The Kir4.1 channel mutations to human diseases are EAST syndrome (Bockenhauer et al., 2009) [16] and SeSAME syndrome (Scholl et al., 2009) [17] describing epilepsy, ataxia, sensorineural deafness, tubulopathy (EAST syndrome) and seizures, sensorineural deafness, ataxia mental retardation, and electrolyte imbalance (SeSAME syndrome) caused by mutations in KCNJ10 gene encoding Kir4.1 channels [16, 17]. Here, KCNJ10 is linked to cerebellar ataxia.