Duchenne muscular dystrophy (DMD) is a severe, progressive, muscle-wasting disease that leads to difficulties with movement and premature death.
Duchenne muscular dystrophy (DMD) is caused by mutations in the DMD gene. These mutations frequently entail deletions of one or more exons, which disrupt the open reading frame and introduce a premature stop codon. This leads to the production of a nonfunctional truncated dystrophin protein, resulting in a severe muscle degeneration phenotype.
The exons 45-50 of mouse Dmd gene were deleted in B-DMD KO mice.
Mouse Dmd mRNA and DMD protein were detectable in wild-type C57BL/6JNifdc mice but not in homozygous DMD KO mice.
This product is used for pharmacodynamics of Duchenne muscular dystrophy.
Targeting strategy
Gene targeting strategy for B-DMD KO mice. The exons 45-50 of mouse Dmd gene were deleted in B-DMD KO mice.
mRNA expression analysis
Strain specific analysis of Dmd mRNA expression in wild-type C57BL/6JNifdc mice and B-DMD KO mice by RT-PCR. Heart and muscle RNA were isolated from wild-type C57BL/6JNifdc mice (+/+) and B-DMD KO mice (-/-), then cDNA libraries were synthesized by reverse transcription, followed by PCR with Dmd primers. Mouse Dmd mRNA was detectable in wild-type C57BL/6JNifdc mice (+/+) but not in B-DMD KO mice (-/-).
Protein expression analysis
Western blot analysis of DMD protein expression in B-DMD KO mice. Various tissue lysates were collected from wild-type C57BL/6JNifdc mice (+/+) and B-DMD KO mice (-/-), and then analyzed by western blot with anti-Dystrophin antibody (Sigma, D8168). 50 μg total proteins were loaded for western blotting analysis. DMD was detectable in wild-type C57BL/6JNifdc mice (+/+) but not in B-DMD KO mice (-/-).