Published in J Biol Chem on July 26, 2006
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
Reduced histone deacetylase 7 activity restores function to misfolded CFTR in cystic fibrosis. Nat Chem Biol (2009) 2.70
ENaC at the cutting edge: regulation of epithelial sodium channels by proteases. J Biol Chem (2009) 2.57
Loss of anion transport without increased sodium absorption characterizes newborn porcine cystic fibrosis airway epithelia. Cell (2010) 2.24
Epithelial integrity is maintained by a matriptase-dependent proteolytic pathway. Am J Pathol (2009) 1.62
SPLUNC1 regulates airway surface liquid volume by protecting ENaC from proteolytic cleavage. Proc Natl Acad Sci U S A (2009) 1.61
A segment of gamma ENaC mediates elastase activation of Na+ transport. J Gen Physiol (2007) 1.57
Regulation of cell surface protease matriptase by HAI2 is essential for placental development, neural tube closure and embryonic survival in mice. Development (2009) 1.38
Liquid movement across the surface epithelium of large airways. Respir Physiol Neurobiol (2007) 1.28
Constraint-based, homology model of the extracellular domain of the epithelial Na+ channel α subunit reveals a mechanism of channel activation by proteases. J Biol Chem (2010) 1.23
Prostasin expression is regulated by airway surface liquid volume and is increased in cystic fibrosis. Am J Physiol Lung Cell Mol Physiol (2008) 1.21
ENaC proteolytic regulation by channel-activating protease 2. J Gen Physiol (2008) 1.20
Acute regulation of the epithelial sodium channel in airway epithelia by proteases and trafficking. Am J Respir Cell Mol Biol (2010) 1.18
Mutations in SPINT2 cause a syndromic form of congenital sodium diarrhea. Am J Hum Genet (2009) 1.15
Regulation of the epithelial sodium channel by phosphatidylinositides: experiments, implications, and speculations. Pflugers Arch (2007) 1.10
Regulation of the epithelial Na+ channel and airway surface liquid volume by serine proteases. Pflugers Arch (2010) 1.08
Epithelial Na(+) channel regulation by cytoplasmic and extracellular factors. Exp Cell Res (2012) 1.08
Defining an inhibitory domain in the gamma subunit of the epithelial sodium channel. Am J Physiol Renal Physiol (2010) 1.06
Does epithelial sodium channel hyperactivity contribute to cystic fibrosis lung disease? J Physiol (2013) 1.05
Airway surface liquid volume regulation determines different airway phenotypes in liddle compared with betaENaC-overexpressing mice. J Biol Chem (2010) 1.05
Measurement of the airway surface liquid volume with simple light refraction microscopy. Am J Respir Cell Mol Biol (2011) 1.04
Regulation of endogenous ENaC functional expression by CFTR and ΔF508-CFTR in airway epithelial cells. Am J Physiol Lung Cell Mol Physiol (2010) 1.03
Interleukin-17A induces bicarbonate secretion in normal human bronchial epithelial cells. Am J Physiol Lung Cell Mol Physiol (2008) 1.01
A mutation of the epithelial sodium channel associated with atypical cystic fibrosis increases channel open probability and reduces Na+ self inhibition. J Physiol (2010) 1.01
TMPRSS4-dependent activation of the epithelial sodium channel requires cleavage of the γ-subunit distal to the furin cleavage site. Am J Physiol Renal Physiol (2011) 1.00
Epidermal growth factor-mediated proliferation and sodium transport in normal and PKD epithelial cells. Biochim Biophys Acta (2010) 1.00
SPLUNC1 expression reduces surface levels of the epithelial sodium channel (ENaC) in Xenopus laevis oocytes. Channels (Austin) (2010) 0.97
Activation of the epithelial sodium channel (ENaC) by the alkaline protease from Pseudomonas aeruginosa. J Biol Chem (2012) 0.94
Cleavage of endogenous γENaC and elevated abundance of αENaC are associated with increased Na⁺ transport in response to apical fluid volume expansion in human H441 airway epithelial cells. Pflugers Arch (2011) 0.91
The epithelial sodium channel (ENaC) establishes a trafficking vesicle pool responsible for its regulation. PLoS One (2012) 0.89
Lipopolysaccharide modifies amiloride-sensitive Na+ transport processes across human airway cells: role of mitogen-activated protein kinases ERK 1/2 and 5. Pflugers Arch (2009) 0.89
Cr(VI)-stimulated STAT3 tyrosine phosphorylation and nuclear translocation in human airway epithelial cells requires Lck. Biochem J (2007) 0.89
Regulation of epithelial sodium channel trafficking by proprotein convertase subtilisin/kexin type 9 (PCSK9). J Biol Chem (2012) 0.86
Liquid hyper-absorption as a cause of increased DTPA clearance in the cystic fibrosis airway. EJNMMI Res (2013) 0.85
Modulation of the epithelial sodium channel (ENaC) by bacterial metalloproteases and protease inhibitors. PLoS One (2014) 0.85
Proteases, cystic fibrosis and the epithelial sodium channel (ENaC). Cell Tissue Res (2012) 0.85
Epithelial Na⁺ channel activity in human airway epithelial cells: the role of serum and glucocorticoid-inducible kinase 1. Br J Pharmacol (2012) 0.80
Correlation of apical fluid-regulating channel proteins with lung function in human COPD lungs. PLoS One (2014) 0.79
Effect of apical hyperosmotic sodium challenge and amiloride on sodium transport in human bronchial epithelial cells from cystic fibrosis donors. Am J Physiol Cell Physiol (2013) 0.79
Status of fluid and electrolyte absorption in cystic fibrosis. Cold Spring Harb Perspect Med (2013) 0.78
Effect of neutrophil elastase and its inhibitor EPI-hNE4 on transepithelial sodium transport across normal and cystic fibrosis human nasal epithelial cells. Respir Res (2010) 0.78
New pulmonary therapies directed at targets other than CFTR. Cold Spring Harb Perspect Med (2013) 0.78
Oxidized glutathione (GSSG) inhibits epithelial sodium channel activity in primary alveolar epithelial cells. Am J Physiol Lung Cell Mol Physiol (2015) 0.78
Proteolytic activation of the epithelial sodium channel and therapeutic application of a serine protease inhibitor for the treatment of salt-sensitive hypertension. Clin Exp Nephrol (2011) 0.77
Intracellular Na+ regulates epithelial Na+ channel maturation. J Biol Chem (2015) 0.77
A mechanochemical model for auto-regulation of lung airway surface layer volume. J Theor Biol (2013) 0.76
Inhibition of airway surface fluid absorption by cholinergic stimulation. Sci Rep (2016) 0.76
Activation of a latent nuclear localization signal in the NH2 terminus of γ-ENaC initiates feedback regulation of channel activity. Am J Physiol Renal Physiol (2010) 0.75
Airway surface liquid volume expansion induces rapid changes in amiloride-sensitive Na+ transport across upper airway epithelium-Implications concerning the resolution of pulmonary edema. Physiol Rep (2015) 0.75
Prostasin: An Epithelial Sodium Channel Regulator. J Biomark (2013) 0.75
Hypotonic shock modulates Na(+) current via a Cl(-) and Ca(2+)/calmodulin dependent mechanism in alveolar epithelial cells. PLoS One (2013) 0.75
SPX-101 is a Promising and Novel Nebulized ENaC Inhibitor. Am J Respir Crit Care Med (2017) 0.75
IL-22 mediates mucosal host defense against Gram-negative bacterial pneumonia. Nat Med (2008) 9.05
Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation. N Engl J Med (2010) 6.23
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
A critical role for eosinophils in allergic airways remodeling. Science (2004) 5.53
Epithelial sodium channels are activated by furin-dependent proteolysis. J Biol Chem (2004) 3.36
Epithelial Na+ channels are fully activated by furin- and prostasin-dependent release of an inhibitory peptide from the gamma-subunit. J Biol Chem (2007) 3.09
Results of a phase IIa study of VX-809, an investigational CFTR corrector compound, in subjects with cystic fibrosis homozygous for the F508del-CFTR mutation. Thorax (2011) 2.84
Reduced histone deacetylase 7 activity restores function to misfolded CFTR in cystic fibrosis. Nat Chem Biol (2009) 2.70
Egr-1 regulates autophagy in cigarette smoke-induced chronic obstructive pulmonary disease. PLoS One (2008) 2.67
Comprehensive gene expression profiles reveal pathways related to the pathogenesis of chronic obstructive pulmonary disease. Proc Natl Acad Sci U S A (2004) 2.42
Maturation of the epithelial Na+ channel involves proteolytic processing of the alpha- and gamma-subunits. J Biol Chem (2003) 2.37
Autoantibodies in patients with chronic obstructive pulmonary disease. Am J Respir Crit Care Med (2007) 1.94
Distinct pools of epithelial sodium channels are expressed at the plasma membrane. J Biol Chem (2004) 1.87
Plasmin activates epithelial Na+ channels by cleaving the gamma subunit. J Biol Chem (2008) 1.86
Palliative care and location of death in decedents with idiopathic pulmonary fibrosis. Chest (2015) 1.85
Furin cleavage activates the epithelial Na+ channel by relieving Na+ self-inhibition. Am J Physiol Renal Physiol (2006) 1.82
The epithelial Na+ channel is inhibited by a peptide derived from proteolytic processing of its alpha subunit. J Biol Chem (2006) 1.80
Proteolytic processing of the epithelial sodium channel gamma subunit has a dominant role in channel activation. J Biol Chem (2008) 1.79
Patients with idiopathic pulmonary fibrosis with antibodies to heat shock protein 70 have poor prognoses. Am J Respir Crit Care Med (2013) 1.77
Acute ENaC stimulation by cAMP in a kidney cell line is mediated by exocytic insertion from a recycling channel pool. J Gen Physiol (2005) 1.70
Epithelial sodium channel inhibition by AMP-activated protein kinase in oocytes and polarized renal epithelial cells. J Biol Chem (2005) 1.70
Activated CD47 promotes pulmonary arterial hypertension through targeting caveolin-1. Cardiovasc Res (2012) 1.69
Derlin-1 promotes the efficient degradation of the cystic fibrosis transmembrane conductance regulator (CFTR) and CFTR folding mutants. J Biol Chem (2006) 1.69
The role of regulated CFTR trafficking in epithelial secretion. Am J Physiol Cell Physiol (2003) 1.55
Alternatively spliced proline-rich cassettes link WNK1 to aldosterone action. J Clin Invest (2015) 1.53
ENaC structure and function in the wake of a resolved structure of a family member. Am J Physiol Renal Physiol (2011) 1.44
SLC26A9 is a constitutively active, CFTR-regulated anion conductance in human bronchial epithelia. J Gen Physiol (2009) 1.44
The deubiquitinating enzyme UCH-L3 regulates the apical membrane recycling of the epithelial sodium channel. J Biol Chem (2007) 1.43
Rab11b regulates the apical recycling of the cystic fibrosis transmembrane conductance regulator in polarized intestinal epithelial cells. Mol Biol Cell (2009) 1.41
Regulation of the epithelial sodium channel by membrane trafficking. Am J Physiol Renal Physiol (2008) 1.41
Cellular and humoral autoreactivity in idiopathic pulmonary fibrosis. J Immunol (2007) 1.39
Differential effects of Hsc70 and Hsp70 on the intracellular trafficking and functional expression of epithelial sodium channels. Proc Natl Acad Sci U S A (2006) 1.39
High mortality rates among solid organ transplant recipients infected with extensively drug-resistant Acinetobacter baumannii: using in vitro antibiotic combination testing to identify the combination of a carbapenem and colistin as an effective treatment regimen. Diagn Microbiol Infect Dis (2011) 1.38
Regulation of cation transport in the distal nephron by mechanical forces. Am J Physiol Renal Physiol (2006) 1.37
Role of proteolysis in the activation of epithelial sodium channels. Curr Opin Nephrol Hypertens (2007) 1.36
Epithelial Na+ channels are activated by laminar shear stress. J Biol Chem (2003) 1.34
Ca2+ dependence of flow-stimulated K secretion in the mammalian cortical collecting duct. Am J Physiol Renal Physiol (2007) 1.34
Lung tissues in patients with systemic sclerosis have gene expression patterns unique to pulmonary fibrosis and pulmonary hypertension. Arthritis Rheum (2011) 1.33
Extracellular histidine residues crucial for Na+ self-inhibition of epithelial Na+ channels. J Biol Chem (2003) 1.33
Dietary K+ regulates apical membrane expression of maxi-K channels in rabbit cortical collecting duct. Am J Physiol Renal Physiol (2005) 1.29
AMPK agonists ameliorate sodium and fluid transport and inflammation in cystic fibrosis airway epithelial cells. Am J Respir Cell Mol Biol (2009) 1.27
Secreted and transmembrane mucins inhibit gene transfer with AAV4 more efficiently than AAV5. J Biol Chem (2002) 1.27
Clathrin-mediated endocytosis of the epithelial sodium channel. Role of epsin. J Biol Chem (2006) 1.27
Vitamin D3 attenuates Th2 responses to Aspergillus fumigatus mounted by CD4+ T cells from cystic fibrosis patients with allergic bronchopulmonary aspergillosis. J Clin Invest (2010) 1.27
Small heat-shock proteins select deltaF508-CFTR for endoplasmic reticulum-associated degradation. Mol Biol Cell (2006) 1.24
Constraint-based, homology model of the extracellular domain of the epithelial Na+ channel α subunit reveals a mechanism of channel activation by proteases. J Biol Chem (2010) 1.23
Prostasin expression is regulated by airway surface liquid volume and is increased in cystic fibrosis. Am J Physiol Lung Cell Mol Physiol (2008) 1.21
Role of epithelial sodium channels and their regulators in hypertension. J Biol Chem (2010) 1.20
Novel determinants of epithelial sodium channel gating within extracellular thumb domains. J Biol Chem (2009) 1.20
Mechanism underlying flow stimulation of sodium absorption in the mammalian collecting duct. Am J Physiol Renal Physiol (2006) 1.20
Ontogeny of flow-stimulated potassium secretion in rabbit cortical collecting duct: functional and molecular aspects. Am J Physiol Renal Physiol (2003) 1.18
Acute regulation of the epithelial sodium channel in airway epithelia by proteases and trafficking. Am J Respir Cell Mol Biol (2010) 1.18
Chaperone displacement from mutant cystic fibrosis transmembrane conductance regulator restores its function in human airway epithelia. FASEB J (2008) 1.17
The endoplasmic reticulum-associated degradation of the epithelial sodium channel requires a unique complement of molecular chaperones. Mol Biol Cell (2010) 1.17
Insulin-like growth factor-binding protein-5 induces pulmonary fibrosis and triggers mononuclear cellular infiltration. Am J Pathol (2006) 1.17
Arachidonic acid regulates surface expression of epithelial sodium channels. J Biol Chem (2003) 1.15
Effect of aldosterone on BK channel expression in mammalian cortical collecting duct. Am J Physiol Renal Physiol (2008) 1.15
c-Cbl facilitates endocytosis and lysosomal degradation of cystic fibrosis transmembrane conductance regulator in human airway epithelial cells. J Biol Chem (2010) 1.15
Defining an inhibitory domain in the alpha-subunit of the epithelial sodium channel. Am J Physiol Renal Physiol (2007) 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
Posaconazole serum concentrations among cardiothoracic transplant recipients: factors impacting trough levels and correlation with clinical response to therapy. Antimicrob Agents Chemother (2010) 1.15
External nickel inhibits epithelial sodium channel by binding to histidine residues within the extracellular domains of alpha and gamma subunits and reducing channel open probability. J Biol Chem (2002) 1.13
Molecular and functional properties of lung SP cells. Am J Physiol Lung Cell Mol Physiol (2006) 1.12
Regulation of epithelial Na+ transport by soluble adenylyl cyclase in kidney collecting duct cells. J Biol Chem (2009) 1.12
An obligatory heterodimer of 14-3-3beta and 14-3-3epsilon is required for aldosterone regulation of the epithelial sodium channel. J Biol Chem (2008) 1.12
Syntaxin 1A regulates ENaC channel activity. J Biol Chem (2003) 1.11
Cysteine string protein interacts with and modulates the maturation of the cystic fibrosis transmembrane conductance regulator. J Biol Chem (2002) 1.11
Prospective, observational study of voriconazole therapeutic drug monitoring among lung transplant recipients receiving prophylaxis: factors impacting levels of and associations between serum troughs, efficacy, and toxicity. Antimicrob Agents Chemother (2012) 1.11
14-3-3 isoforms are induced by aldosterone and participate in its regulation of epithelial sodium channels. J Biol Chem (2006) 1.11
Functional role of extracellular loop cysteine residues of the epithelial Na+ channel in Na+ self-inhibition. J Biol Chem (2007) 1.10
Extracellular Zn2+ activates epithelial Na+ channels by eliminating Na+ self-inhibition. J Biol Chem (2004) 1.10
Mutations in the pore region modify epithelial sodium channel gating by shear stress. J Biol Chem (2004) 1.09
Epithelial Na(+) channel regulation by cytoplasmic and extracellular factors. Exp Cell Res (2012) 1.08
Allosteric inhibition of the epithelial Na+ channel through peptide binding at peripheral finger and thumb domains. J Biol Chem (2010) 1.08
Base of the thumb domain modulates epithelial sodium channel gating. J Biol Chem (2011) 1.07
Defining an inhibitory domain in the gamma subunit of the epithelial sodium channel. Am J Physiol Renal Physiol (2010) 1.06
Regulatory R region of the CFTR chloride channel is a dynamic integrator of phospho-dependent intra- and intermolecular interactions. Proc Natl Acad Sci U S A (2013) 1.06
CX3CL1 up-regulation is associated with recruitment of CX3CR1+ mononuclear phagocytes and T lymphocytes in the lungs during cigarette smoke-induced emphysema. Am J Pathol (2008) 1.06
The epithelial sodium channel (ENaC) traffics to apical membrane in lipid rafts in mouse cortical collecting duct cells. J Biol Chem (2007) 1.05