Tg(Ckm-Dmd*)11956Chmb

Mice with this transgene express an internally deleted mouse dystrophin protein under direction of the muscle specific enhancer and promoter of the mouse creatine kinase, muscle (Ckm) gene. The transgene contains nucleotides -3300 through +7 from Ckm followed by the SV40 VP1 intron, the dystrophin cDNA and the SV40 late polyadenylation signal. The dystrophin, muscular dystrophy (Dmd) cDNA includes the full length 5' and 3' untranslated regions (UTRs) and contains a deletion mimicking a human Becker muscular dystrophy mutation associated with a very mild phenotype; this deletion encompasses 5,106 base pairs of the coding region, derived from exons 17-48, of the full length muscle mRNA isoform, comprising nearly half the coding sequence. Immunoblot analysis demonstrates uniform expression of the truncated protein in quadriceps at >10-fold and in diaphragm at 0.9-fold the the level of full length dystrophin in the corresponding muscles of wild-type mice. (J:27471)