Figure 1.

Generation of Kcnq2 A306T and Kcnq3 G311V knockin mice Schematic representation of the wild-type and targeting construct used to make Kcnq2^A306T/A306T (A) and Kcnq3^G311V/G311V (B) mutant mice. Numbered boxes denote exons; the asterisk (*) represents the A306T missense change introduced into exon 6 of Kcnq2 and G311V into exon 5 of Kcnq3; B denotes BamH1 sites used in Southern blot analysis. The 5′ probe is used to confirm the presence of the endogenous and targeted alleles on genomic Southern blot of ES cells. The ACN cassette is made up of Cre-recombinase gene (Cre) driven by the testes-specific promoter from the angiotensin-converting enzyme gene (tACE); Cre is linked to the Neo^r selectable marker driven by the mouse RNA polymerase II large subunit gene (polII); the entire cassette is flanked by 34 bp loxP sites orientated in parallel. The HSV-TK (TK) gene is used for negative selection of ES cells. Following Cre-mediated self-excision in the chimeric mouse germline, a single loxP site and the point mutation remain (third panel in A and B).