Mislocalization of K+ channels causes the renal salt wasting in EAST/SeSAME syndrome

Masayuki Tanemoto, Takaaki Abe, Shunya Uchida, Katsumasa Kawahara

Research output: Contribution to journalArticlepeer-review

12 Citations (Scopus)


The Kir4.1/Kir5.1 channel mediates basolateral K+ recycling in renal distal tubules; this process is critical for Na+ reabsorption at the tubules. Mutations in Kir4.1 are associated with EAST/SeSAME syndrome, a genetic disorder characterized by renal salt wasting. In this study, we found that MAGI-1 anchors Kir4.1 channels (Kir4.1 homomer and Kir4.1/Kir5.1 heteromer) and contributes to basolateral K+ recycling. The Kir4.1 A167V mutation associated with EAST/SeSAME syndrome caused mistrafficking of the mutant channels and inhibited their expression on the basolateral surface of tubular cells. These findings suggest mislocalization of the Kir4.1 channels contributes to renal salt wasting.

Original languageEnglish
Pages (from-to)899-905
Number of pages7
JournalFEBS Letters
Issue number6
Publication statusPublished - 2014 Mar 18


  • EAST/SeSAME syndrome
  • Epithelial sodium transport
  • Intracellular trafficking
  • Kidney
  • Potassium channel
  • Tubulopathy

ASJC Scopus subject areas

  • Biophysics
  • Structural Biology
  • Biochemistry
  • Molecular Biology
  • Genetics
  • Cell Biology


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