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²»Á¼Ñо¿Ëù iPSC-derived skeletal myocytes DMD exon 51 deletion desmin expression
²»Á¼Ñо¿Ëù iPSC-derived skeletal myocytes DMD Exon 51 deletion ICC shows lack of dystrophin
²»Á¼Ñо¿Ëù iPSC-derived skeletal myocytes DMD exon 51 deletion show classical myocyte morphology
Gene expression of myogenic markers for DMD exon 45 deletion skeletal myocytes
²»Á¼Ñо¿Ëù iPSC-derived skeletal myocytes DMD exon 51 deletion desmin expression
²»Á¼Ñо¿Ëù iPSC-derived skeletal myocytes DMD Exon 51 deletion ICC shows lack of dystrophin
²»Á¼Ñо¿Ëù iPSC-derived skeletal myocytes DMD exon 51 deletion show classical myocyte morphology
Gene expression of myogenic markers for DMD exon 45 deletion skeletal myocytes

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ioSkeletal Myocytes DMD Exon 51 Deletion

Human iPSC-derived Duchenne muscular dystrophy model

  • Cryopreserved human iPSC-derived cells powered by opti-ox that are ready for experiments in days

  • Study Duchenne muscular dystrophy in a human in vitro model engineered with a DMD exon 51 deletion

  • Consistent, functional model with genetically matched wild type control, suitable for experiments in 2D and 3D muscle bundles

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²»Á¼Ñо¿Ëù iPSC-derived skeletal myocytes DMD exon 51 deletion desmin expression

Human iPSC-derived Duchenne muscular dystrophy model

Immunocytochemistry showing robust expression of desmin (red) with DAPI nuclear stain (blue).
²»Á¼Ñо¿Ëù iPSC-derived skeletal myocytes DMD Exon 51 deletion ICC shows lack of dystrophin

ioSkeletal Myocytes DMD Exon 51 Deletion disease model cells express skeletal muscle cell specific markers and lack expression of dystrophin, demonstrating a Duchenne muscular dystrophy phenotype

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 Ex51/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 Ex51/Y and ioSkeletal Myocytes (lower panel). Anti-dystrophin antibody clone 2C6 (MANDYS106).

²»Á¼Ñо¿Ëù iPSC-derived skeletal myocytes DMD exon 51 deletion show classical myocyte morphology

ioSkeletal Myocytes DMD Exon 51 Deletion disease model cells demonstrate classical skeletal myocytes morphology

ioSkeletal Myocytes DMD Exon 51 Deletion form elongated, multinucleated myocytes over 10 days, comparable to the wild-type ioSkeletal Myocytes isogenic control. Day 3 to 10 post-revival; 10X, scale bar 400 um.

Gene expression of myogenic markers for DMD exon 45 deletion skeletal myocytes

ioSkeletal Myocytes DMD Exon 51 Deletion disease model cells demonstrate gene expression of key myogenic markers following deterministic cell programming

Following deterministic cell programming, ioSkeletal Myocytes DMD Exon 51 Deletion (DMD Del Ex51/Y) and wild type ioSkeletal Myocytes (WT Control) downregulate expression of pluripotency genes, while demonstrating expected expression of key myogenic markers. Gene expression levels were assessed by RT-qPCR (data normalised to HMBS; cDNA samples of the parental human iPSC line (hiPSC) were included as reference). Data represents day 10 post-revival samples, n=2 replicates.

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A maximum number of 20 vials applies. If you would like to order more than 20 vials, please contact us at orders@²»Á¼Ñо¿Ëù.

Human iPSC-derived Duchenne muscular dystrophy model

ioSkeletal Myocytes DMD Exon 51 Deletion are opti‑ox deterministically programmed skeletal myocytes carrying a genetically engineered hemizygous deletion in exon 51 of the DMD gene encoding the dystrophin protein. These cells offer a rapidly maturing, consistent, scalable system to study Duchenne muscular dystrophy in a functional human cell model.

This disease model is part of a Duchenne muscular dystrophy panel of physiologically relevant human iPSC-derived cells that can be incorporated into translational research and drug discovery workflows. The panel includes DMD exon 44, exon 45 and exon 52 deletions. All can be used alongside their genetically matched control ioSkeletal Myocytes.

Benchtop benefits

 
Compare disease model skeletal myocytes with wild type control

Make True Comparisons

Pair the DMD disease model cells with the genetically matched wild type to study the impact of the deletion and to test methods for dystrophin restoration.

Consistent iPSC-derived skeletal myocytes

Consistent

Our platform ensures consistency, overcoming the challenges associated with immortalised cell lines and the donor variability of primary cells and patient-derived iPS cell lines.

Rapidly maturing skeletal myocytes

Quick

Cells arrive programmed to mature rapidly, forming striated, multinucleated myocytes by day 10 post revival.

Cells arrive ready to plate


²»Á¼Ñо¿Ëù_ioSkeletal_Myocytes_with_disease_models_timeline

ioSkeletal Myocytes DMD Exon 51 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 specifications

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, aged 55-60 years old (skin fibroblast), Genotype APOE 3/4

Vial size

Large: >5 x 106 viable cells

Quality control

Sterility, protein expression (ICC), gene expression (RT-qPCR) and genotype validation (gel electrophoresis)

Differentiation method

opti-ox deterministic cell programming

Recommended seeding density

100,000 cells/cm2

User storage

LN2 or -150°C

Format

Cryopreserved cells

Genetic modification

Hemizygous exon 51 deletion in the DMD gene

Applications

Muscle and neuromuscular research
Disease modelling
Contractility assays
3D muscle tissue engineering

Product use

ioCells are for research use only

 

Scale your study with volume pricing

Enabling scientists to use human cells in their research, running additional experiments without rationing cells or limiting experimental scale

 

Order quantity Total vials received Pricing tier 
1 - 9 packs 2 - 18 vials Standard price
10 - 49 packs 20 - 98 vials Automatic 10% discount
> 50 packs > 100 vials > Contact us for a quote

 

Academic pricing: Academic users can purchase any ioCells in 3-vial packs ($/€/£ 999 per pack), available year-round with any cell type combination.
Technical data

Disease-related phenotype

ioSkeletal Myocytes DMD Exon 51 Deletion disease model cells express skeletal muscle cell specific markers and lack expression of dystrophin, demonstrating a Duchenne muscular dystrophy phenotype

²»Á¼Ñо¿Ëù iPSC-derived skeletal myocytes DMD exon 51 deletion ICC shows lack of dystrophin

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 Ex51/Y disease model cells, whereas ioSkeletal Myocytes, the wild type, genetically matched 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 Ex51/Y and ioSkeletal Myocytes (lower panel). Anti-dystrophin antibody clone 2C6 (MANDYS106).

Highly characterised and defined

ioSkeletal Myocytes DMD Exon 51 Deletion disease model cells demonstrate classical skeletal myocytes morphology

²»Á¼Ñо¿Ëù iPSC-derived skeletal myocytes DMD exon 51 deletion show classical morphology

ioSkeletal Myocytes DMD Exon 51 Deletion form elongated, multinucleated myocytes over 10 days, comparable to the wild-type ioSkeletal Myocytes genetically matched (isogenic) control. Day 3 to 10 post-revival; 10X objective lens, scale bar 400 um.

ioSkeletal Myocytes DMD Exon 51 Deletion disease model cells demonstrate gene expression of key myogenic markers following deterministic cell programming
²»Á¼Ñо¿Ëù skeletal myocytes DMD exon 51 deletion gene expression by RT-qPCR
Following deterministic cell programming, ioSkeletal Myocytes DMD Exon 51 Deletion (DMD Del Ex51/Y) and wild type ioSkeletal Myocytes (WT Control) downregulate expression of pluripotency genes, while demonstrating expected expression of key myogenic markers. Gene expression levels were assessed by RT-qPCR (data normalised to HMBS; cDNA samples of the parental human iPSC line (hiPSC) were included as reference). Data represents day 10 post-revival samples, n=2 replicates.

How to culture ioSkeletal Myocytes

In this video, our scientist will take you through the step-by-step process of how to thaw, seed and culture ioSkeletal Myocytes.

Cell culture hacks | human iPSC-derived skeletal myocytes 

Read this blog on skeletal myocytes cell culture for our top tips on careful handling, cell plating and media changes to achieve success from the outset.

Read the blog
²»Á¼Ñо¿Ëù_ioskeletal myocytes About us

Product resources

ioSkeletal Myocytes Brochure
ioSkeletal Myocytes

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Stimulating 3D Skeletal Muscle Microtissues in a Novel Perfusable Microphysiological System with Integrated Electrodes

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Generation of human iPSC-derived Duchenne muscular dystrophy skeletal myocytes suitable for 3D functional studies and investigating methods for dystrophin restoration Poster
Generation of human iPSC-derived Duchenne muscular dystrophy skeletal myocytes suitable for 3D functional studies and investigating methods for dystrophin restoration

Bernard, et al

²»Á¼Ñо¿Ëù

2024

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