Bartter's, Gitelman's, and Gordon's syndromes. From physiology to molecular biology and back, yet still some unanswered questions

Nephron. 2002;92 Suppl 1:18-27. doi: 10.1159/000065373.


The molecular basis of many of the inherited disorders of potassium homeostasis has become much clearer in the last two decades. Despite these new insights into the physiology of renal potassium handling, a number of questions remain to be answered. The examples we use to illustrate these issues are Gordon's syndrome, Bartter's syndrome, and Gitelman's syndrome. Our objective is to integrate these new insights into an understanding of the pathophysiology of renal potassium handling. We also propose different ways to think about some of the unresolved issues in this area.

Publication types

  • Review

MeSH terms

  • Aldosterone / metabolism
  • Bartter Syndrome / genetics
  • Bartter Syndrome / metabolism*
  • Bartter Syndrome / physiopathology
  • Calcium / metabolism
  • Chlorides / metabolism
  • Homeostasis
  • Humans
  • Ion Transport
  • Kidney Tubules, Collecting / metabolism*
  • Magnesium / metabolism
  • Models, Biological
  • Potassium / metabolism*
  • Renal Tubular Transport, Inborn Errors / genetics
  • Renal Tubular Transport, Inborn Errors / metabolism*
  • Renal Tubular Transport, Inborn Errors / physiopathology
  • Sodium / metabolism
  • Syndrome


  • Chlorides
  • Aldosterone
  • Sodium
  • Magnesium
  • Potassium
  • Calcium