The GABRG2 nonsense mutation, Q40X, associated with Dravet syndrome activated NMD and generated a truncated subunit that was partially rescued by aminoglycoside-induced stop codon read-through

► Dravet syndrome-associated mutation GABRG2(Q40X) decreased γ2 subunit mRNA levels. ► Undegraded mutant mRNA was translated to truncated γ2 subunits that were cleaved. ► Mutant γ2(Q40X) subunits were not assembled into functional GABAA receptors. ► Aminoglycosides partially rescued wildtype γ2 subunit expression from mutant mRNA. ► Rescued γ2 subunits had the same expression and function as wildtype γ2 subunits.