ArticleThe Journal of physiology2007
beta-Liddle mutation of the epithelial sodium channel increases alveolar fluid clearance and reduces the severity of hydrostatic pulmonary oedema in mice.
Article in The Journal of physiology, 2007. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 17 papers, 1 of them a synthesis that pooled it.
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Who cites it
17 citing papers in PubMed, 1 synthesis or guideline pooled it, 31 citations in OpenAlex.
- Systematic review and meta-analysis of nasal potential difference in hypoxia-induced lung injury.Scientific reports · 2016Pooled it
- Suppression of Endoplasmic Reticulum Stress by 4-PBA Protects Against Hyperoxia-Induced Acute Lung InjuryFrontiers in immunology · 2021Article
- Cofilin participates in regulating alpha-epithelial sodium channel by interaction with 14-3-3 isoforms.Journal of biomedical research · 2020Article
- The function and regulation of acid-sensing ion channels (ASICs) and the epithelial Na(+) channel (ENaC): IUPHAR Review 19.British journal of pharmacology · 2016Review
- Regulation of epithelial sodium channels in urokinase plasminogen activator deficiency.American journal of physiology. Lung cellular and molecular physiology · 2014Article
- ENaCs and ASICs as therapeutic targets.American journal of physiology. Cell physiology · 2012Review
- Nedd4-2 and the regulation of epithelial sodium transport.Frontiers in physiology · 2012Article
- Enhancement of alveolar epithelial sodium channel activity with decreased cystic fibrosis transmembrane conductance regulator expression in mouse lung.American journal of physiology. Lung cellular and molecular physiology · 2011Article
- Amiloride-sensitive sodium channels and pulmonary edema.Pulmonary medicine · 2011Article
- Role of the ubiquitin system in regulating ion transport.Pflugers Archiv : European journal of physiology · 2011Article
- Respiratory distress and perinatal lethality in Nedd4-2-deficient mice.Nature communications · 2011Article
- Airway surface liquid volume regulation determines different airway phenotypes in liddle compared with betaENaC-overexpressing mice.The Journal of biological chemistry · 2010Article
- A mutation of the epithelial sodium channel associated with atypical cystic fibrosis increases channel open probability and reduces Na+ self inhibition.The Journal of physiology · 2010Article
- ENaC-mediated alveolar fluid clearance and lung fluid balance depend on the channel-activating protease 1.EMBO molecular medicine · 2010Article
- Effect of ventilation pressure on alveolar fluid clearance and beta-agonist responses in mice.American journal of physiology. Lung cellular and molecular physiology · 2009Article
- Article
- Aldosterone responsiveness of the epithelial sodium channel (ENaC) in colon is increased in a mouse model for Liddle's syndrome.The Journal of physiology · 2008Article
Corrections and comments
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Authors and funding
9 authors at 5 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Transepithelial sodium transport via alveolar epithelial Na(+) channels and Na(+),K(+)-ATPase constitutes the driving force for removal of alveolar oedema fluid. Decreased activity of the amiloride-sensitive epithelial Na(+) channel (ENaC) in the apical membrane of alveolar epithelial cells impairs sodium-driven alveolar fluid clearance (AFC) and predisposes to pulmonary oedema. We hypothesized that hyperactivity of ENaC in the distal lung could improve AFC and facilitate the resolution of pulmonary oedema. AFC and lung fluid balance were studied at baseline and under conditions of hydrostatic pulmonary oedema in the beta-Liddle (L) mouse strain harbouring a gain-of-function mutation (R(566)(stop)) within the Scnn1b gene. As compared with wild-type (+/+), baseline AFC was increased by 2- and 3-fold in heterozygous (+/L) and homozygous mutated (L/L) mice, respectively, mainly due to increased amiloride-sensitive AFC. The beta(2)-agonist terbutaline stimulated AFC in +/+ and +/L mice, but not in L/L mice. Acute volume overload induced by saline infusion (40% of body weight over 2 h) significantly increased extravascular (i.e. interstitial and alveolar) lung water as assessed by the bloodless wet-to-dry lung weight ratio in +/+ and L/L mice, as compared with baseline. However, the increase was significantly larger in +/+ than in L/L groups (P=0.01). Volume overload also increased the volume of the alveolar epithelial lining fluid in +/+ mice, indicating the presence of alveolar oedema, but not in L/L mice. Cardiac function as evaluated by echocardiography was comparable in both groups. These data show that constitutive ENaC activation improved sodium-driven AFC in the mouse lung, and attenuated the severity of hydrostatic pulmonary oedema.
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