| Strain Name |
C57BL/6JNifdc-Dmdtm1(mDmd Exon44-46 del; hDMD Exon44, Exon46 ins)Bcgen/Bcgen |
Common Name | B-hDMD(exon44-46, del45) mice |
| Background | C57BL/6JNifdc | Catalog number | 114088 |
|
Aliases |
BMD, CMD3B, MRX85, DXS142, DXS164, DXS206, DXS230, DXS239, DXS268, DXS269, DXS270, DXS272 |
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NCBI Gene ID |
1756 | ||
- 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 44, exon 45 and exon 46 of mouse Dmd are replaced by exon 44 and exon 46 of human DMD in B-hDMD(exon44-46, del45) mice.
- Mouse DMD protein was only detectable in wild-type C57BL/6JNifdcNifdc mice but not in homozygous B-hDMD(exon44-46, del45) mice. And serum creatine kinase activity was significantly greater in homozygous B-hDMD(exon44-46, del45) mice compared to that in wild-type mice.
- This product is used for pharmacodynamics of Duchenne muscular dystrophy.
Serum creatine kinase activity analysis in wild-type C57BL/6JNifdc mice and homozygous B-hDMD(exon44-46, del45) mice by colorimetric. Serum was collected from wild-type C57BL/6JNifdc mice (+/+, n=3, 6-week old male) and homozygous B-hDMD(exon44-46, del45) mice (H/Y, n=3, 6-week old male). Creatine kinase activity was analyzed by colorimetric in a kinetic mode every 2 min for a total of 36 min at 37℃ (creatine kinase activity assay kit: Abcam, ab155901). OD450 readings were linear between T1 (at time 6) and T2 (12min for WT and homozygous mice). Creatine kinase activity was significantly greater in homozygous B-hDMD(exon44-46, del45) mice compared to that in wild-type mice. Values are expressed as mean ± SEM. Multiple t test, p=0.01.
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