KCNA1 (Q09470) variants and mutations

KCNA1 (also known as Q09470) is a human protein-coding gene encoding a potassium voltage-gated channel subfamily A member 1 protein. Its potassium current limits neuronal excitability and shapes action-potential repolarization, especially in axons and presynaptic terminals. Pathogenic variants classically cause episodic ataxia type 1 and can also produce epilepsy, myokymia, and related neurologic phenotypes. This analysis covers 1,165 KCNA1 variants and mutations. Of these, 76% have computational variant effect predictions. Disease context includes episodic ataxia type 1, hereditary continuous muscle fiber activity, and multiple sclerosis. Example KCNA1 variants include M1L, M1R, and M1T.

Variant analysis overview

Variant and mutation evidence

Clinical, disease, and population context

Protein structure and variant hotspots

Data sources

Evidence in this analysis draws on EBI Proteins Variation, UniProt, gnomAD v4, EuropePMC, 3D Hotspot Analysis, Interaction Network Analysis, AlphaFold DB, gnomAD constraint, Open Targets, MaveDB, LitVar.

Notable KCNA1 variants

Examples include M1L, M1R, M1T, M1V, T2K, T2M, T2R, T2T. Listed records include available protein-change notation, database identifiers, clinical classifications, computational predictions, population evidence, experimental measurements, and disease context.