The GABA A R is the major inhibitory neurotransmitter receptor in the central nervous system (CNS) (25, 26) with important roles in development (27, 28) and disease (29) (30) (31) . The receptor is believed to be a pentamer made up of multiple subunits that come from at least four different subunit classes (a, b, g and d) (32) . At least 19 genes code for the various subunits that differentially combine to form numerous pharmacologically distinct GABA A receptor isoforms (29, 30) . Isoform utilization depends in part on the relative abundance of the subunits, which may change under various conditions (33) (34) (35) . Understanding subunit regulatory mechanisms may provide insight into GABA A receptor isoform usage and related phenotypes (36) .