Surprisingly, however, Teulon and colleagues (Paulais et al., 2011) had found in a Kir5.1 knockout mouse that although the animals also had hypokalemia, their remaining phenotypes were opposite to EAST/SeSAME syndrome: instead of alkalosis, metabolic acidosis was found, and instead of hypocalciuria, hypercalciuria was observed. Here, KCNJ16 is linked to Hypokalemia.