The neurotransmitter GABA (γ-aminobutyric acid) inhibits the activity of signal-receiving neurons by interacting with the GABAA receptor on these cells.1 There are two major types of GABA receptors: the ionotropic GABAA (GABAA R) and the metabotropic GABAB receptors. nGABAA R belongs to the ligand gated ion channel superfamily.It is a heteropentamer, with all of its five subunits contributing to the pore formation. To date, eight subunit isoforms were cloned: α, β, γ, δ, ε, π, θ, and ρ.The native GABAA receptor, in most cases, consists of 2α, 2β and 1γ subunit. Three γ subunits genes have been identified in mammals. The binding of GABA to its GABAA receptor results in conformational changes that open a Cl- channel, producing an increase in membrane conductance, resulting in inhibition of neural activity.nRecently, a genetic linkage between familial epilepsy syndrome and mutations in the γ2 subunit of the GABAA receptor have been demonstrated.