hDMD/mdx ES cell line

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 […]

Contributors

Inventor Institute
Annemieke Aartsma-Rus Leiden University and Leiden University Medical Center
Cat. #: 154188
Unit size: 1×10^6 cells / vial
Research Fields: Cell biology;Drug development
Organism: Human
Tissue: Embryo
Model: Stem Cells
Alternate name: Dystrophin, Muscular Dystrophy, Duchenne And Becker Types, DXS164, DXS26, DXS23, DXS239, DXS268, DXS269, DXS27, DXS272
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 an ES mouse cell line carrying the complete human DMD gene integrated in the mouse genome on an mdx background. This cell line was used to generate the hDMD/mdx mouse (Cat No:154187)
Conditional: No
Production details: Blastocysts were isolated from time mated hDMD male mice and super ovulated mdx female mice. These blastocysts were layered on murine embryonic fibroblast (MEF) feeder cells in a well of a 24-well plate, in knockout DMEM supplemented with 2 mM L-glutamine, 1 mM sodium pyruvate, non-essential amino acids, 50 units/ml of penicillin as well as streptomycin, 1000 units/ml of LIF) and 15% knockout serum replacement. Usually after 6 days blastocysts had hatched and a small colony of cells had forme…
Parental cell line: Blastocysts were cultured to generate ES cell lines
disease: Duchenne muscular dystrophy
Format: Frozen
Shipping conditions: Dry ice
Growth medium: ES medium with 15% knock serum replacement, on MEF coated plates
References:

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

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