Hero image Ex 44 Del

cat no | io1018

ioSkeletal Myocytes
DMD Exon 44 Deletion

Human iPSC-derived Duchenne muscular dystrophy model

ioSkeletal Myocytes DMD Exon 44 Deletion are opti‑ox™ precision reprogrammed skeletal myocytes carrying a genetically engineered hemizygous deletion in exon 44 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 a physiologically relevant human cell model. Use the cells to study how the exon deletion impacts muscle cell function, and investigate methods for dystrophin restoration, such as ASO-mediated exon skipping.

 

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Confidently investigate your phenotype of interest across multiple clones with our disease model clone panel. Detailed characterisation data (below) and bulk RNA sequencing data (upon request) help you select specific clones if required.

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Benchtop benefits

 
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Disease-related phenotype

Immunocytochemistry shows a lack of dystrophin in the DMD Exon 44 Deletion cells, and dystrophin restoration has been demonstrated by ASO exon skipping.

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Consistent

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

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Make True Comparisons

Pair the DMD disease model cells with the genetically matched wild-type skeletal muscle cells to study the impact of the deletion, or test methods for dystrophin restoration.

Technical data

Highly characterised and defined

ioSkeletal Myocytes DMD Exon 44 Deletion disease model cells express skeletal muscle cell specific markers and lack expression of Dystrophin, demonstrating a Duchenne muscular dystrophy phenotype

ioSkeletal Myocytes DMD Ex 44 DMD ICC combined (1)
Immunocytochemistry staining at day 10 post revival demonstrates robust expression of Desmin, a component of the contractile apparatus, and no expression of Dystrophin in the ioSkeletal Myocytes DMD Del Ex44/Y disease model cells, whereas ioSkeletal Myocytes, the wild type isogenic control, express both markers (upper panel). Robust expression of Myosin Heavy Chain (MHC) and the muscle transcription factor Myogenin is observed in both ioSkeletal Myocytes DMD Del Ex44/Y and ioSkeletal Myocytes (lower panel). Anti-dystrophin antibody clone 2C6 (MANDYS106).

ioSkeletal Myocytes DMD Exon 44 Deletion disease model cells demonstrate classical skeletal myocytes morphology

 ioSkeletal Myocytes DMD Exon 44 classical myocyte morphology shown at day 10 post revival by bright-field imaging.

ioSkeletal Myocytes DMD Exon 44 Deletion form elongated, multinucleated myocytes over 10 days, comparable to the wild-type ioSkeletal Myocytes isogenic control. Day 3 to 10 post-revival; 100X magnification.

ioSkeletal Myocytes DMD Exon 44 Deletion disease model cells demonstrate gene expression of key myogenic markers following reprogramming

 ioSkeletal Myocytes DMD Exon 44 Deletion gene expression of key myogenic markers by RT-qPCR

Following reprogramming, ioSkeletal Myocytes DMD Exon 44 Deletion (DMD DelEx44/Y) and wild type ioSkeletal Myocytes (WT Control) downregulate expression of pluripotency genes (A), while demonstrating expected expression of key myogenic markers (B). Gene expression levels assessed by RT-qPCR. Data expressed relative to the parental human iPSC (hiPSC), normalised to HMBS. Data represents day 10 post-revival samples.

Seeding density

ioioSkeletal Myocytes generic DM seeding density

ioSkeletal Myocytes DMD Del Ex44/Y are compatible with plates ranging from 6 to 384 wells and are available in two vial sizes, tailored to suit your experimental needs with minimal waste.
The recommended seeding density is 100,000 cells/cm2.
One small vial can plate a minimum of 0.5 x 24-well plate, 0.75 x 96-well plate, or 1 x 384-well plate. One large vial can plate a minimum of 1 x 24-well plate, 1.5 x 96-well plates, or 2 x 384-well plates.

Cells arrive ready to plate

ioSkeletal Myocytes DMD Exon 44 Deletion cells arrive ready to plate and are ready for experimental use in less than 10 days

ioSkeletal Myocytes DMD Exon 44 Deletion are delivered in a cryopreserved format and are programmed to mature rapidly upon revival in the recommended medium. The protocol for the generation of these cells is a two-phase process: Phase 1. Stabilisation for 3 days. Phase 2. Maintenance during which the skeletal myocytes mature.

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 44 deletion in the DMD gene

Applications

ASO-mediated exon skipping
Muscular dystrophy research
Dystrophin restoration
Muscle disease modelling

Product use

ioCells are for research use only

Product resources

Advancements in 3D modeling: Building mature, functional 3D skeletal muscle microtissues in vitro Webinar
Advancements in 3D modeling: Building mature, functional 3D skeletal muscle microtissues in vitro

Dr Marieke Aarts | Principal Scientist | Bi/ond

Amanda Turner | Senior Product Manager | bit.bio

Watch now
Generation of 3D skeletal muscle microtissues using ioSkeletal Myocytes Poster
Generation of 3D skeletal muscle microtissues using ioSkeletal Myocytes

Dr Mitchell Han

Bi/ond

2023

Download
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™

Dr Luke Flatt | Senior Scientist | Charles River Laboratories

Dr Will Bernard | Senior Scientist | 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

2021

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

2020

Download

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.

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