The GABRA1 A322D knock-in mouse model recapitulated the absence seizure phenotype associated with juvenile myoclonic epilepsy, and molecular mechanism study showed that the A322D mutation in the transmembrane helix 3 reduced the α1 subunit protein level as well as the miniature IPSCs in motor cortex [50]. This evidence concerns the gene GABRA1 and juvenile myoclonic epilepsy.