Tg(tetO-Chrnb2*V287L)H3Gica

This transgene contains the coding sequence, with the intact first intron, of mouse cholinergic receptor, nicotinic, beta polypeptide 2 (neuronal) in which the valine at amino acid position 287 has been replaced by leucine (V287L), replicating a pathogenic human mutation associated with autosomal dominant nocturnal frontal lobe epilepsy (ADNFLE). It is expressed under transcriptional control of the tetracycline transactivator (tTA)( also called the tetracycline response element, TRE), which consists of seven repeats of the tetO operator fused to a minimal cytomegalovirus (CMV) promoter. The mutant acetylcholine receptor subunit is produced only in the presence of the tetracycline transactivator (tTA), expressed from a second transgene; administration of doxycycline inhibits tTA binding to tetO, repressing transcription from the latter. Line H3 was estimated by quantitative Southern blot analysis to carry ~4 tandem copies of the transgene. (J:145855)