{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T14:04:25Z","timestamp":1791295465130,"version":"4.1.0"},"reference-count":37,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2004,10,29]],"date-time":"2004-10-29T00:00:00Z","timestamp":1099008000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["The Journal of Physiology"],"published-print":{"date-parts":[[2004,11]]},"abstract":"<jats:p>Pulmonary oedema results from an imbalance between the forces driving fluid into the airspace and the biological mechanisms for its removal. In mice lacking the \u03b1\u2010subunit of the amiloride\u2010sensitive sodium channel (\u03b1ENaC(\u2212\/\u2212)), impaired sodium transport\u2010mediated lung liquid clearance at birth results in neonatal death. Transgenic expression of \u03b1ENaC driven by a cytomegalovirus (CMV) promoter (\u03b1ENaC(\u2212\/\u2212)Tg+) rescues the lethal pulmonary phenotype, but only partially restores respiratory sodium transport <jats:italic>in vitro<\/jats:italic>. To test whether this may also be true <jats:italic>in vivo<\/jats:italic>, and to assess the functional consequences of this defect on experimental pulmonary oedema, we measured respiratory transepithelial potential difference (PD) and alveolar fluid clearance (AFC), and quantified pulmonary oedema during experimental acute lung injury in these mice. Both respiratory PD and AFC were roughly 50% lower (<jats:italic>P<\/jats:italic> &lt; 0.01) in \u03b1ENaC(\u2212\/\u2212)Tg+ than in control mice. This impairment was associated with a significantly larger increase of the wet\/dry lung weight ratio in \u03b1ENaC(\u2212\/\u2212)Tg+ than in control mice, both after exposure to hyperoxia and thiourea. Moreover, the rate of resolution of thiourea\u2010induced pulmonary oedema was more than three times slower (<jats:italic>P<\/jats:italic> &lt; 0.001) in \u03b1ENaC(\u2212\/\u2212)Tg+ mice. \u03b1ENaC(\u2212\/\u2212)Tg+ mice represent the first model of a constitutively impaired respiratory transepithelial sodium transport, and provide direct evidence that this impairment facilitates pulmonary oedema in conscious freely moving animals. These data in mice strengthen indirect evidence provided by clinical studies, suggesting that defective respiratory transepithelial sodium transport may also facilitate pulmonary oedema in humans.<\/jats:p>","DOI":"10.1113\/jphysiol.2004.066704","type":"journal-article","created":{"date-parts":[[2004,8,13]],"date-time":"2004-08-13T00:13:03Z","timestamp":1092355983000},"page":"857-865","source":"Crossref","is-referenced-by-count":80,"title":["Defective respiratory amiloride\u2010sensitive sodium transport predisposes to pulmonary oedema and delays its resolution in 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