Renal tubular NEDD4-2 deficiency causes NCC-mediated salt-dependent hypertension.

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Accès restreint UNIL
Etat: Public
Version: Final published version
ID Serval
serval:BIB_218B3682F156
Type
Article: article d'un périodique ou d'un magazine.
Collection
Publications
Institution
Titre
Renal tubular NEDD4-2 deficiency causes NCC-mediated salt-dependent hypertension.
Périodique
Journal of Clinical Investigation
Auteur⸱e⸱s
Ronzaud C., Loffing-Cueni D., Hausel P., Debonneville A., Malsure S.R., Fowler-Jaeger N., Boase N.A., Perrier R., Maillard M., Yang B., Stokes J.B., Koesters R., Kumar S., Hummler E., Loffing J., Staub O.
ISSN
1558-8238 (Electronic)
ISSN-L
0021-9738
Statut éditorial
Publié
Date de publication
2013
Volume
123
Numéro
2
Pages
657-665
Langue
anglais
Notes
Publication types: Journal Article ; Research Support, Non-U.S. Gov't Publication Status: ppublish. PDF type: Research article
Résumé
The E3 ubiquitin ligase NEDD4-2 (encoded by the Nedd4L gene) regulates the amiloride-sensitive epithelial Na+ channel (ENaC/SCNN1) to mediate Na+ homeostasis. Mutations in the human β/γENaC subunits that block NEDD4-2 binding or constitutive ablation of exons 6-8 of Nedd4L in mice both result in salt-sensitive hypertension and elevated ENaC activity (Liddle syndrome). To determine the role of renal tubular NEDD4-2 in adult mice, we generated tetracycline-inducible, nephron-specific Nedd4L KO mice. Under standard and high-Na+ diets, conditional KO mice displayed decreased plasma aldosterone but normal Na+/K+ balance. Under a high-Na+ diet, KO mice exhibited hypercalciuria and increased blood pressure, which were reversed by thiazide treatment. Protein expression of βENaC, γENaC, the renal outer medullary K+ channel (ROMK), and total and phosphorylated thiazide-sensitive Na+Cl- cotransporter (NCC) levels were increased in KO kidneys. Unexpectedly, Scnn1a mRNA, which encodes the αENaC subunit, was reduced and proteolytic cleavage of αENaC decreased. Taken together, these results demonstrate that loss of NEDD4-2 in adult renal tubules causes a new form of mild, salt-sensitive hypertension without hyperkalemia that is characterized by upregulation of NCC, elevation of β/γENaC, but not αENaC, and a normal Na+/K+ balance maintained by downregulation of ENaC activity and upregulation of ROMK.
Mots-clé
Animals, Blood Pressure, Disease Models, Animal, Endosomal Sorting Complexes Required for Transport/deficiency, Endosomal Sorting Complexes Required for Transport/genetics, Epithelial Sodium Channels/metabolism, Humans, Hypertension/etiology, Hypertension/genetics, Kidney Tubules/physiopathology, Liddle Syndrome/etiology, Liddle Syndrome/genetics, Mice, Mice, Knockout, Potassium/blood, Potassium/urine, Potassium Channels, Inwardly Rectifying/metabolism, Receptors, Drug/metabolism, Sodium/blood, Sodium/urine, Sodium, Dietary/administration & dosage, Sodium, Dietary/adverse effects, Symporters/metabolism, Ubiquitin-Protein Ligases/deficiency, Ubiquitin-Protein Ligases/genetics
Pubmed
Web of science
Open Access
Oui
Création de la notice
30/01/2013 14:11
Dernière modification de la notice
20/10/2020 11:08
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