Due to the large amount of Na+ and the increased aldosterone, Na+ reabsorption is increased in the CS and, in parallel, K+ secretion is enhanced; (ii) in EAST/SeSAME syndrome, the inadequately high K+ secretion is worsened by a dysregulation of the CS itself, which is no longer able to properly adapt the apical transport of Na+ and K+ to dietary Na+ and K+ intake due to the lack of functioning Kir4.1 channels. Here, KCNJ10 is linked to EAST syndrome.