B-Dmd KO(del45-50) mice
| Strain Name | C57BL/6JNifdc-Dmdtm1(Dmd Exon45-50 del) /Bcgen | Common Name | B-Dmd KO(del45-50) mice |
| Background | C57BL/6JNifdc | Catalog number | 113915 |
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Aliases |
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NCBI Gene ID |
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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(del45-50) mice.
- Mouse Dmd mRNA and DMD protein were detectable in wild-type C57BL/6JNifdc mice but not in homozygous B-Dmd KO(del45-50) mice.
- This product is used for pharmacodynamics of Duchenne muscular dystrophy.
Gene targeting strategy for B-Dmd KO(del45-50) mice. The exons 45-50 of mouse Dmd gene were deleted in B-Dmd KO(del45-50) mice.
Strain specific analysis of DMD mRNA expression in wild-type C57BL/6JNifdc mice and B-Dmd KO(del45-50) mice by RT-PCR. Heart and skeletal muscle RNA were isolated from wild-type C57BL/6JNifdc mice (+/+) and homozygous B-Dmd KO(del45-50) mice (H/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(del45-50) mice (-/Y).
Western blot analysis of DMD protein expression in B-Dmd KO(del45-50) mice. Various tissue lysates were collected from wild-type C57BL/6JNifdc mice (+/+) and B-Dmd KO(del45-50) mice (-/Y), 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 heart, skeletal muscle and skin from wild-type C57BL/6JNifdc mice (+/+) but not homozygous B-Dmd KO(del45-50) mice (-/Y).
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