#157741

DM1-2 iPAX7-hiPS cell line

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
Target Details
Handling
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Tool Details

*FOR RESEARCH USE ONLY

  • 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