Published in J Membr Biol on July 15, 2003
Toward gene therapy for cystic fibrosis using a lentivirus pseudotyped with Sendai virus envelopes. Mol Ther (2010) 1.03
5-Nitro-1-nonyl-1H-benzimidazol-2(3H)-one. Acta Crystallogr Sect E Struct Rep Online (2011) 0.91
Phosphorylation alters the pharmacology of Ca(2+)-activated Cl channels in rabbit pulmonary arterial smooth muscle cells. Br J Pharmacol (2009) 0.90
4-Chloro-benzo[F]isoquinoline (CBIQ) activates CFTR chloride channels and KCNN4 potassium channels in Calu-3 human airway epithelial cells. Br J Pharmacol (2004) 0.85
The use of carboxymethylcellulose gel to increase non-viral gene transfer in mouse airways. Biomaterials (2009) 0.81
Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA. Science (1989) 40.22
Molecular mechanisms of CFTR chloride channel dysfunction in cystic fibrosis. Cell (1993) 6.25
A cluster of cystic fibrosis mutations in the first nucleotide-binding fold of the cystic fibrosis conductance regulator protein. Nature (1990) 5.42
Phosphorylation-regulated Cl- channel in CHO cells stably expressing the cystic fibrosis gene. Nature (1991) 3.61
Nucleoside triphosphates are required to open the CFTR chloride channel. Cell (1991) 3.21
Chloride conductance expressed by delta F508 and other mutant CFTRs in Xenopus oocytes. Science (1991) 3.12
Control of CFTR channel gating by phosphorylation and nucleotide hydrolysis. Physiol Rev (1999) 2.89
Protein kinase A (PKA) still activates CFTR chloride channel after mutagenesis of all 10 PKA consensus phosphorylation sites. J Biol Chem (1993) 2.85
Physiological basis of cystic fibrosis: a historical perspective. Physiol Rev (1999) 2.60
Pharmacology of CFTR chloride channel activity. Physiol Rev (1999) 2.41
ATPase activity of the cystic fibrosis transmembrane conductance regulator. J Biol Chem (1996) 2.33
Maturation and function of cystic fibrosis transmembrane conductance regulator variants bearing mutations in putative nucleotide-binding domains 1 and 2. Mol Cell Biol (1991) 2.25
Coupling of CFTR Cl- channel gating to an ATP hydrolysis cycle. Neuron (1994) 2.20
cAMP-independent activation of CFTR Cl channels by the tyrosine kinase inhibitor genistein. Am J Physiol (1995) 1.69
Pharmacological modulation of ion transport across wild-type and DeltaF508 CFTR-expressing human bronchial epithelia. Am J Physiol Cell Physiol (2000) 1.68
Defective function of the cystic fibrosis-causing missense mutation G551D is recovered by genistein. Am J Physiol (1999) 1.57
The substituted benzimidazolone NS004 is an opener of the cystic fibrosis chloride channel. J Biol Chem (1994) 1.51
A simple assay for agonist-regulated Cl and K conductances in salt-secreting epithelial cells. Am J Physiol (1990) 1.50
Phosphatase inhibitors activate normal and defective CFTR chloride channels. Proc Natl Acad Sci U S A (1994) 1.34
Correction of delF508-CFTR activity with benzo(c)quinolizinium compounds through facilitation of its processing in cystic fibrosis airway cells. J Cell Sci (2001) 1.34
Phosphorylation by cAMP-dependent protein kinase causes a conformational change in the R domain of the cystic fibrosis transmembrane conductance regulator. Biochemistry (1994) 1.32
Functional roles of the nucleotide-binding folds in the activation of the cystic fibrosis transmembrane conductance regulator. Proc Natl Acad Sci U S A (1993) 1.28
Development of substituted Benzo[c]quinolizinium compounds as novel activators of the cystic fibrosis chloride channel. J Biol Chem (1999) 1.26
Regulation of CFTR channel gating. Trends Biochem Sci (1994) 1.24
Modulation of Cl- secretion by benzimidazolones. II. Coordinate regulation of apical GCl and basolateral GK. Am J Physiol (1996) 1.23
Cystic fibrosis transmembrane conductance regulator mutations that disrupt nucleotide binding. J Clin Invest (1994) 1.20
ATP hydrolysis by a CFTR domain: pharmacology and effects of G551D mutation. Biochem Biophys Res Commun (2000) 1.08
A common mechanism for cystic fibrosis transmembrane conductance regulator protein activation by genistein and benzimidazolone analogs. J Pharmacol Exp Ther (2001) 1.07
A1 adenosine-receptor antagonists activate chloride efflux from cystic fibrosis cells. Proc Natl Acad Sci U S A (1992) 1.03
The cystic fibrosis mutation G551D alters the non-Michaelis-Menten behavior of the cystic fibrosis transmembrane conductance regulator (CFTR) channel and abolishes the inhibitory Genistein binding site. J Biol Chem (2002) 0.92
Activation of G551D CFTR channel with MPB-91: regulation by ATPase activity and phosphorylation. Am J Physiol Cell Physiol (2001) 0.91
CFTR-mediated chloride permeability is regulated by type III phosphodiesterases in airway epithelial cells. Am J Respir Cell Mol Biol (1995) 0.86
cAMP- and Ca2+-independent activation of cystic fibrosis transmembrane conductance regulator channels by phenylimidazothiazole drugs. J Biol Chem (1996) 0.85
Structural basis for specificity and potency of xanthine derivatives as activators of the CFTR chloride channel. Br J Pharmacol (1998) 0.84
Properties of CFTR activated by the xanthine derivative X-33 in human airway Calu-3 cells. Am J Physiol Cell Physiol (2000) 0.83
Cystic fibrosis transmembrane conductance regulator activation by cAMP-independent mechanisms. Am J Physiol (1998) 0.82
Development of chloride channel modulators. Kidney Int (1995) 0.81
Genistein modifies the activation kinetics and magnitude of phosphorylated wild-type and G551D-CFTR chloride currents. J Membr Biol (2002) 0.77
Interleukin-1beta secretion is impaired by inhibitors of the Atp binding cassette transporter, ABC1. Blood (1997) 1.67
9-phenanthrol inhibits human TRPM4 but not TRPM5 cationic channels. Br J Pharmacol (2008) 1.48
Correction of delF508-CFTR activity with benzo(c)quinolizinium compounds through facilitation of its processing in cystic fibrosis airway cells. J Cell Sci (2001) 1.34
Sildenafil (Viagra) corrects DeltaF508-CFTR location in nasal epithelial cells from patients with cystic fibrosis. Thorax (2005) 0.98
Tat1, a novel sulfate transporter specifically expressed in human male germ cells and potentially linked to rhogtpase signaling. J Biol Chem (2001) 0.95
A cystic fibrosis tracheal gland cell line, CF-KM4. Correction by adenovirus-mediated CFTR gene transfer. Am J Respir Cell Mol Biol (1999) 0.95
Activation of G551D CFTR channel with MPB-91: regulation by ATPase activity and phosphorylation. Am J Physiol Cell Physiol (2001) 0.91
Expression and function of cystic fibrosis transmembrane conductance regulator in rat intrapulmonary arteries. Eur Respir J (2007) 0.89
The expression of carbonic anhydrases II and IV in the human pancreatic cancer cell line (Capan 1) is associated with bicarbonate ion channels. Biol Cell (1994) 0.88
Localisation of wild-type and DeltaF508-CFTR in nasal epithelial cells. Pflugers Arch (2001) 0.87
Structural basis for specificity and potency of xanthine derivatives as activators of the CFTR chloride channel. Br J Pharmacol (1998) 0.84
Properties of CFTR activated by the xanthine derivative X-33 in human airway Calu-3 cells. Am J Physiol Cell Physiol (2000) 0.83
Function and dysfunction of the CFTR chloride channel. Soc Gen Physiol Ser (1995) 0.83
Acute effects of adenosine triphosphates, cyclic 3',5'-adenosine monophosphates, and follicle-stimulating hormone on cytosolic calcium level in cultured immature rat Ssertoli cells. Biol Reprod (1999) 0.83
From the vasodilator and hypotensive effects of an extract fraction from Agelanthus dodoneifolius (DC) Danser (Loranthaceae) to the active compound dodoneine. J Ethnopharmacol (2010) 0.78
Cystic fibrosis transmembrane conductance regulator (CFTR) confers glibenclamide sensitivity to outwardly rectifying chloride channel (ORCC) in Hi-5 insect cells. J Membr Biol (1999) 0.78
Genistein modifies the activation kinetics and magnitude of phosphorylated wild-type and G551D-CFTR chloride currents. J Membr Biol (2002) 0.77
Deficit of osteoprotegerin release by osteoblasts from a patient with cystic fibrosis. Eur Respir J (2012) 0.76
Roscovitine is a proteostasis regulator that corrects the trafficking defect of F508del-CFTR by a CDK-independent mechanism. Br J Pharmacol (2014) 0.76
Identification of a novel water-soluble activator of wild-type and F508del CFTR: GPact-11a. Eur Respir J (2010) 0.75
N- and C-alkylation of seven-membered iminosugars generates potent glucocerebrosidase inhibitors and F508del-CFTR correctors. Org Biomol Chem (2014) 0.75