Published in Mol Pharmacol on February 18, 2005
Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770. Proc Natl Acad Sci U S A (2009) 5.72
Small-molecule correctors of defective DeltaF508-CFTR cellular processing identified by high-throughput screening. J Clin Invest (2005) 4.19
Chloride channels as drug targets. Nat Rev Drug Discov (2008) 3.22
Chemistry and biology of multicomponent reactions. Chem Rev (2012) 2.09
CFTR: folding, misfolding and correcting the ΔF508 conformational defect. Trends Mol Med (2011) 1.99
G551D and G1349D, two CF-associated mutations in the signature sequences of CFTR, exhibit distinct gating defects. J Gen Physiol (2007) 1.64
Genetically encoded optical sensors for monitoring of intracellular chloride and chloride-selective channel activity. Front Mol Neurosci (2009) 1.25
Cystic fibrosis transmembrane regulator correctors and potentiators. Cold Spring Harb Perspect Med (2013) 1.20
Small molecule correctors of F508del-CFTR discovered by structure-based virtual screening. J Comput Aided Mol Des (2010) 1.17
4'-Methyl-4,5'-bithiazole-based correctors of defective delta F508-CFTR cellular processing. Bioorg Med Chem Lett (2008) 1.16
Diversity of Cl(-) channels. Cell Mol Life Sci (2006) 1.15
Pharmacological rescue of the mutant cystic fibrosis transmembrane conductance regulator (CFTR) detected by use of a novel fluorescence platform. Mol Med (2012) 1.15
Cyanoquinolines with independent corrector and potentiator activities restore ΔPhe508-cystic fibrosis transmembrane conductance regulator chloride channel function in cystic fibrosis. Mol Pharmacol (2011) 1.07
A unified view of cystic fibrosis transmembrane conductance regulator (CFTR) gating: combining the allosterism of a ligand-gated channel with the enzymatic activity of an ATP-binding cassette (ABC) transporter. J Biol Chem (2011) 1.03
Thermal instability of ΔF508 cystic fibrosis transmembrane conductance regulator (CFTR) channel function: protection by single suppressor mutations and inhibiting channel activity. Biochemistry (2012) 1.01
Rescue of the mutant CFTR chloride channel by pharmacological correctors and low temperature analyzed by gene expression profiling. Am J Physiol Cell Physiol (2011) 0.98
Dual activity of aminoarylthiazoles on the trafficking and gating defects of the cystic fibrosis transmembrane conductance regulator chloride channel caused by cystic fibrosis mutations. J Biol Chem (2011) 0.97
Design and synthesis of a hybrid potentiator-corrector agonist of the cystic fibrosis mutant protein DeltaF508-CFTR. Bioorg Med Chem Lett (2009) 0.95
Innovative strategies to treat protein misfolding in inborn errors of metabolism: pharmacological chaperones and proteostasis regulators. J Inherit Metab Dis (2014) 0.92
Cystic fibrosis: a new target for 4-Imidazo[2,1-b]thiazole-1,4-dihydropyridines. J Med Chem (2011) 0.92
CFTR regulation in human airway epithelial cells requires integrity of the actin cytoskeleton and compartmentalized cAMP and PKA activity. J Cell Sci (2012) 0.91
Regulatory domain phosphorylation to distinguish the mechanistic basis underlying acute CFTR modulators. Am J Physiol Lung Cell Mol Physiol (2011) 0.91
Fixing cystic fibrosis by correcting CFTR domain assembly. J Cell Biol (2012) 0.90
Targets for cystic fibrosis therapy: proteomic analysis and correction of mutant cystic fibrosis transmembrane conductance regulator. Expert Rev Proteomics (2010) 0.89
Novel amino-carbonitrile-pyrazole identified in a small molecule screen activates wild-type and ΔF508 cystic fibrosis transmembrane conductance regulator in the absence of a cAMP agonist. Mol Pharmacol (2013) 0.89
Targeting F508del-CFTR to develop rational new therapies for cystic fibrosis. Acta Pharmacol Sin (2011) 0.88
MRP4 and CFTR in the regulation of cAMP and β-adrenergic contraction in cardiac myocytes. Eur J Pharmacol (2012) 0.87
Mutation-specific potency and efficacy of cystic fibrosis transmembrane conductance regulator chloride channel potentiators. J Pharmacol Exp Ther (2009) 0.86
Structure-activity relationships of cyanoquinolines with corrector-potentiator activity in ΔF508 cystic fibrosis transmembrane conductance regulator protein. J Med Chem (2012) 0.86
CFTR potentiators partially restore channel function to A561E-CFTR, a cystic fibrosis mutant with a similar mechanism of dysfunction as F508del-CFTR. Br J Pharmacol (2014) 0.86
Local modulation of cystic fibrosis conductance regulator: cytoskeleton and compartmentalized cAMP signalling. Br J Pharmacol (2013) 0.83
Cystic fibrosis transmembrane conductance regulator (CFTR) potentiators protect G551D but not ΔF508 CFTR from thermal instability. Biochemistry (2014) 0.83
Isoxazolopyrimidines as Novel ΔF508-CFTR Correctors. Synlett (2010) 0.82
CFTR inhibitors. Curr Pharm Des (2013) 0.81
Potentiators of Defective ΔF508-CFTR Gating that Do Not Interfere with Corrector Action. Mol Pharmacol (2015) 0.80
Modulation of cystic fibrosis transmembrane conductance regulator (CFTR) activity and genistein binding by cytosolic pH. J Biol Chem (2010) 0.80
Curcumin and genistein: the combined effects on disease-associated CFTR mutants and their clinical implications. Curr Pharm Des (2013) 0.80
Histone deacetylase inhibitors influence chemotherapy transport by modulating expression and trafficking of a common polymorphic variant of the ABCG2 efflux transporter. Cancer Res (2012) 0.79
Correcting the cystic fibrosis disease mutant, A455E CFTR. PLoS One (2014) 0.79
Increased efficacy of VX-809 in different cellular systems results from an early stabilization effect of F508del-CFTR. Pharmacol Res Perspect (2015) 0.78
Enhancing the Potency of F508del Correction: A Multi-Layer Combinational Approach to Drug Discovery for Cystic Fibrosis. J Pharmacol Clin Toxicol (2013) 0.77
Microfluidics platform for single-shot dose-response analysis of chloride channel-modulating compounds. Lab Chip (2013) 0.76
miR-16 rescues F508del-CFTR function in native cystic fibrosis epithelial cells. Gene Ther (2015) 0.75
Potentiation of ΔF508- and G551D-CFTR-Mediated Cl- Current by Novel Hydroxypyrazolines. PLoS One (2016) 0.75
Fluorinated ΔF508-CFTR correctors and potentiators for PET imaging. Bioorg Med Chem Lett (2012) 0.75
Correctors and Potentiators Rescue Function of the Truncated W1282X-CFTR Translation Product. J Biol Chem (2016) 0.75
Potentiators exert distinct effects on human, murine, and Xenopus CFTR. Am J Physiol Lung Cell Mol Physiol (2016) 0.75
Improved fluorescence assays to measure the defects associated with F508del-CFTR allow identification of new active compounds. Br J Pharmacol (2017) 0.75
IgG marker of optic-spinal multiple sclerosis binds to the aquaporin-4 water channel. J Exp Med (2005) 10.39
TMEM16A, a membrane protein associated with calcium-dependent chloride channel activity. Science (2008) 8.74
Aquaporin-4 deletion in mice reduces brain edema after acute water intoxication and ischemic stroke. Nat Med (2000) 6.27
Chemical inhibition of the mitochondrial division dynamin reveals its role in Bax/Bak-dependent mitochondrial outer membrane permeabilization. Dev Cell (2008) 5.67
Thiazolidinone CFTR inhibitor identified by high-throughput screening blocks cholera toxin-induced intestinal fluid secretion. J Clin Invest (2002) 4.97
Tracking of single fluorescent particles in three dimensions: use of cylindrical optics to encode particle position. Biophys J (1994) 4.77
Translational diffusion of macromolecule-sized solutes in cytoplasm and nucleus. J Cell Biol (1997) 4.53
Chloride accumulation and swelling in endosomes enhances DNA transfer by polyamine-DNA polyplexes. J Biol Chem (2003) 4.40
Impairment of angiogenesis and cell migration by targeted aquaporin-1 gene disruption. Nature (2005) 4.20
Small-molecule correctors of defective DeltaF508-CFTR cellular processing identified by high-throughput screening. J Clin Invest (2005) 4.19
Intra-cerebral injection of neuromyelitis optica immunoglobulin G and human complement produces neuromyelitis optica lesions in mice. Brain (2010) 3.95
Plasma microRNA panel to diagnose hepatitis B virus-related hepatocellular carcinoma. J Clin Oncol (2011) 3.80
Aquaporin-4 facilitates reabsorption of excess fluid in vasogenic brain edema. FASEB J (2004) 3.80
Green fluorescent protein as a noninvasive intracellular pH indicator. Biophys J (1998) 3.61
Determinants of the translational mobility of a small solute in cell cytoplasm. J Cell Biol (1993) 3.46
Generation and phenotype of a transgenic knockout mouse lacking the mercurial-insensitive water channel aquaporin-4. J Clin Invest (1997) 3.45
Size-dependent DNA mobility in cytoplasm and nucleus. J Biol Chem (2000) 3.29
Chloride channels as drug targets. Nat Rev Drug Discov (2008) 3.22
Discovery of glycine hydrazide pore-occluding CFTR inhibitors: mechanism, structure-activity analysis, and in vivo efficacy. J Gen Physiol (2004) 3.10
Severely impaired urinary concentrating ability in transgenic mice lacking aquaporin-1 water channels. J Biol Chem (1998) 3.00
Crowding effects on diffusion in solutions and cells. Annu Rev Biophys (2008) 3.00
Defective secretion of saliva in transgenic mice lacking aquaporin-5 water channels. J Biol Chem (1999) 2.91
Aquaporin-1 plays an essential role in water permeability and ultrafiltration during peritoneal dialysis. Kidney Int (2006) 2.89
Molecular cloning of a mercurial-insensitive water channel expressed in selected water-transporting tissues. J Biol Chem (1994) 2.85
Chemical chaperones correct the mutant phenotype of the delta F508 cystic fibrosis transmembrane conductance regulator protein. Cell Stress Chaperones (1996) 2.80
Defective proximal tubular fluid reabsorption in transgenic aquaporin-1 null mice. Proc Natl Acad Sci U S A (1998) 2.73
Low viscosity in the aqueous domain of cell cytoplasm measured by picosecond polarization microfluorimetry. J Cell Biol (1991) 2.73
TMEM16A inhibitors reveal TMEM16A as a minor component of calcium-activated chloride channel conductance in airway and intestinal epithelial cells. J Biol Chem (2010) 2.66
Diffusion of green fluorescent protein in the aqueous-phase lumen of endoplasmic reticulum. Biophys J (1999) 2.59
Aquaporin 4 and neuromyelitis optica. Lancet Neurol (2012) 2.58
Water and glycerol permeabilities of aquaporins 1-5 and MIP determined quantitatively by expression of epitope-tagged constructs in Xenopus oocytes. J Biol Chem (1997) 2.56
Localization of MIWC and GLIP water channel homologs in neuromuscular, epithelial and glandular tissues. J Cell Sci (1995) 2.55
Involvement of aquaporin-4 in astroglial cell migration and glial scar formation. J Cell Sci (2005) 2.48
Anti-aquaporin-4 monoclonal antibody blocker therapy for neuromyelitis optica. Ann Neurol (2012) 2.45
Green fluorescent protein-based halide indicators with improved chloride and iodide affinities. FEBS Lett (2001) 2.38
Mechanism and cellular applications of a green fluorescent protein-based halide sensor. J Biol Chem (2000) 2.38
Immunolocalization of the mercurial-insensitive water channel and glycerol intrinsic protein in epithelial cell plasma membranes. Proc Natl Acad Sci U S A (1995) 2.37
Hyperacidity of secreted fluid from submucosal glands in early cystic fibrosis. Am J Physiol Cell Physiol (2005) 2.32
BMP4 supports self-renewal of embryonic stem cells by inhibiting mitogen-activated protein kinase pathways. Proc Natl Acad Sci U S A (2004) 2.25
Nanomolar affinity small molecule correctors of defective Delta F508-CFTR chloride channel gating. J Biol Chem (2003) 2.25
Localization of the CHIP28 water channel in rat kidney. Am J Physiol (1992) 2.17
Novel role for CFTR in fluid absorption from the distal airspaces of the lung. J Gen Physiol (2002) 2.16
The mercurial insensitive water channel (AQP-4) forms orthogonal arrays in stably transfected Chinese hamster ovary cells. J Biol Chem (1996) 2.16
Regulation of TMEM16A chloride channel properties by alternative splicing. J Biol Chem (2009) 2.16
Rapid diffusion of green fluorescent protein in the mitochondrial matrix. J Cell Biol (1998) 2.12
Submucosal gland secretions in airways from cystic fibrosis patients have normal [Na(+)] and pH but elevated viscosity. Proc Natl Acad Sci U S A (2001) 2.10
Comparison of intravitreal triamcinolone acetonide versus intravitreal bevacizumab as the primary treatment of clinically significant macular edema. Retina (2015) 2.08
Aquaporin-4 gene disruption in mice reduces brain swelling and mortality in pneumococcal meningitis. J Biol Chem (2005) 2.08
Increased migration and metastatic potential of tumor cells expressing aquaporin water channels. FASEB J (2006) 2.07
Lung fluid transport in aquaporin-5 knockout mice. J Clin Invest (2000) 2.07
Loss of tolerance in C57BL/6 mice to the autoantigen E2 subunit of pyruvate dehydrogenase by a xenobiotic with ensuing biliary ductular disease. Hepatology (2008) 2.05
Aquaporins and cell migration. Pflugers Arch (2007) 2.02
Water and urea permeability properties of Xenopus oocytes: expression of mRNA from toad urinary bladder. Am J Physiol (1991) 2.01
Monte Carlo analysis of obstructed diffusion in three dimensions: application to molecular diffusion in organelles. Biophys J (1998) 2.00
Novel CFTR chloride channel activators identified by screening of combinatorial libraries based on flavone and benzoquinolizinium lead compounds. J Biol Chem (2001) 1.99
CFTR: folding, misfolding and correcting the ΔF508 conformational defect. Trends Mol Med (2011) 1.99
Sevenfold-reduced osmotic water permeability in primary astrocyte cultures from AQP-4-deficient mice, measured by a fluorescence quenching method. Am J Physiol Cell Physiol (2003) 1.99
Binding affinity and specificity of neuromyelitis optica autoantibodies to aquaporin-4 M1/M23 isoforms and orthogonal arrays. J Biol Chem (2011) 1.98
K+ waves in brain cortex visualized using a long-wavelength K+-sensing fluorescent indicator. Nat Methods (2005) 1.98
Analysis of fluorophore diffusion by continuous distributions of diffusion coefficients: application to photobleaching measurements of multicomponent and anomalous diffusion. Biophys J (1998) 1.95
Nephrogenic diabetes insipidus in mice lacking aquaporin-3 water channels. Proc Natl Acad Sci U S A (2000) 1.94
Delivery of antioxidative enzyme genes protects against ischemia/reperfusion-induced liver injury in mice. Liver Transpl (2006) 1.90
In vivo measurement of brain extracellular space diffusion by cortical surface photobleaching. J Neurosci (2004) 1.90
High-affinity activators of cystic fibrosis transmembrane conductance regulator (CFTR) chloride conductance identified by high-throughput screening. J Biol Chem (2002) 1.89
Defective aquaporin-2 trafficking in nephrogenic diabetes insipidus and correction by chemical chaperones. J Clin Invest (1998) 1.87
Contribution of CFTR to apical-basolateral fluid transport in cultured human alveolar epithelial type II cells. Am J Physiol Lung Cell Mol Physiol (2005) 1.87
Highly water-permeable type I alveolar epithelial cells confer high water permeability between the airspace and vasculature in rat lung. Proc Natl Acad Sci U S A (1998) 1.85
Functional reconstitution of the isolated erythrocyte water channel CHIP28. J Biol Chem (1992) 1.85
Small-molecule screen identifies inhibitors of a human intestinal calcium-activated chloride channel. Mol Pharmacol (2007) 1.85
Tetrameric assembly of CHIP28 water channels in liposomes and cell membranes: a freeze-fracture study. J Cell Biol (1993) 1.84
Three-dimensional organization of a human water channel. Nature (1997) 1.83
Salt and water transport across alveolar and distal airway epithelia in the adult lung. Am J Physiol (1996) 1.82
Noninvasive in vivo fluorescence measurement of airway-surface liquid depth, salt concentration, and pH. J Clin Invest (2001) 1.80
Cytoplasmic viscosity near the cell plasma membrane: translational diffusion of a small fluorescent solute measured by total internal reflection-fluorescence photobleaching recovery. Biophys J (1996) 1.79
Urea-selective concentrating defect in transgenic mice lacking urea transporter UT-B. J Biol Chem (2002) 1.79
Absence of orthogonal arrays in kidney, brain and muscle from transgenic knockout mice lacking water channel aquaporin-4. J Cell Sci (1997) 1.78
Lack of aquaporin-4 water transport inhibition by antiepileptics and arylsulfonamides. Bioorg Med Chem (2008) 1.75
A small molecule CFTR inhibitor produces cystic fibrosis-like submucosal gland fluid secretions in normal airways. FASEB J (2004) 1.75
Second messengers regulate endosomal acidification in Swiss 3T3 fibroblasts. J Cell Biol (1992) 1.74
Greatly impaired migration of implanted aquaporin-4-deficient astroglial cells in mouse brain toward a site of injury. FASEB J (2006) 1.72
Mapping of fluorescence anisotropy in living cells by ratio imaging. Application to cytoplasmic viscosity. Biophys J (1990) 1.72
Two microRNA panels to discriminate three subtypes of lung carcinoma in bronchial brushing specimens. Am J Respir Crit Care Med (2012) 1.72
Ex vivo spinal cord slice model of neuromyelitis optica reveals novel immunopathogenic mechanisms. Ann Neurol (2011) 1.72
Inhibition of Ca2+-activated Cl- channels by gallotannins as a possible molecular basis for health benefits of red wine and green tea. FASEB J (2010) 1.71
AQP4 gene deletion in mice does not alter blood-brain barrier integrity or brain morphology. Neuroscience (2009) 1.70