cat no | io6023
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
Human iPSC-derived Duchenne muscular dystrophy model
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).
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.
A maximum number of 20 vials applies. If you would like to order more than 20 vials, please contact us at orders@bit.bio.
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.
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.
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.
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.
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, 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).
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 genetically matched (isogenic) control. Day 3 to 10 post-revival; 10X objective lens, scale bar 400 um.
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.
Bernard, et al
bit.bio
2024
bit.bio
Dr Marieke Aarts | Principal Scientist | Bi/ond
Amanda Turner | Senior Product Manager | bit.bio
Dr Mitchell Han
Bi/ond
2023
Dr Will Bernard | Director of Cell Type Development | bit.bio
Dr Luke Flatt | Senior Scientist | Charles River Laboratories
Dr Will Bernard | Senior Scientist | bit.bio
Further your disease research by pairing our wild type cells with isogenic disease models.
Consistent. Defined. Scalable.