Inward-rectifying potassium channelopathies: new insights into disorders of sodium and potassium homeostasis

Chih Jen Cheng, Chih Chien Sung, Chou Long Huang, Shih Hua Lin

Research output: Contribution to journalReview articlepeer-review

13 Scopus citations


Inward-rectifying potassium (Kir) channels allow more inward than outward potassium flux when channels are open in mammalian cells. At physiological resting membrane potentials, however, they predominantly mediate outward potassium flux and play important roles in regulating the resting membrane potential in diverse cell types and potassium secretion in the kidneys. Mutations of Kir channels cause human hereditary diseases collectively called Kir channelopathies, many of which are characterized by disorders of sodium and potassium homeostasis. Studies on these genetic Kir channelopathies have shed light on novel pathophysiological mechanisms, including renal sodium and potassium handling, potassium shifting in skeletal muscles, and aldosterone production in the adrenal glands. Here, we review several recent advances in Kir channels and their clinical implications in sodium and potassium homeostasis.

Original languageEnglish (US)
Pages (from-to)373-383
Number of pages11
JournalPediatric Nephrology
Issue number3
StatePublished - Mar 2014


  • Aldosterone-producing adenoma
  • Bartter’s syndrome
  • Hypokalemic periodic paralysis
  • Kir channels
  • Potassium
  • SeSAME syndrome
  • Sodium

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Nephrology


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