hDMD/mdx mouse

Duchenne muscular dystrophy (DMD) is a muscle-wasting disease in which muscle is continuously damaged, resulting in loss of muscle tissue and function. Antisense-mediated exon skipping is a promising therapeutic approach for DMD which uses sequence specific antisense oligonucleotides (AONs) to reframe disrupted dystrophin transcripts. As AONs function in a sequence specific manner, human specific AONs cannot be tested in the current mdx mouse model for DMD as it carries a mutation in the murine Dmd gene. In order to model the human disease more accurately we generated a mouse model carrying the complete human DMD gene integrated in the mouse…

Contributors

Inventor Institute
Annemieke Aartsma-Rus Leiden University and Leiden University Medical Center
SKU: 154187
Product description: Duchenne muscular dystrophy (DMD) is a muscle-wasting disease in which muscle is continuously damaged, resulting in loss of muscle tissue and function. Antisense-mediated exon skipping is a promising therapeutic approach for DMD which uses sequence specific antisense oligonucleotides (AONs) to reframe disrupted dystrophin transcripts. As AONs function in a sequence specific manner, human specific AONs cannot be tested in the current mdx mouse model for DMD as it carries a mutation in the murine Dmd gene. In order to model the human disease more accurately we generated a mouse model carrying the complete human DMD gene integrated in the mouse genome on an mdx background, which can be used as a control when comparing to the disease model we also generated which harbours a mutation in exon 52 the human DMD gene (Cat No:154189)
Alternate name: Dystrophin, Muscular Dystrophy, Duchenne And Becker Types, DXS164, DXS26, DXS23, DXS239, DXS268, DXS269, DXS27, DXS272
Conditional: No
Production details: Blastocysts were isolated from time mated hDMD male mice and super ovulated mdx female mice. Briefly, female mice of 5-6 weeks of age were intraperitoneally injected with 0.1 ml folligonan (5 IU/100 Îźl) and 48 hrs later with 0.1 ml of chorulon (5 IU/100 Îźl). Directly after the second injection the female mdx mice were housed with a male hDMD mouse during the night. In the morning females were checked for a vaginal plug and were separated from the male mouse. Two and half day later female mdx mice were sacrificed and the ovaries were isolated and flushed with phosphate buffered saline (PBS) to collect the blastocysts. These blastocysts were cultured to generate ES cell line, cells of the male hDMD/mdx A1 cell line were injected in C57/BL6 blastocysts and these blastocysts were subsequently transplanted in foster mothers
Cat. #: 154187
Research Fields: Cell biology; Drug development
Target: DMD
Disease: Duchenne muscular dystrophy
Model: Transgenic
Strain: C57BL/6
References:

Veltrop et al. 2013. PLoS Curr. 5:. PMID: 24057032

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