Strategies for correcting the delta F508 CFTR protein-folding defect (Q41725526)

8 October 2017

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title

Strategies for correcting the delta F508 CFTR protein-folding defect (English)

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8 October 2017

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Brown CR

1

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8 October 2017

Hong-Brown LQ

series ordinal

2

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8 October 2017

Welch WJ

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3

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8 October 2017

language of work or name

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1 October 1997

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Journal of Bioenergetics and Biomembranes

8 October 2017

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8 October 2017

29

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8 October 2017

491-502

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8 October 2017

5

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https://scigraph.springernature.com/pub.10.1023/a:1022491124939

8 October 2017

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cites work

Folding intermediates are involved in genetic diseases?

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12 December 2020

Identification of the cystic fibrosis gene: chromosome walking and jumping

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12 December 2020

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

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12 December 2020

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

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12 December 2020

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

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12 December 2020

Accumulation of p53 in a mutant cell line defective in the ubiquitin pathway

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12 December 2020

Protection of heat induced cytoxicity by glycerol

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12 December 2020

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

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12 December 2020

Molecular mechanisms of CFTR chloride channel dysfunction in cystic fibrosis

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12 December 2020

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

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12 December 2020

Chemical chaperones interfere with the formation of scrapie prion protein

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12 December 2020

Intracellular protein trafficking defects in human disease

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12 December 2020

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

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12 December 2020

Molecular basis of osmotic regulation

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12 December 2020

Renal medullary organic osmolytes

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12 December 2020

Increased thermal stability of proteins in the presence of sugars and polyols

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12 December 2020

Protection against heat-induced cell killing by polyols in vitro

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12 December 2020

Cellular localization and cell cycle regulation by a temperature-sensitive p53 protein

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12 December 2020

Defective protein folding as a basis of human disease

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12 December 2020

Non-invasive liposome-mediated gene delivery can correct the ion transport defect in cystic fibrosis mutant mice

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12 December 2020

Influence of molecular and chemical chaperones on protein folding

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12 December 2020

Severe deficiency of cystic fibrosis transmembrane conductance regulator messenger RNA carrying nonsense mutations R553X and W1316X in respiratory epithelial cells of patients with cystic fibrosis

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12 December 2020

Phosphatase inhibitors activate normal and defective CFTR chloride channels

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12 December 2020

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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12 December 2020

Aminoglycoside antibiotics restore CFTR function by overcoming premature stop mutations

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12 December 2020

Protons, osmolytes, and fitness of internal milieu for protein function

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12 December 2020

Inhibition of heat shock (stress) protein induction by deuterium oxide and glycerol: additional support for the abnormal protein hypothesis of induction

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12 December 2020

Living with water stress: evolution of osmolyte systems

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12 December 2020

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

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12 December 2020

cAMP- and Ca2+-independent activation of cystic fibrosis transmembrane conductance regulator channels by phenylimidazothiazole drugs

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12 December 2020

OSMOTIC-REMEDIAL MUTANTS. A NEW CLASSIFICATION FOR NUTRITIONAL MUTANTS IN YEAST

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12 December 2020

Repeat administration of an adenovirus vector encoding cystic fibrosis transmembrane conductance regulator to the nasal epithelium of patients with cystic fibrosis

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12 December 2020

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

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12 December 2020

Identification of the cystic fibrosis gene: genetic analysis

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12 December 2020

Alternate stimulation of apical CFTR by genistein in epithelia

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12 December 2020

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

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12 December 2020

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

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12 December 2020

Detailed analysis of structures and formulations of cationic lipids for efficient gene transfer to the lung

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12 December 2020

Thermodynamic and kinetic examination of protein stabilization by glycerol

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12 December 2020

Stimulation by alkylxanthines of chloride efflux in CFPAC-1 cells does not involve A1 adenosine receptors

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12 December 2020

Liposome-mediated CFTR gene transfer to the nasal epithelium of patients with cystic fibrosis

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12 December 2020

Increased thermal stability of collagen in the presence of sugars and polyols

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12 December 2020

Correcting temperature-sensitive protein folding defects

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12 December 2020

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

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12 December 2020

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

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12 December 2020

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

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12 December 2020

Identifiers

DOI

10.1023/A:1022491124939

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Dimensions Publication ID

8 October 2017

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