Basic Information
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Description
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- 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.
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Targeting strategy
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Gene targeting strategy for B-DMD KO mice. The exons 45-50 of mouse Dmd gene were deleted in B-DMD KO mice.
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mRNA expression analysis
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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 (-/-).
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Protein expression analysis
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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 (-/-).
