KCNC2 encodes Kv3.2, a voltage-gated potassium channel crucial for neuronal excitability and brain function. The primary function involves mediating delayed-rectifier potassium currents that enable sustained high-frequency firing in GABAergic interneurons, particularly fast-spiking parvalbumin-positive cortical interneurons 1 2. The channel operates through voltage-dependent activation and slow inactivation, forming homomeric or heteromeric tetramers that regulate action potential repolarization and maintain excitation-inhibition balance in neural circuits 1 3. KCNC2 variants are increasingly recognized as causative for developmental and epileptic encephalopathy (DEE), with both gain-of-function and loss-of-function mechanisms identified 1 3. De novo variants cause severe phenotypes including early-onset seizures, developmental delay, and spastic tetraplegia 4 5 2. Functional studies demonstrate that pathogenic variants alter channel kinetics, voltage dependence, and current density, ultimately impairing interneuron firing frequency and disrupting cortical inhibition 3 2. Clinically, valproic acid shows therapeutic efficacy in patients with KCNC2-related epilepsy, achieving seizure freedom in several cases, though the mechanism remains unclear 1 6. Additionally, KCNC2 variants may contribute to metabolic disorders, with associations reported for childhood obesity and diabetes risk 7.