Cat. #157741
DM1-2 iPAX7-hiPS cell line
Cat. #: 157741
Unit size: 1x10^6 cells / vial
Organism: Human
Disease: Muscular dystrophy
Model: Stem Cells
£575.00
This fee is applicable only for non-profit organisations. If you are a for-profit organisation or a researcher working on commercially-sponsored academic research, you will need to contact our licensing team for a commercial use license.
Contributor
Inventor: Rita Perlingeiro Kyba
Institute: The University of Minnesota Twin Cities
Tool Details
*FOR RESEARCH USE ONLY (for other uses, please contact the licensing team)
- Name: DM1-2 iPAX7-hiPS cell line
- Alternate name: Myotonic dystrophy 1 (DM-1)
- Research fields: Cell signaling and signal transduction;Drug development
- Parental cell: skin fibroblasts from a diagnosed DM1 patient
- Organism: Human
- Disease: Muscular dystrophy
- Model: Stem Cells
- Description: Myotonic dystrophy is a genetic disease characterized by a loss of muscle function. Myotonic dystrophy type 1 (DM1), the most common form of myotonia, is caused by mutations in the DMPK gene. Currently, there is no cure or treatment for DM1. 4 total cell lines are available: 2 undifferentiated hiPSC lines from two DM1 patients (DM1-1 hiPS cell line, DM1-2 hiPS cell line) and 2 differentiated skeletal muscle cell lines (DM1-1 iPAX7-hiPS cell line and DM1-2 iPAX7-hiPS cell line).
- Production details: To generate a cell-based model for DM1, researchers obtained a sample of skin fibroblasts from two diagnosed DM1 patients. These fibroblasts (DM1-1 and DM1-2) were reprogrammed to hiPS cells via the Sendai virus method. Following reprogramming, DM1-1 and DM1-2 hiPS cells were modified to express PAX7 under the presence of exogenous doxycycline. Expression of PAX7 results in the differentiation of the DM1-1 and DM1-2 hiPSC cells into skeletal muscle cells. These differentiated muscle cells re...
Target Details
- Target: myotonic dystrophy protein kinase (DMPK)
Handling
- Format: Frozen
- Unit size: 1x10^6 cells / vial
- Shipping conditions: Dry ice
Related Tools
- Related tools: DM1-1 hiPS cell line ; DM1-1 iPAX7-hiPS cell line ; DM1-2 hiPS cell line
