2-3-dioxo-6-nitro-7-sulfamoylbenzo(f)quinoxaline and Spasms--Infantile

Infantile spasms (IS) is a catastrophic childhood seizure disorder that is characterized by extensor and/or flexor spasms, cognitive deterioration and a characteristic EEG abnormality. The latter consists of a pattern of a spike-wave followed by an electrodecremental response (EDR), which is a flattening of the EEG waveform amplitude. The mechanism/circuitry that underpins IS is unknown. Children with Down Syndrome (DS) are particularly vulnerable to IS. The standard mouse model of DS is the Ts65Dn mutant mouse (Ts). Using the Ts mouse, we have created an animal model of IS in DS. This model entails the treatment of Ts mice with a GABA. To address this question, we used kcnj6 triploid mice, and compared the number of spasms via video analysis and EDR events via EEG to that of the WT mice.. We now show that GABA. It is therefore likely that GIRK2 is working in concert with another factor or factors that are altered in the Ts brain in the production of the GABA

Topics: 2-Amino-5-phosphonovalerate; Animals; Anticonvulsants; Disease Models, Animal; Dose-Response Relationship, Drug; Down Syndrome; Electroencephalography; Embryo, Mammalian; G Protein-Coupled Inwardly-Rectifying Potassium Channels; Genotype; Hippocampus; Humans; In Vitro Techniques; Infant; Membrane Potentials; Mice; Mice, Inbred C57BL; Patch-Clamp Techniques; Peptide Hydrolases; Quinoxalines; Sodium Oxybate; Spasms, Infantile; Trisomy