Mouse-derived FSHD muscle cell lines
Primary skeletal muscle cell lines isolated from DUX4 expressing FSHD mice.
Applications
- In vitro cell culture
- Drug development
Key Benefits & Differentiators
- Derived from mouse models with DUX4 gene conformation appropriate for drug testing
Technology Overview
Facioscapulohumeral muscular dystrophy (FSHD) is one of the most frequent hereditary muscle disorders. The DUX4 gene encodes a protein that is expressed in FSHD but not in healthy muscle cells. This inappropriate expression initiates a gene deregulation cascade causing differentiation defects, muscle atrophy, and oxidative stress. To test potential drug candidates, appropriate research tools (e.g. cell lines) are needed.
Researchers at the University of Minnesota have developed primary skeletal muscle cell lines isolated from DUX4 expressing FSHD mice. These myoblast and fibroblast cell lines are appropriate for testing potential drug candidates against DUX4 protein.
Technical Information
Primary mouse myoblasts from iDUX4pA mouse model
Organism: Mus musculus, mouse
Tissue: Skeletal Muscle Cell Type: Myoblast
Disease: Facioscapulohumeral muscular dystrophy (FSHD)
Applications: These primary adherent cells are grown in culture to model DUX4 myopathy. Suitable for in vitro testing of drug candidates and mechanistic studies.
Product format: Frozen
Description: Primary myoblasts isolated from the skeletal muscle of FSHD mouse model described in Dandapat, A. et al. (2014). Cell Rep. 8(5):1484-96.
Primary mouse fibroblasts from iDUX4pA mouse model
Organism: Mus musculus, mouse
Tissue: Skeletal Muscle
Cell Type: Fibroblast
Disease: Facioscapulohumeral muscular dystrophy (FSHD)
Applications: These primary adherent cells are grown in culture to model DUX4 myopathy. Suitable for in vitro testing of drug candidates and mechanistic studies.
Product format: Frozen
Description: Primary fibroblasts isolated from the skeletal muscle of FSHD mouse model described in Dandapat, A. et al. (2014). Cell Rep. 8(5):1484-96.
Phase of Development
Cell lines have been established and characterized.
Desired Partnerships
This technology is now available for:- License
- Sponsored research
- Co-development
Please contact our office to share your business’ needs and learn more.
Researchers
- Michael Kyba, PhD Position, Department of Pediatrics
- Darko Bosnakovski, DVM, PhD Assistant Professor, Department of Pediatrics