cat no | io1019
ioSkeletal Myocytes DMD Exon 52 Deletion are opti‑ox™ precision reprogrammed skeletal myocytes carrying a genetically engineered hemizygous deletion in exon 52 of the DMD gene encoding the Dystrophin protein. These cells offer a rapidly maturing, consistent and scalable isogenic system to study Duchenne muscular dystrophy in vitro. Use the cells to study how the exon deletion impacts muscle cell function, and investigate methods for dystrophin restoration.
A related disease model is coming soon with a hemizygous exon 44 deletion. Use these models alongside their genetically matched control, ioSkeletal Myocytes™ to make true comparisons in your experiments.
Coming Soon
Register your interest, and we will notify you as soon as the product is available.
Disease-related phenotype
The disease model cells lack expression of dystrophin, as determined by immuno-cytochemistry.
Consistent
Our platform ensures consistency and reproducibility, overcoming the challenges associated with the use of primary muscle cells.
Make True Comparisons
Pair the ioDisease Model Cells with the genetically matched wild-type ioSkeletal Myocytes to study the impact of the mutation, or test methods for dystrophin restoration.
Starting material
Human iPSC line
Karyotype
Normal (46, XY)
Seeding compatibility
6, 12, 24, 48, 96 & 384 well plates
Shipping info
Dry ice
Donor
Caucasian adult male (skin fibroblast)
Vial size
Small: >2.5 x 106 viable cells
Large: >5 x 106 viable cells
Quality control
Sterility, protein expression (ICC), gene expression (RT-qPCR) and genotype validation (Sanger sequencing)
Differentiation method
opti-ox cellular reprogramming
Recommended seeding density
100,000 cells/cm2
User storage
LN2 or -150°C
Format
Cryopreserved cells
Genetic modification
Hemizygous exon 52 deletion in the DMD gene
Applications
Muscular dystrophy research
Dystrophin restoration
Muscle disease modelling
Product use
ioCells are for research use only
Dr Will Bernard | Director of Cell Type Development | bit.bio
Charles River Laboratories & bit.bio
Bernard | et al
bit.bio
bit.bio
Bernard et al
bit.bio
Prof Hagan Bayley | University of Oxford
Dr Mark Kotter | Founder and CEO | bit.bio
Read this blog to find out how experts from across academia and industry are approaching the challenges of reproducibility of in vitro cell models as well as potential solutions.
Further your disease research by pairing our wild type cells with isogenic disease models.