德晋贵宾厅

• 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 exon 52 of mouse Dmd gene was deleted in B-Dmd KO(del52) mice.
• Mouse Dmd mRNA and DMD protein were only detectable in wild-type C57BL/6JNifdc mice, but not in homozygous B-Dmd KO(del52) mice.
• This product is used for pharmacodynamics of Duchenne muscular dystrophy.

Gene targeting strategy for B-Dmd KO(del52) mice. The exon 52 of mouse Dmd gene was deleted in B-Dmd KO(del52) mice.

Strain specific analysis of DMD mRNA expression in wild-type C57BL/6JNifdc mice and B-Dmd KO(del52) mice by RT-PCR. Heart and skeletal muscle RNA were isolated from wild-type C57BL/6JNifdc mice (+/+) and homozygous B-Dmd KO(del52) mice (-/Y), then cDNA libraries were synthesized by reverse transcription, followed by PCR with mouse Dmd primers. Dmd mRNA was detectable in wild-type C57BL/6JNifdc mice (+/+) but not the homozygous B-Dmd KO(del52) mice (-/Y).

Western blot analysis of DMD protein expression in B-Dmd KO(del52) mice. Various tissue lysates were collected from wild-type C57BL/6JNifdc mice (+/+) and homozygous B-Dmd KO(del52) mice (-/Y), and then analyzed by western blot with anti-Dystrophin antibody (Sigma, D8168). 40 μg total proteins were loaded for western blot analysis. DMD was only detectable in heart and muscle from wild-type C57BL/6JNifdc mice (+/+) but not homozygous B-Dmd KO(del52) mice (-/Y).