GABAA receptors are pentamers composed of multiple subunits, which not only determine the cellular location of GABAA receptors but also affect their biological functions. When the subunit is mutated, the receptor cannot effectively respond to GABA released by presynaptic neurons and glia, resulting in central nervous system diseases. This article will review the relationship between GABAA receptor and common diseases of central nervous system, and strive to provide theoretical basis for the treatment of these diseases.