KCNA4 encodes a voltage-gated potassium channel (Kv1.4) that mediates transmembrane potassium transport in excitable membranes by forming tetrameric channels that open in response to membrane depolarization and rapidly inactivate 12. The channel can assemble as homotetramers or heterotetramers with other Kv1 family members (KCNA1, KCNA2, KCNA5), with channel properties depending on subunit composition 1. KCNA4 is essential for regulating action potential duration in cardiac tissue 3. Expression is regulated post-transcriptionally, with miR-448 binding its 3'-untranslated region to suppress KCNA4 expression during ischemia, thereby modulating arrhythmic risk 3. Pathogenic variants in KCNA4 cause disease through loss-of-function mechanisms. De novo missense mutations (e.g., V558L in the selectivity filter/S6 hinge) cause early-onset developmental epileptic encephalopathy with severe reduction in channel current 4. An autosomal recessive missense variant (R89Q) associates with a syndrome characterized by congenital cataracts, abnormal striatum, intellectual disability, and attention deficit hyperactivity disorder, with KCNA4 expressed in brain, lens, and retina 5. Beyond neurological disease, KCNA4 variants show association with stimulant dependence in genome-wide studies 6 and endurance running performance 7, suggesting broader roles in neurological and metabolic function.