KCNJ10 encodes Kir4.1, an inwardly rectifying potassium channel critical for cellular potassium homeostasis in multiple tissues. The channel primarily functions in potassium buffering by glial cells in the brain and potassium recycling in renal distal tubules 1. In the kidney, KCNJ10 localizes to the basolateral membrane of distal convoluted tubules, connecting tubules, and cortical collecting ducts, where it mediates potassium recycling essential for sodium reabsorption 1. In the nervous system, KCNJ10 is expressed in satellite glial cells of dorsal root ganglia and astrocytes, where it regulates neuronal excitability through potassium homeostasis 2 3. Loss-of-function mutations cause EAST syndrome, characterized by epilepsy, ataxia, sensorineural deafness, and renal tubulopathy, demonstrating the channel's critical role in brain and kidney function 1. Recent studies have identified heterozygous KCNJ10 variants as a cause of paroxysmal kinesigenic dyskinesia through haploinsufficiency mechanisms, expanding the phenotypic spectrum beyond classical EAST syndrome 4 5. Additionally, variants show association with seizure susceptibility, with certain alleles conferring resistance to epilepsy 6. The channel represents a potential therapeutic target for depression and neurological disorders involving glial dysfunction 3.