GABRA1 (P14867) variants and mutations

GABRA1 (also known as P14867) is a human protein-coding gene encoding a gamma-aminobutyric acid receptor subunit alpha-1 protein. The gene product supplies the alpha-1 subunit of a pentameric GABA-A receptor, a ligand-gated chloride channel in the brain. GABA binding allows chloride influx that dampens neuronal activity, making this receptor important for inhibition and seizure biology. This analysis covers 928 GABRA1 variants and mutations. Of these, 52% have computational variant effect predictions. Disease context includes developmental and epileptic encephalopathy, 19, juvenile myoclonic epilepsy, and epilepsy. Example GABRA1 variants include M1?, R2K, and R2W.

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, Protein Data Bank, AlphaFold DB, gnomAD constraint, Open Targets, ClinGen, MaveDB, LitVar.

Notable GABRA1 variants

Examples include M1?, R2K, R2W, K3*, K3E, K3N, K3R, S4I. Listed records include available protein-change notation, database identifiers, clinical classifications, computational predictions, population evidence, experimental measurements, and disease context.