Tg(CAG-Bgeo,-SMN2)E9Dscd

The pCCALL2/hSMN2 transgene was designed with, from 5' to 3', the CMV-IE enhancer/chicken beta-actin/rabbit beta-globin hybrid promoter (originally from the pCAGG vector), a loxP-flanked STOP cassette (beta-galactosidase/neomycin resistance cassette [beta-geo] with three copies of the SV40 polyA signal), the 1.37 kbp full-length human SMN2 cDNA sequence including all exons (1-8) from the start codon to the stop codon, and a rabbit polyA signal. The 1.37 kbp human SMN2 cDNA sequence (encoding exons 1-8) was originally derived via RT-PCR performed on total RNA isolated from SMA type 1-derived patient fibroblast cells (Coriell Institute GM 3813; shown to express both full-length and truncated SMN2 transcripts). Lines A9 and E9 was generated. (J:185111)