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

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title

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

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15 December 2019

2

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15 December 2019

author name string

Kristina Powell

series ordinal

1

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15 December 2019

language of work or name

English

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publication date

1 December 2002

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Advanced Drug Delivery Reviews

15 December 2019

published in

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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

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15 December 2019

issue

11

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15 December 2019

page(s)

1395-1408

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Clinical implications of cystic fibrosis transmembrane conductance regulator mutations

15 December 2019

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

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

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Molecular mechanisms of CFTR chloride channel dysfunction in cystic fibrosis

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

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

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

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

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7 January 2021

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7 January 2021

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7 January 2021

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

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7 January 2021

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7 January 2021

Isobutylmethylxanthine fails to stimulate chloride secretion in cystic fibrosis airway epithelia

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7 January 2021

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

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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

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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).

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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

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7 January 2021

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7 January 2021

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

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7 January 2021

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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

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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.

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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

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7 January 2021

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

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7 January 2021

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

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7 January 2021

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

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7 January 2021

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

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7 January 2021

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

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Identifiers

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15 December 2019

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