Potassium-related inherited tubulopathies

D. Landau

Research output: Contribution to journalReview articlepeer-review

26 Scopus citations

Abstract

Hyper- and hypokalemia may carry severe clinical consequences. Different regulatory mechanisms, including the kidney, exert a tight regulation of plasma potassium levels. The renal pathway of potassium handling begins in the proximal tubule followed by the fine-tuning of its secretion or absorption at the distal tubule, including the thick ascending limb of Henle's loop, the distal convoluted tubule and the cortical collecting duct. Genetic studies in recent years have clarified the role of specific tubular channels and transporters in the pathogenesis of unique hyper- and hypokalemic tubulopathies, some of them non-hypertensive (pseudohypoaldosteronism, Bartter and Gitelman syndromes) and others hypertensive by definition (including Liddle and Gordon syndromes). This article reviews the genetic and clinical spectrum of hypokalemic and hyperkalemic tubulopathies.

Original languageEnglish
Pages (from-to)1962-1968
Number of pages7
JournalCellular and Molecular Life Sciences
Volume63
Issue number17
DOIs
StatePublished - 1 Sep 2006
Externally publishedYes

Keywords

  • Aldosterone
  • Bartter syndrome
  • Gitelman syndrome
  • Gordon syndrome
  • Liddle syndrome
  • Loop of Henle

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