KCNA1 encodes Kv1.1, a voltage-gated potassium channel that mediates transmembrane potassium transport in the brain and central nervous system, with additional roles in the kidney. The channel regulates membrane potential and prevents neuronal hyperexcitability by opening in response to membrane depolarization. KCNA1 forms both homotetrameric and heterotetrameric channels with other KCNA family members, with channel properties modulated by cytoplasmic beta subunits that promote rapid inactivation. The channel is essential for normal neuromuscular responses, regulates neuronal excitability in the hippocampus, and contributes to magnesium ion homeostasis in the kidney. KCNA1 mutations cause multiple channelopathies with complex genotype-phenotype correlations. Episodic ataxia type 1 (EA1), the most common manifestation, is characterized by brief recurrent attacks of ataxia and dysarthria often triggered by physical exertion or emotional stress, sometimes with associated interictal myokymia 12. Mutations can also cause epilepsy, sometimes combined with EA1, or paroxysmal kinesigenic dyskinesia, with both gain- and loss-of-function variants identified 3. Epilepsy-associated variants tend to cluster in the pore domain and transmembrane regions, while EA1-associated variants are distributed throughout the protein 45. Treatment options include potassium channel blockers such as dalfampridine, 4-aminopyridine, and acetazolamide, which can reduce attack frequency and severity in many patients 1.