Mutations in the human KCNJ10 gene encoding Kir4.1 were reported to cause the epileptic disorders known as “EAST” (Epilepsy, Ataxia, Sensorineural deafness and Tubulopathy) and “SeSAME” (Seizures, Sensorineural deafness, Ataxia, Mental retardation, and Electrolyte imbalance) syndrome (OMIM 612780) (51–53). Here, KCNJ10 is linked to Abnormality of metabolism/homeostasis.