Severe hyperkalemia is rescued by low-potassium diet in renal βENaC-deficient mice

Boscardin, Emilie; Perrier, Romain; Sergi, Chloé; Maillard, Marc; Loffing, Johannes; Loffing-Cueni, Dominique; Koesters, Robert; Rossier, Bernard Claude; Hummler, Edith

doi:10.1007/s00424-017-1990-2

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Boscardin, E., Perrier, R., Sergi, C., Maillard, M., Loffing, J., Loffing-Cueni, D., Koesters, R., Rossier, B. C., & Hummler, E. (2017). Severe hyperkalemia is rescued by low-potassium diet in renal βENaC-deficient mice. Pflügers Archiv : European Journal of Physiology, 469(10), 1387–1399. https://doi.org/10.1007/s00424-017-1990-2

Abstract

In adulthood, an induced nephron-specific deficiency of αENaC (Scnn1a) resulted in pseudohypoaldosteronism type 1 (PHA-1) with sodium loss, hyperkalemia, and metabolic acidosis that is rescued through high-sodium/low-potassium (HNa+/LK+) diet. In the present study, we addressed whether renal βENaC expression is required for sodium and potassium balance or can be compensated by remaining (α and γ) ENaC subunits using adult nephron-specific knockout (Scnn1bPax8/LC1) mice. Upon induction, these mice present a severe PHA-1 phenotype with