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*Institut de Pharmacologie et de Toxicologie de l’Université, Lausanne, Switzerland; INSERM U478, Institut Fédératif de Recherche 02, Faculté de Médecine Xavier Bichat, Paris, France; and Anatomisches Institut, Universität Zürich-Irchel, Zürich, Switzerland.
Correspondence to Dr. Bernard C. Rossier, Institut de pharmacologie et de toxicologie de l’Université, Rue du Bugnon 27, CH-1005 Lausanne, Switzerland. Phone: +4121/692-5351; Fax: +4121/692-5355;
ABSTRACT. The Liddle syndrome is a dominant form of salt-sensitive hypertension resulting from mutations in the 
or 
subunit of ENaC. A previous study established a mouse model carrying a premature Stop codon corresponding to the R566stop mutation (L) found in the original pedigree that recapitulates to a large extent the human disease. This study investigated the renal Na+ transport in vivo, ex vivo (intact perfused tubules), and in vitro (primary cultured cortical collecting ducts [CCD]). In vivo, upon 6 to 12 h of salt repletion, after 1 week of low-salt diet, the L/L mice showed a delayed urinary sodium excretion, despite a lower aldosterone secretion as compared with controls. After 6 h salt of repletion, ENaC subunit is rapidly removed from the apical plasma membrane in wild-type mice, whereas it is retained at the apical membrane in L/L mice. Ex vivo, isolated perfused CCD from L/L mice exhibited higher transepithelial potential differences than perfused CCD isolated from +/+ mice. In vitro, confluent primary cultures of CCD microdissected from L/L kidneys grown on permeable filters exhibited significant lower transepithelial electrical resistance and higher negative potential differences than their cultured L/+ and +/+ CCD counterparts. The equivalent short-circuit current (Ieq) and the amiloride-sensitive Ieq was approximately twofold higher in cultured L/L CCD than in +/+ CCD. Aldosterone (5 x 10-7M for 3 h) further increased Ieq from cultured L/L CCD. Thus, this study brings three independent lines of evidence for the constitutive hyperactivity of ENaC in CCD from mice harboring the Liddle mutation. e-mail: Bernard.Rossier@ipharm.unil.ch
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