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

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

High susceptibility for cystic fibrosis human airway gland cells to produce IL-8 through the I kappa B kinase alpha pathway in response to extracellular NaCl content

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

Selective up-regulation of chemokine IL-8 expression in cystic fibrosis bronchial gland cells in vivo and in vitro

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

Relationship between IkappaBalpha deficiency, NFkappaB activity and interleukin-8 production in CF human airway epithelial cells

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

Genistein inhibits constitutive and inducible NFkappaB activation and decreases IL-8 production by human cystic fibrosis bronchial gland cells

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

Genistein restores functional interactions between Delta F508-CFTR and ENaC in Xenopus oocytes

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

Quercetin inhibits heat shock protein induction but not heat shock factor DNA-binding in human breast carcinoma cells

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

Cell-based assay for high-throughput quantitative screening of CFTR chloride transport agonists

1 reference

https://api.crossref.org/works/10.1016%2FS0169-409X%2802%2900148-5

7 January 2021

10.1016/S0169-409X(02)00148-5

Identifiers

DOI

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:12458151%20AND%20SRC:MED&resulttype=core&format=json

15 December 2019

1 reference