Gt(ROSA)26Sor^{tm1.1(DUX4*)Plj}

The targeting vector was designed with (from 5 to 3) a loxP site, an FRT-flanked neomycin resistance gene, a lox511 site, an inverted human double homeobox 4 (DUX4) gene with a polyadenylation sequence, and another loxP site and lox511 site, both in opposite orientation to the 5 sites. The DUX4 gene contains 4 silent point mutations in exon 1 that prevent alternative splicing of a truncated isoform, DUX4-s, while maintaining the DUX4-fl ORF. This entire construct was inserted between exons 1 and 2 of the Gt(ROSA)26Sor locus. Flp-mediated recombination removed the FRT-flanked neo cassette. (J:256652)