Early death due to defective neonatal lung liquid clearance in alpha-ENaC-deficient mice

Détails

ID Serval
serval:BIB_483D56DAA2EF
Type
Article: article d'un périodique ou d'un magazine.
Collection
Publications
Titre
Early death due to defective neonatal lung liquid clearance in alpha-ENaC-deficient mice
Périodique
Nature Genetics
Auteur(s)
Hummler E., Barker P., Gatzy J., Beermann F., Verdumo C., Schmidt A., Boucher R., Rossier B. C.
ISSN
1061-4036
Statut éditorial
Publié
Date de publication
03/1996
Peer-reviewed
Oui
Volume
12
Numéro
3
Pages
325-28
Notes
Journal Article Research Support, Non-U.S. Gov't Research Support, U.S. Gov't, P.H.S. --- Old month value: Mar
Résumé
The amiloride-sensitive epithelial sodium channel, ENaC, is a heteromultimeric protein made up of three homologous subunits (alpha, beta and gamma) (1,2). In vitro, assembly and expression of functional active sodium channels in the Xenopus oocyte is strictly dependent on alpha-ENaC--the beta and gamma subunits by themselves are unable to induce an amiloride-sensitive sodium current in this heterologous expression system (2). In vivo, ENaC constitutes the limiting step for sodium absorption in epithelial cells that line the distal renal tubule, distal colon and the duct of several exocrine glands. The adult lung expresses alpha, beta and gamma ENaC (3,4), and an amiloride-sensitive electrogenic sodium reabsorption has been documented in upper and lower airways (3-7), but it is not established whether this sodium transport is mediated by ENaC in vivo. We inactivated the mouse alpha-ENaC gene by gene targeting. Amiloride-sensitive electrogenic Na+ transport was abolished in airway epithelia from alpha-ENaC(-/-) mice. Alpha-ENaC(-/-) neonates developed respiratory distress and died within 40 h of birth from failure to clear their lungs of liquid. This study shows that ENaC plays a critical role in the adaptation of the newborn lung to air breathing.
Mots-clé
Animals Animals, Newborn Base Sequence Body Fluids/metabolism Culture Techniques DNA Primers Epithelial Sodium Channel Gene Targeting Ion Transport Lung Diseases/*metabolism/mortality Mice Molecular Sequence Data Sodium/metabolism Sodium Channels/*deficiency/genetics/physiology
Pubmed
Web of science
Création de la notice
24/01/2008 14:01
Dernière modification de la notice
20/08/2019 14:55
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