bit.bio iPSC-derived skeletal myocytes DMD exon 51 deletion desmin expression

bit.bio iPSC-derived skeletal myocytes DMD Exon 51 deletion ICC shows lack of dystrophin

bit.bio iPSC-derived skeletal myocytes DMD exon 51 deletion show classical myocyte morphology

cat no | io6023

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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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

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

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.

Quick

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

Cells arrive ready to plate

bit.bio_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)

Vial size

Large: >5 x 10⁶ viable cells,
Evaluation pack*: 3 large vials of >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

* Evaluation packs are intended for first-time users, or for existing users testing a new cell type or derivative. A user can request multiple evaluation packs as long as each one is for a different product.

Highly characterised and defined

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

bit.bio 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.

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

Product resources

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