Published in Hum Gene Ther on March 01, 2003
Cystic fibrosis pulmonary guidelines: chronic medications for maintenance of lung health. Am J Respir Crit Care Med (2007) 4.34
Variant cystic fibrosis phenotypes in the absence of CFTR mutations. N Engl J Med (2002) 3.50
Structure of nucleotide-binding domain 1 of the cystic fibrosis transmembrane conductance regulator. EMBO J (2003) 3.35
Identification of a urate transporter, ABCG2, with a common functional polymorphism causing gout. Proc Natl Acad Sci U S A (2009) 3.13
Defective lipoxin-mediated anti-inflammatory activity in the cystic fibrosis airway. Nat Immunol (2004) 2.86
Impact of the deltaF508 mutation in first nucleotide-binding domain of human cystic fibrosis transmembrane conductance regulator on domain folding and structure. J Biol Chem (2004) 2.66
Cystic fibrosis pulmonary guidelines: treatment of pulmonary exacerbations. Am J Respir Crit Care Med (2009) 2.65
Inflammatory and microbiologic markers in induced sputum after intravenous antibiotics in cystic fibrosis. Am J Respir Crit Care Med (2003) 2.26
High citrate diet delays progression of renal insufficiency in the ClC-5 knockout mouse model of Dent's disease. Kidney Int (2005) 2.02
A Golgi-associated PDZ domain protein modulates cystic fibrosis transmembrane regulator plasma membrane expression. J Biol Chem (2001) 1.97
Multicenter evaluation of infant lung function tests as cystic fibrosis clinical trial endpoints. Am J Respir Crit Care Med (2010) 1.93
WNK4 enhances the degradation of NCC through a sortilin-mediated lysosomal pathway. J Am Soc Nephrol (2009) 1.87
Loss of chloride channel ClC-5 impairs endocytosis by defective trafficking of megalin and cubilin in kidney proximal tubules. Proc Natl Acad Sci U S A (2003) 1.87
Significant microbiological effect of inhaled tobramycin in young children with cystic fibrosis. Am J Respir Crit Care Med (2002) 1.70
PDZ domain interaction controls the endocytic recycling of the cystic fibrosis transmembrane conductance regulator. J Biol Chem (2002) 1.65
Polycystin 2 interacts with type I inositol 1,4,5-trisphosphate receptor to modulate intracellular Ca2+ signaling. J Biol Chem (2005) 1.64
Standardized procedure for measurement of nasal potential difference: an outcome measure in multicenter cystic fibrosis clinical trials. Pediatr Pulmonol (2004) 1.64
Location and duration of treatment of cystic fibrosis respiratory exacerbations do not affect outcomes. Am J Respir Crit Care Med (2010) 1.62
Selective inhibition of endoplasmic reticulum-associated degradation rescues DeltaF508-cystic fibrosis transmembrane regulator and suppresses interleukin-8 levels: therapeutic implications. J Biol Chem (2006) 1.61
CFTR is a negative regulator of NFkappaB mediated innate immune response. PLoS One (2009) 1.57
The penetration of fresh undiluted sputum expectorated by cystic fibrosis patients by non-adhesive polymer nanoparticles. Biomaterials (2009) 1.54
Drosophila TRPA1 channel mediates chemical avoidance in gustatory receptor neurons. Proc Natl Acad Sci U S A (2010) 1.51
Repeated aerosolized AAV-CFTR for treatment of cystic fibrosis: a randomized placebo-controlled phase 2B trial. Hum Gene Ther (2007) 1.47
Diagnostic sweat testing: the Cystic Fibrosis Foundation guidelines. J Pediatr (2007) 1.45
Impaired acidification in early endosomes of ClC-5 deficient proximal tubule. Biochem Biophys Res Commun (2005) 1.44
Longitudinal association between medication adherence and lung health in people with cystic fibrosis. J Cyst Fibros (2011) 1.41
A phase II, double-blind, randomized, placebo-controlled clinical trial of tgAAVCF using maxillary sinus delivery in patients with cystic fibrosis with antrostomies. Hum Gene Ther (2002) 1.40
Respiratory epithelial gene expression in patients with mild and severe cystic fibrosis lung disease. Am J Respir Cell Mol Biol (2006) 1.39
Functional genomic screening identifies dual leucine zipper kinase as a key mediator of retinal ganglion cell death. Proc Natl Acad Sci U S A (2013) 1.36
Phase I trial of intranasal and endobronchial administration of a recombinant adeno-associated virus serotype 2 (rAAV2)-CFTR vector in adult cystic fibrosis patients: a two-part clinical study. Hum Gene Ther (2003) 1.36
Drosophila TRPA1 channel is required to avoid the naturally occurring insect repellent citronellal. Curr Biol (2010) 1.36
Characterization of aquaporin-6 as a nitrate channel in mammalian cells. Requirement of pore-lining residue threonine 63. J Biol Chem (2002) 1.35
Proteome of synaptosome-associated proteins in spinal cord dorsal horn after peripheral nerve injury. Proteomics (2009) 1.27
Structures of a minimal human CFTR first nucleotide-binding domain as a monomer, head-to-tail homodimer, and pathogenic mutant. Protein Eng Des Sel (2010) 1.26
Modulation of mature cystic fibrosis transmembrane regulator protein by the PDZ domain protein CAL. J Biol Chem (2003) 1.17
PKB and megalin determine the survival or death of renal proximal tubule cells. Proc Natl Acad Sci U S A (2006) 1.16
Functional characterization of a recombinant adeno-associated virus 5-pseudotyped cystic fibrosis transmembrane conductance regulator vector. Hum Gene Ther (2004) 1.16
Targeting CAL as a negative regulator of DeltaF508-CFTR cell-surface expression: an RNA interference and structure-based mutagenetic approach. J Biol Chem (2006) 1.15
Localization studies of rare missense mutations in cystic fibrosis transmembrane conductance regulator (CFTR) facilitate interpretation of genotype-phenotype relationships. Hum Mutat (2008) 1.15
Lubiprostone activates non-CFTR-dependent respiratory epithelial chloride secretion in cystic fibrosis mice. Am J Physiol Lung Cell Mol Physiol (2008) 1.14
Syntaxin 6 and CAL mediate the degradation of the cystic fibrosis transmembrane conductance regulator. Mol Biol Cell (2010) 1.14
Autocrine extracellular purinergic signaling in epithelial cells derived from polycystic kidneys. Am J Physiol Renal Physiol (2002) 1.12
Cystic fibrosis pulmonary guidelines: pulmonary complications: hemoptysis and pneumothorax. Am J Respir Crit Care Med (2010) 1.11
Chemical rescue of deltaF508-CFTR mimics genetic repair in cystic fibrosis bronchial epithelial cells. Mol Cell Proteomics (2008) 1.09
Multiple sequences in the C terminus of MaxiK channels are involved in expression, movement to the cell surface, and apical localization. Proc Natl Acad Sci U S A (2004) 1.07
ClC-5: role in endocytosis in the proximal tubule. Am J Physiol Renal Physiol (2005) 1.07
Nedd4-2 functionally interacts with ClC-5: involvement in constitutive albumin endocytosis in proximal tubule cells. J Biol Chem (2004) 1.06
Pharmacoproteomics of 4-phenylbutyrate-treated IB3-1 cystic fibrosis bronchial epithelial cells. J Proteome Res (2006) 1.06
Ion permeation of AQP6 water channel protein. Single channel recordings after Hg2+ activation. J Biol Chem (2002) 1.06
Epac1 mediates protein kinase A-independent mechanism of forskolin-activated intestinal chloride secretion. J Gen Physiol (2010) 1.06
Gout-causing Q141K mutation in ABCG2 leads to instability of the nucleotide-binding domain and can be corrected with small molecules. Proc Natl Acad Sci U S A (2013) 1.05
CHOP transcription factor mediates IL-8 signaling in cystic fibrosis bronchial epithelial cells. Am J Respir Cell Mol Biol (2007) 1.05
Regulation of cystic fibrosis transmembrane regulator trafficking and protein expression by a Rho family small GTPase TC10. J Biol Chem (2004) 1.04
Safety and tolerability of denufosol tetrasodium inhalation solution, a novel P2Y2 receptor agonist: results of a phase 1/phase 2 multicenter study in mild to moderate cystic fibrosis. Pediatr Pulmonol (2005) 1.04
Chloride channels in the kidney: lessons learned from knockout animals. Am J Physiol Renal Physiol (2002) 1.04
Early eradication of Pseudomonas aeruginosa in patients with cystic fibrosis. Paediatr Respir Rev (2010) 1.03
Gene expression profile analysis of 4-phenylbutyrate treatment of IB3-1 bronchial epithelial cell line demonstrates a major influence on heat-shock proteins. Physiol Genomics (2004) 1.02
Modulation of deltaF508 cystic fibrosis transmembrane regulator trafficking and function with 4-phenylbutyrate and flavonoids. Am J Respir Cell Mol Biol (2004) 1.02
Variants in mannose-binding lectin and tumour necrosis factor alpha affect survival in cystic fibrosis. J Med Genet (2006) 1.02
The loss of the chloride channel, ClC-5, delays apical iodide efflux and induces a euthyroid goiter in the mouse thyroid gland. Endocrinology (2005) 1.02
Identification of a polycystin-1 cleavage product, P100, that regulates store operated Ca entry through interactions with STIM1. PLoS One (2010) 1.01
Dual reporter comparative indexing of rAAV pseudotyped vectors in chimpanzee airway. Mol Ther (2009) 1.01
Deposition and expression of aerosolized rAAV vectors in the lungs of Rhesus macaques. Mol Ther (2002) 1.00
De novo biosynthetic profiling of high abundance proteins in cystic fibrosis lung epithelial cells. Mol Cell Proteomics (2006) 1.00
Protein microarray platforms for clinical proteomics. Proteomics Clin Appl (2007) 1.00
A phase I trial of intranasal Moli1901 for cystic fibrosis. Chest (2004) 1.00
Serum proteomic signature for cystic fibrosis using an antibody microarray platform. Mol Genet Metab (2006) 1.00
Atypical cystic fibrosis and CFTR-related diseases. Clin Rev Allergy Immunol (2008) 0.99
N-acetylcysteine enhances cystic fibrosis sputum penetration and airway gene transfer by highly compacted DNA nanoparticles. Mol Ther (2011) 0.98
Cystic fibrosis transmembrane regulator missing the first four transmembrane segments increases wild type and DeltaF508 processing. J Biol Chem (2008) 0.97
Albumin endocytosis in proximal tubule cells is modulated by angiotensin II through an AT2 receptor-mediated protein kinase B activation. Proc Natl Acad Sci U S A (2005) 0.97
Cofilin interacts with ClC-5 and regulates albumin uptake in proximal tubule cell lines. J Biol Chem (2003) 0.97
Polycystin-1 interacts with inositol 1,4,5-trisphosphate receptor to modulate intracellular Ca2+ signaling with implications for polycystic kidney disease. J Biol Chem (2009) 0.96
Biological Differences in rAAV Transduction of Airway Epithelia in Humans and in Old World Non-human Primates. Mol Ther (2007) 0.96
Multisite comparison of mucociliary and cough clearance measures using standardized methods. J Aerosol Med Pulm Drug Deliv (2013) 0.96
Hypoxia-induced mitogenic factor/FIZZ1 induces intracellular calcium release through the PLC-IP(3) pathway. Am J Physiol Lung Cell Mol Physiol (2009) 0.95
The risk, prevention, and outcome of cytomegalovirus after pediatric lung transplantation. Transplantation (2009) 0.94
The ClC-5 knockout mouse model of Dent's disease has renal hypercalciuria and increased bone turnover. J Bone Miner Res (2003) 0.94
Future directions in early cystic fibrosis lung disease research: an NHLBI workshop report. Am J Respir Crit Care Med (2012) 0.94
Determinants of AQP6 trafficking to intracellular sites versus the plasma membrane in transfected mammalian cells. Biol Cell (2006) 0.93
Ciprofloxacin-induced renal insufficiency in cystic fibrosis. J Cyst Fibros (2003) 0.92
Knockdown of NHERF1 enhances degradation of temperature rescued DeltaF508 CFTR from the cell surface of human airway cells. Cell Physiol Biochem (2007) 0.92
The cytoplasmic tail of large conductance, voltage- and Ca2+-activated K+ (MaxiK) channel is necessary for its cell surface expression. J Biol Chem (2002) 0.91
Spontaneous resolution of diffuse persistent pulmonary interstitial emphysema. Pediatr Pulmonol (2008) 0.91
Description of a standardized nutrition classification plan and its relation to nutritional outcomes in children with cystic fibrosis. J Pediatr Psychol (2009) 0.90
Successful transgene expression with serial doses of aerosolized rAAV2 vectors in rhesus macaques. Mol Ther (2003) 0.90
Polycystin-1, 2, and STIM1 interact with IP(3)R to modulate ER Ca release through the PI3K/Akt pathway. Cell Physiol Biochem (2011) 0.90
Cadmium impairs albumin reabsorption by down-regulating megalin and ClC5 channels in renal proximal tubule cells. Environ Health Perspect (2010) 0.90
Therapeutic approaches to repair defects in deltaF508 CFTR folding and cellular targeting. Adv Drug Deliv Rev (2002) 0.90