ioSkeletal_Myocytes_crop

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

Register your interest, and we will notify you as soon as the product is available.

Benchtop benefits

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Disease-related phenotype

The disease model cells lack expression of dystrophin, as determined by immuno-cytochemistry.

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Consistent

Our platform ensures consistency and reproducibility, overcoming the challenges associated with the use of primary muscle cells.

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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

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

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

Watch now
Research in Motion with ioSkeletal Myocytes™ Webinar
Research in Motion with ioSkeletal Myocytes™

Charles River Laboratories &  bit.bio


Watch more
Scalable human skeletal myocytes by opti-ox™ reprogramming of iPSCs for the study of muscle and metabolic disorders 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
ioSkeletal Myocytes™ Brochure
ioSkeletal Myocytes™

bit.bio

Download
A novel human skeletal muscle in vitro model using opti-ox™ mediated cellular reprogramming of induced pluripotent stem cells 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
Advances in cellular reprogramming: from stem cells to printed tissues 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.

bit-bio_Addressing the challenges of cell models_Blog_Image1200x755

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Wild Type and Isogenic Disease Model cells: A true comparison.

Further your disease research by pairing our wild type cells with isogenic disease models.

bitbio-vials-Wild_and_Disease-staggered-2500px_wide

Related pages

Discover ioCells Learn about our range of human iPSC-derived cells for research and drug discovery
Resources Explore our latest scientific insights, webinars, blogs and videos
Our platform Discover the cell identity coding platform that powers our ioCells