cat no | io1019

ioSkeletal Myocytes
DMD Exon 52 Deletion™

Human iPSC-derived Duchenne muscular dystrophy model

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

Benchtop benefits

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.

Product information

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

Product resources

Video

Introducing ioSkeletal Myocytes™ | Developing the next generation of human muscle cells

Dr Will Bernard | Director of Cell Type Development | bit.bio

Watch now

Webinar

Research in Motion with ioSkeletal Myocytes™

Charles River Laboratories & bit.bio

Watch more

Poster

Scalable human skeletal myocytes by opti-ox™ reprogramming of iPSCs for the study of muscle and metabolic disorders

Bernard | et al
bit.bio

Download

Brochure

ioSkeletal Myocytes™

bit.bio

Download

Poster

A novel human skeletal muscle in vitro model using opti-ox™ mediated cellular reprogramming of induced pluripotent stem cells

Bernard et al
bit.bio

Download

Webinar

Advances in cellular reprogramming: from stem cells to printed tissues

Prof Hagan Bayley | University of Oxford
Dr Mark Kotter | Founder and CEO | bit.bio

Watch now

Addressing current challenges of in vitro cell models

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.

Read the blog