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B-hDMD(exon44-46, del45) mice
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

NCBI Gene ID
1756
Description

  • 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.

Targeting strategy

Gene targeting strategy for B-hDMD(exon44-46, del45) mice. 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. 

mRNA expression analysis

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Strain specific analysis of DMD mRNA expression in wild-type C57BL/6JNifdc mice and B-hDMD(exon44-46, del45) mice by RT-PCR. Heart and skeletal muscle RNA were isolated from wild-type C57BL/6JNifdc mice (+/+) and homozygous B-hDMD(exon44-46, del45) mice (H/Y), then cDNA libraries were synthesized by reverse transcription, followed by PCR with mouse Dmd primers. The transcripts in homozygous mice are shorter than that in wild-type mice, and the sequences were confirmed via Sanger Sequencing.

Protein expression analysis

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Western blot analysis of DMD protein expression in wild-type C57BL/6JNifdc mice and homozygous B-hDMD(exon44-46, del45) mice. Various tissue lysates were collected from wild-type C57BL/6JNifdc mice (+/+) and homozygous B-hDMD(exon44-46, del45) mice (H/Y), and then analyzed by western blot with anti-Dystrophin antibody (Sigma-Aldrich, D8168). 40 μg total proteins were loaded for western blotting analysis. DMD was detected in heart, skeletal muscle, skin and brain, but not in liver and kidney.

Serum creatine kinase activity

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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.