Molecular mechanisms of CFTR chloride channel dysfunction in cystic fibrosis

Jun 1, 1996·European Journal of Cancer : Official Journal for European Organization for Research and Treatment of Cancer (EORTC) [and] European Association for Cancer Research (EACR)·D W LoeS P Cole

Gates of Janus: cystic fibrosis and diarrhea

Mar 1, 1994·Trends in Microbiology·S E Guggino

Regulation of CFTR channel gating

Nov 1, 1994·Trends in Biochemical Sciences·D C Gadsby, A C Nairn

ATP-binding cassette proteins Common denominators between ion channels, transporters, and enzymes

Jul 1, 1994·Trends in Cardiovascular Medicine·L M Greenberger, Y Ishikawa

Association between Staphylococcus aureus alone or combined with Pseudomonas aeruginosa and the clinical condition of patients with cystic fibrosis

Jan 8, 2013·Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society·Dominique HubertJoël Coste

Correlation of sweat chloride concentration with genotypes in cystic fibrosis patients in Saguenay Lac-Saint-Jean, Quebec, Canada

Apr 29, 1998·Clinical Biochemistry·M De BraekeleerF Simard

Inhibitory effect of endothelin-1 on the isoproterenol-induced chloride current in human cardiac myocytes

Jul 31, 2001·European Journal of Pharmacology·C S TsaiS H Loh

Common variants in the gene encoding ATP-binding cassette transporter 1 in men with low HDL cholesterol levels and coronary heart disease

Mar 21, 2001·Atherosclerosis·M E BrousseauG Schmitz

Synthesis of a molecular mimic of the Glc1Man9 oligoside as potential inhibitor of calnexin binding to DeltaF508 CFTR protein

Apr 20, 2002·Bioorganic & Medicinal Chemistry Letters·Slim CherifClaude Monneret

Beta-sarcoglycan: genomic analysis and identification of a novel missense mutation in the LGMD2E Amish isolate

May 5, 1998·Neuromuscular Disorders : NMD·F DuclosK P Campbell

Evolution of the delta F508 CFTR mutation

Mar 19, 1999·Trends in Microbiology·S E Guggino

Increased expression of a novel Aspergillus fumigatus ABC transporter gene, atrF, in the presence of itraconazole in an itraconazole resistant clinical isolate

Jul 24, 2002·Fungal Genetics and Biology : FG & B·John W SlavenDavid W Denning

❮ Previous

Next ❯

Related Concepts

Related Feeds

Cell eTOC

Cell is a scientific journal publishing research across a broad range of disciplines within the life sciences field. Discover the latest research from Cell here.

Molecular mechanisms of CFTR chloride channel dysfunction in cystic fibrosis

Abstract

References

Mutations and sequence variations detected in the cystic fibrosis transmembrane conductance regulator (CFTR) gene: a report from the Cystic Fibrosis Genetic Analysis Consortium

Mislocalization of delta F508 CFTR in cystic fibrosis sweat gland

Cystic fibrosis transmembrane conductance regulator: a chloride channel with novel regulation

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

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

Regulation by ATP and ADP of CFTR chloride channels that contain mutant nucleotide-binding domains

A cluster of cystic fibrosis mutations in the first nucleotide-binding fold of the cystic fibrosis conductance regulator protein

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

Defective acidification of intracellular organelles in cystic fibrosis

Cystic fibrosis with three mutations in the cystic fibrosis transmembrane conductance regulator gene

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

Altered chloride ion channel kinetics associated with the delta F508 cystic fibrosis mutation

Chloride conductance expressed by delta F508 and other mutant CFTRs in Xenopus oocytes

Cystic fibrosis gene mutation in two sisters with mild disease and normal sweat electrolyte levels

Cystic fibrosis: a disease in electrolyte transport

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

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

Mutations in CFTR associated with mild-disease-form Cl- channels with altered pore properties

Identification of revertants for the cystic fibrosis delta F508 mutation using STE6-CFTR chimeras in yeast

Citations

Interactions between secondhand smoke and genes that affect cystic fibrosis lung disease.

Turnover of the cystic fibrosis transmembrane conductance regulator (CFTR): slow degradation of wild-type and delta F508 CFTR in surface membrane preparations of immortalized airway epithelial cells

Genotype-phenotype correlations in cystic fibrosis

Serum lipase levels as a diagnostic marker in cystic fibrosis patients with normal or borderline sweat tests

European Epidemiologic Registry of Cystic Fibrosis (ERCF): comparison of major disease manifestations between patients with different classes of mutations

Dysregulation of proteoglycan production by intrahepatic biliary epithelial cells bearing defective (delta-f508) cystic fibrosis transmembrane conductance regulator

Gene therapy for cystic fibrosis

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

Bone marrow stem cells do not repopulate the healthy upper respiratory tract

Molecular biology of cystic fibrosis: state of the art

The significance of sweat Cl/Na ratio in patients with borderline sweat test

A recombinant peptide model of the first nucleotide-binding fold of the cystic fibrosis transmembrane conductance regulator: comparison of wild-type and delta F508 mutant forms

Four adult patients with the missense mutation L206W and a mild cystic fibrosis phenotype

Treatment of cystic fibrosis based on understanding CFTR

Characterization of a 7,8-benzoflavone double effect on CFTR Cl(-) channel activity

Cystic fibrosis lung disease: genetic influences, microbial interactions, and radiological assessment

ClC-5 mutations associated with Dent's disease: a major role of the dimer interface.

Congenital nephrogenic diabetes insipidus: the current state of affairs.

Serum lipase levels pre and post Lundh meal: evaluation of exocrine pancreatic status in cystic fibrosis

Light and alcohol evoked electro-oculograms in cystic fibrosis

Controlling protein compartmentalization to overcome disease

Quantitative atlas of membrane transporter proteins: development and application of a highly sensitive simultaneous LC/MS/MS method combined with novel in-silico peptide selection criteria.

Expression and localization of cystic fibrosis transmembrane conductance regulator in the rat endocrine pancreas.

Managing the underlying cause of cystic fibrosis: a future role for potentiators and correctors

Cystic fibrosis and congenital agenesis of the vas deferens, antisperm antibodies and CF-genotype

Chloride channels of intracellular organelles

Biology of the multidrug resistance-associated protein, MRP

Gates of Janus: cystic fibrosis and diarrhea

Regulation of CFTR channel gating

ATP-binding cassette proteins Common denominators between ion channels, transporters, and enzymes

Association between Staphylococcus aureus alone or combined with Pseudomonas aeruginosa and the clinical condition of patients with cystic fibrosis

CFTR-mutation specific applications of CFTR-directed monoclonal antibodies

Macromolecular complexes of cystic fibrosis transmembrane conductance regulator and its interacting partners

Cystic fibrosis: a multiple exocrinopathy caused by dysfunctions in a multifunctional transport protein

Correlation of sweat chloride concentration with genotypes in cystic fibrosis patients in Saguenay Lac-Saint-Jean, Quebec, Canada

Inhibitory effect of endothelin-1 on the isoproterenol-induced chloride current in human cardiac myocytes

Common variants in the gene encoding ATP-binding cassette transporter 1 in men with low HDL cholesterol levels and coronary heart disease

Localization of G-protein-coupled receptors in health and disease

Molecular pharmacology of the CFTR Cl- channel

The Wilson's disease gene and phenotypic diversity

Molecular regulation of hepatocellular transport systems in cholestasis

Liver involvement in cystic fibrosis

An overview of the pathogenesis of cystic fibrosis lung disease

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

Airway delivery of cationic lipid: DNA complexes for cystic fibrosis

Toxicological relevance of the multidrug resistance protein 1, MRP1 (ABCC1) and related transporters